Abstracts of the Fourth Joint Meeting of the European Congenital Heart Surgeons Association and the Congenital Heart Surgeons' Society,Venice,Italy,June 24–26,2016

¹Department of Surgery, University of Nebraska Medical Center, Omaha, Nebraska USA, ²Department of Pediatrics, University of Nebraska Medical Center, Omaha, Nebraska USA

The Melody transcatheter valve has been adopted for replacement of the mitral or tricuspid valve. Previously demonstrated techniques for Melody atrioventricular valve implantation require time-consuming preparation of the prosthesis. This video demonstrates a rapid, simplified technique for Melody atrioventricular valve implantation.

In this technique, the annulus is sized, and a similar-sized ePTFE tube graft, 16 to 24 mm, is selected. A 15 mm long cuff of tube graft is cut, and one end is sutured to the annulus, so that the cuff sits in the atrium. Meanwhile, the valve is prepared by folding over the last row of stent wire spaces at each end, to shorten the valve from 33 to 23 mm length, and reduce projection into the atrium or ventricle. The valve is mounted to a balloon of equal diameter to the tube graft, and then deployed by dilation at the desired depth within the tube graft. If there is a concern for impingement on the ventricular outflow tract, the ventricular end of the stent can be bent inward slightly. The stent is tacked to the tube graft.

The video consists of a demonstration of these techniques during mitral valve Melody implantation in the arrested heart. For extra clarity, the steps will also be demonstrated in a porcine cardiac specimen. This technique reduces the time from decision to completed implantation to approximately 20 minutes, making the procedure particularly applicable as rescue after a long ischemic time for complex attempted native valve repair.

¹German Heart Center Munich, Munich, GERMANY

Objective: Primary tricuspid valve surgery carries the highest risk of isolated valve procedures, and repair of tricuspid defects is associated with a halving of early and late mortality. However, in primary tricuspid disease with irreparable leaflets or tethering, conventional repair can be difficult. This video illustrates cylinder reconstruction of the tricuspid valve for severely diseased and tethered leaflets.

Methods: A 44 year-old female presented with resting cyanosis, NYHA Class III CHF, and severe peripheral edema. Evaluation showed a large untreated secundum ASD, severe tricuspid regurgitation, right ventricular dysfunction, and pulmonary hypertension (Rp/Rs=0.6). The tricuspid annulus was 6 cm, and all leaflets were prolapsing and tethered. Using cardiopulmonary bypass and cardioplegic arrest, the large tricuspid annulus was reduced to 33 mm with posterior suture annuloplasty. A 33 mm cylinder of tissue-engineered bovine pericardium (CardioCel®, Admedus, Fremantle, Australia) then was attached to 3 equally spaced trabeculae in the RV inflow, and subsequently sutured circumferentially to the tricuspid annulus. The secundum ASD was closed with a pericardial patch.

Results: Postoperatively, the tricuspid cylinder reconstruction opened and closed well, was fully competent, and had a low transvalvular gradient. The patient recovered uneventfully, and has become asymptomatic at 1 year of follow-up.

Conclusions: Clinical outcomes are better after repair of the tricuspid valve, and with reparable leaflets, standard ring/patch/chord techniques work well. However, when leaflets are irreparable or tethered, pericardial cylinder reconstruction can be applied, and is a highly reproducible procedure that produces excellent hemodynamic results. Cylinder reconstruction could increase repair rates, even for primary structural tricuspid disease.

¹Department of Cardiac Surgery, Ukrainian Childrens Cardiac Center, Kyiv, UKRAINE, ²Department of Cardiology, Ukrainian Childrens Cardiac Center, Kyiv, UKRAINE

Introduction: We describe successful Ross-Konno procedure, cylinder mitral valve replacement and subtotal LV fibroelastosis resection in a 49 days old infant after previous aortic balloon valvuloplasty.

Patient and methods: Patient was prenatally diagnosed with critical aortic stenosis, LVOTO, LV fibroelastosis and severe mitral dysplasia (severe stenosis and mild insufficiency). Just after birth baby was admitted to our Center, urgent aortic valve balloon valvuloplasty was performed and LV-Ao systolic pressure gradient decreased from 80 to 30 mm Hg, minimal aortic valve regurgitation developed. The child was readmitted at 44 days with symptoms of congestive heart failure. Intraoperatively AoV and MV were deemed irreparable. Pulmonary autograft was harvested, and LVOTO resection was started. We removed mitral valve and subvalvar apparatus. Subtotal LV fibroelastosis resection was then completed through LVOT and mitral annulus. Mitral valve was replaced by cylinder valve sewn out of decellularized equine pericardium. Ross-Konno procedure was performed and RVOT was reconstructed with handmade trileaflet ePTFE conduit.

Result: Patient was successfully weaned from CPB. Chest was closed in 5 days after surgery. Early postoperative period was uneventful with no signs of aortic, mitral and pulmonary stenosis or regurgitation.

Comment: With described technique the patient was offered a biventricular repair for complex congenital heart disease.

¹Icahn School of Medicine at Mount Sinai - Department of Cardiovascular Surgery, New York, USA

Objectives: The right axillary incision has been used at our institution for congenital cardiac surgery since 2005 and we have expanded its application to more complex lesions. We report this approach in a 2-month-old, 5.5 kg child with Tetralogy of Fallot (TOF) and a Patent Ductus Arteriosus (PDA).

Methods: A transverse limited incision was made in the right axilla allowing access to the 4th intercostal space. After ligation of a PDA, central aortic and bicaval venous cannulation was performed. After initiation of cardiopulmonary bypass, cardioplegic arrest was achieved. A vertical infundibulotomy was made and a pulmonary valvotomy was performed for a bicommissural pulmonary valve. The right atrium was opened and the ventricular septal defect was repaired through a partially detached tricuspid valve using a PTFE patch. The right ventricular outflow tract was enlarged with muscle division and a patch.

Results: The right axillary incision provided excellent access for a PDA ligation and repair of TOF in a 5.5 kg child with resultant right ventricular pressures 1/3 systemic.

Conclusions: The right axillary incision allows central cannulation for cardiopulmonary bypass, exposure for TOF repair, and a cosmetically superior incision. Right axillary incision is a viable approach to repair of TOF in selected patients.

¹University Medical Center Groningen, Groningen, NETHERLANDS

Objective: Surgeons have a mental conception of prosthetic valvar size as it is labelled, where the label is mentally connected to its size and performance. Effective orifice area (EOA) is provided as an objectivization of valvar performance, wherein flow within its complex structure is taken into account. This study is to evaluate the relationship between these two concepts.

Methods: The applicable ISO standard (5840-2015) was analysed as to the definition of valvar positions in relation to the annulus. The methods leading to the calculation of EOA are analysed.

Results: In the new version of the ISO standard of October 2015 the “intra-supra-annular” valve has disappeared. That leaves only intra- and supra-annular valves as choices for the labelling process. The same valvar housing can be fitted with different sewing rings, leading to supra- or intra-annular placement. Thus the same housing can be labelled differently depending on the sewing ring. The maximal difference found is 3 mm between labels for the same housing. Pressure drop and velocity is measured in vitro by the manufacturer, where after EOA is calculated with the Gorlin equation (1951). However, discharge coefficients are ignored, leading to higher gradients than anticipated. This is then demonstrated postoperatively by echocardiography whence the continuity equation is utilised instead.

Discussion: Cardiac prosthetic valvar labelling depends on intended annular position, either intra- or supra-annular, which is rational but counterintuitive. EOA calculation as mandated by ISO standard overestimates valvar performance systematically. The new ISO standard mandates that some valvar prostheses have to be relabelled.

¹University Medical Center Groningen-Center for Congenital Heart Diseases, Department of Cardiothoracic Surgery, Groningen, NETHERLANDS, ²Leiden University Medical Center-Department of Anatomy & Embryology, Leiden, NETHERLANDS, ³Royal Brompton Hospital-Cardiac Morphology, London, UNITED KINGDOM, ⁴Royal Brompton Hospital-Heart Division, London, UNITED KINGDOM

Introduction: Double inlet left ventricle (DILV) patients are currently palliated with a Fontan procedure. Data on long-term survival with a Fontan circulation are still dire. Natural survivors of DILV, on the other hand, have been reported to live to the sixth decade without surgical intervention. It has been hypothesized that unpalliated patients depend on favorable streaming patterns for separation of deoxygenated and oxygenated blood streams. The aim was to investigate unoperated DILV hearts with a focus on the anatomical relationships facilitating streaming patterns that might make certain variants of DILV hearts superior to Fontan palliated hearts regarding function and survival.

Methods: An examination of 42 post-mortem DILV specimens was conducted. Points of interest were the ventriculo-arterial connection, the spatial relationship of the ventricles, the position and size of the great arteries, the ventricular septal defect, the presence of relative outflow tract stenosis and the relationship of the inflow – and outflow tracts.

Results: The most common anatomy was a discordant ventriculo-arterial connection, a left-sided right ventricle and left anteriorly located aorta. The majority showed no relative pulmonary stenosis. The VSD created a relative subpulmonary or subaortic stenosis in almost half the cases. The largest group of hearts had a parallel relationship of the inflow – and outflow tracts.

Conclusions: The relationship of the inflow- and outflow tracts as well as the presence of (sub-)pulmonary stenosis might play a crucial role in keeping the blood streams in DILV hearts separated in vivo.

¹University of Maryland Medical Center, Baltimore, USA

Objectives: The overall survival rate for hypoplastic left heart syndrome (HLHS) patients remains limited. We describe the rationale from a preclinical model and study design for the first Phase I trial in the United States to evaluate the safety and feasibility of allogeneic mesenchymal stem cell (MSC) therapy in HLHS patients.

Methods and Results: Neonatal Yorkshire swine underwent pulmonary artery banding to induce RV dysfunction. Human MSCs or placebo were injected intramyocardially into the RV. Four weeks post-injection, MSC-treated myocardium had a smaller increase in RV end-diastolic area, end-systolic area, and tricuspid vena contracta width (p < 0.01), increased RV fractional area of change (p < 0.01), and improved myocardial strain mechanics (p < 0.01) relative to placebo. Hypertrophic changes in the RV were more significantly pronounced in the placebo group, as evidenced by greater wall thickness by echocardiography (p = 0.008) and increased cardiomyocyte cross-sectional area (p = 0.001). Based on these preclinical results, a FDA approved trial will be conducted in a total of 30 HLHS patients who will be randomized to receive allogeneic MSCs at the Stage II palliative bidirectional cavopulmonary connection operation versus control. This study will examine safety and feasibility endpoints as well as preliminary efficacy of right ventricular function.

Conclusion: This Phase I trial will provide important insights of allogeneic MSC therapy in HLHS patients, and the lessons learned may be applied to other congenital heart diseases.

¹The Icahn School of Medicine at Mount Sinai - Department of Cardiovascular Surgery, New York, USA, ²The Icahn School of Medicine at Mount Sinai - Department of Anesthesia & Critical Care, New York, USA

Objective: It has been known that fresh whole blood reduces coagulopathy in open-heart surgery, however obtaining fresh whole blood for autologous transfusion is difficult in neonates and small infants. We report a novel technique of consistently obtaining fresh autologous blood (FAB) in <10 kg cardiac surgery patients and its effects on intraoperative coagulopathy.

Methods: After heparinization and prior to initiating cardiopulmonary bypass (CPB), the patient’s blood is drained through the venous cannula into a container by the perfusionist. The technique allows 30 cc/kg of blood volume removal. After heparin reversal, the FAB is re-transfused. We retrospectively reviewed patients whom had FAB during surgery from 2012-2014 and compared intraoperative transfusion rates to a similar cohort who did not.

Results: Thirty-six children undergoing cardiac surgery had FAB while 33 children did not. No significant differences were noted in mean age (108 days with FAB vs 81 days with no FAB, p = 0.183), weight (4.9 kg vs 4.4 kg, p = 0.132), CPB time (132 minutes vs 120 minutes, p = 0.493), or cross clamp time (64 minutes vs 61 minutes, p = 0.742). Intra-operatively, the FAB group had significantly decreased rates of any transfusion [14 (38.9%) vs 25 (75.8%), p < 0.001], prbc [6 (16.7%) vs 14 (42.4%), p = 0.019], platelet [10 (27.8%) vs 23 (69.7%), p < 0.001], and cryoprecipitate [3 (8.3%) vs 9(27.2%), p = 0.038] transfusions (Table 1).

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Table 1.

Intraoperative Blood Products.

	FAB	No FAB	p-value*
	(n = 36)	(n = 33)
Any transfusion, n (%)	14 (38.9)	25 (75.8)	.002
Packed red blood cells, n (%)	6 (16.7)	14 (42.4)	.019
Platelets, n (%)	10 (27.8)	23 (69.7)	<.001
Fresh frozen plasma, n (%)	2 (5.6)	6 (18.2)	.102
Cryoprecipitate, n (%)	3 (8.3)	9 (27.2)	.038

*calculated using t-test for continuous variables, chi-squared test for categorical variables.

Conclusions: We demonstrate an innovative and routine way of collecting FAB in small sized congenital heart surgery patients and its potential benefit in decreasing intraoperative transfusions.

¹University of Michigan C.S. Mott Children’s Hospital, Ann Arbor, USA, ²The Children’s Hospital of Philadelphia, Philadelphia, USA

Objectives: With improvements in short-term outcomes following congenital heart surgery, it has become increasingly important to understand longer-term survival, quality of life (QOL), and burden of disease. However, routine collection of these data is challenging and they remain very limited. We report initial results of a collaborative program incorporating standardized follow-up (FU) into usual care at The Children’s Hospital of Philadelphia (CHOP) and University of Michigan (UM).

Methods: Patients undergoing the 10 STS benchmark operations were included. Annual FU data were collected through chart review and phone interviews, and included vital status, re-interventions/hospitalizations, and burden of disease assessed via standardized questionnaires (including medical/other morbidities and QOL).

Results: The program began on 1/2014 at CHOP (n = 1,635 patients undergoing surgery since 1/2007); and 1/2015 at UM (n = 696 patients undergoing surgery since 1/2011). Overall, FU data (of any type) were obtained on 90% of patients at CHOP (median FU 3.2 years, survival 92%), and 95% at UM (median FU 2.2 years, survival 93%). Across all operations (except for one), the FU rate was >90% at both centers. The burden of disease/QOL questionnaire completion rate was 72% at CHOP and 69% at UM (range 60-91% across operations).

Conclusions: Standardized FU data can be obtained for >90% of children undergoing benchmark operations; burden of disease/QOL data can also be collected on the majority of patients. Ongoing efforts to automate identification and notification of eligible patients, and support online questionnaire completion may reduce resource needs, facilitate expansion across centers, and support multi-center research/quality improvement efforts.

¹Saint Louis University, Saint Louis, USA

Objectives: Optimal procedure to repair supracardiac total anomalous pulmonary venous return remains controversial. Alternative repairs include: trans-septal without cardiac rotation; trans atrial with cardiac rotation and this video describes the superior extracardiac (SEC) technique.

Methods: This is 2.4 kg, 2 day old. Following aortic and single atrial cannulation with ductal ligation, superior vena cava and ascending aorta are circumferentially dissected and retracted laterally; right pulmonary artery is retracted cephalad to expose left atrium and pulmonary venous confluence. To align left atrium with confluence, the appendage is retracted leftward with suture. At 18°C circulatory arrest is employed with cardioplegia. Parallel incisions are made first in venous confluence, then in left atrium with care not to enter the orifice of any pulmonary vein. Stay stitches are applied and veno-atrial anastomosis performed with continuous suture. ASD closure and high ligation of vertical vein is performed in all patients.

Results: August 1995 until March 2016, 36 patients (excluding single ventricles and heterotaxy) have undergone this procedure. Two patients with cardiac TAPVR and proximal venous obstruction to coronary sinus required simultaneous SEC and right atrial repair. There have been no hospital deaths. One patient required reoperation for late venous obstruction.

Conclusions: This video demonstrates that superior extracardiac repair is safe and effective. Anastomotic distortion is avoided because structures always remain in their anatomic position. The video suggests this technique is an effective teaching tool as surgeon and assistant have equal exposure throughout the operation.

¹Hospital for Sick Children - Division of Cardiac Surgery, Toronto, CANADA, ²Cleveland Clinic - Departments of Thoracic and Cardiovascular Surgery and Quantitative Health Sciences, Cleveland, USA, ³Duke University Hospital - Division of Thoracic and Cardiovascular Surgery, Durham, USA, ⁴New York Presbyterian Morgan Stanley Childrens Hospital - Division of Cardiology, New York, USA, ⁵Hospital for Sick Children - Division of Cardiology, Toronto, CANADA

Objective: The effect of the “inter-operation interval,” from the Norwood procedure to Stage-2-Palliation (S2P), on outcomes is unknown. We hypothesized that an optimal interval exists that minimizes pre-S2P mortality and maximizes post-S2P survival.

Methods: The NIH/NHLBI Pediatric Heart Network Single Ventricle Reconstruction Trial public dataset was used. Transplant-free survival (TFS) was modeled from (1) Norwood to S2P and (2) S2P to three years, using parametric hazard analysis. To account for staged procedures, net three-year TFS was calculated as the product of both intervals’ survival functions. Parametric models were used to stratify hypothetical patients by risk factors and shunt types.

Results: The analysis included 547 patients. The mean inter-operation interval was 5.1±1.7 months. A shorter inter-operation interval was associated with lower TFS after S2P (p=0.007). Additional risk factors included greater pre-S2P E/E’ ratios and right ventricular end systolic volumes, the presence of failure to thrive, Sano conduits, atrioventricular valve regurgitation, and the absence of aortic atresia. For average risk patients, an inter-operation interval of 2-4 months maximizes three-year TFS. For patients with multiple risk factors, the optimal interval is longer and dependent upon specific risk factors. The optimal interval is shorter for the Blalock-Taussig shunt vs. Sano conduit, 1.5-3 vs. 3-5 month.

Conclusions: Although a shorter inter-operation interval was a risk factor for lower TFS after S2P, an interval less than the study population’s mean appears to maximize three-year net TFS in patients with few risk factors. A longer interval may optimize outcomes in patients with multiple risk factors.

¹Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, CANADA, ²Department of Critical Care Medicine, The Hospital for Sick Children, Toronto, CANADA, ³Department of Pediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, CANADA

Objectives: The purpose of this study is to characterize the cumulative hospital-related costs associated with in-patient episodes of care of patients treated with Hybrid versus Norwood strategies.

Methods: Between April 2007 and March 2015, 74 Hybrid strategies and 79 Norwood strategies (70 with classic Blalock-Taussig shunt and 9 with Sano shunt) were performed at a single institution. Costs associated with in-patient episodes of care were retrieved from the institution’s Decision Support Financial Department. Patients were censored when they reached 6 years of age or earlier if they experienced Fontan completion, transplantation or death. Costs are presented in Canadian dollars.

Results: Patients in the Hybrid group underwent a total of 366 admissions versus 361 in the Norwood group. The median number of admissions per patient was 3.5 (IQR: 1.3-6.8) in the Hybrid group versus 4.0 (IQR: 2.0-6.0) in the Norwood group (p = 0.99). The median length of stay per admission was 6 (IQR: 1-20) days in the Hybrid group versus 7 (IQR: 1-24) days in the Norwood group (p = 0.41). One hundred sixty-two admissions in the Hybrid group (44%) and 166 in the Norwood group (46%) involved at least one surgical intervention in the operating room. The median cumulative cost per patient was 299,819$ (IQR: 197,286$-576,468$) versus 276,086$ (IQR: 195,955$-490,694$) in the Hybrid and Norwood groups, respectively (p = 0.70). Intensive care unit related expenditure was the largest individual contributor to total cumulative costs in both groups (73,953$ [IQR: 40,393$-144,542$] and 73,685$ [IQR: 49,816$-128,182$] per patient in the Hybrid and Norwood groups, respectively; p = 0.78).

Conclusion: Hybrid and Norwood strategies are associated with equivalent cumulative in-patient health related costs.

¹Hospital de Santa Marta - Centro Hospitalar Lisboa, Cardiothoracic Surgery Department, Lisboa, PORTUGAL, ²Hospital de Santa Marta - Centro Hospitalar Lisboa, Pediatric Cardiology Department, Lisboa, PORTUGAL, ³Centro Hospitalar Lisboa, Anesthesiology Department, Lisboa, PORTUGAL

Objectives: Assess outcomes for Fontan circuits performed in patients with two morphological ventricles.

Methods: Retrospective review study (Jan 94 - Dec 2015) including patients with complex biventricular hearts, deemed inadequate candidates for biventricular correction, being offered a cavopulmonary circuit.

Results: In a population of 81 patients submitted to Fontan surgery, 26 had two ventricles, being considered inadequate for septation. Diagnosis were: DORV (13), TGA & VSDs (5), unbalanced AVSD (5), mixed (3). Median age at cavo-pulmonary Fontan completion was 6 yrs (2.8-18 yrs) and 1.6 staged palliations/patient were performed. There were no early deaths, mean follow-up was 8.4 yrs (1,1-20,2 yrs) with 2 patients lost for follow-up. Seventeen patients remained re-intervention free (71%) and there were 2 late deaths (8.3%). NYHA class at latest follow-up: “I” −18 pts (81%), “II” − 3 pts (14%) and “III” − 1 pt (4.5%). Late complications were: arrhythmias in 2 patients (7.7%) and enteropathy in one (4%). For all the Fontan population, late mortality was 4 (4.9% p = 0.58), entheropathy 4 (4.9% p = 0.62) and arrhythmia 5 (6.2% p = 0,65) – not statistically different.

Conclusion: “Two ventricle” Fontans are safe and well tolerated functionally, however usual Fontan complications occur similarly to “univentricular” Fontans. The potential advantages for incorporating two ventricles in the Fontan circuit are still to be determined, eventually needing larger series.

¹Pediatric and Congenital Cardiac Surgery, Universiti of Padova, Padova, ITALY, ²Pediatric Cardiology, University of Padova, Padova, ITALY

Objective: The one and half ventricle repair (1.5VR) is surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. It consists of a bidirectional cavo-pulmonary connection (BCPC) in addition to repair of intracardiac lesions. With the advantage of achieving a complete separation of pulmonary and systemic circulation and maintaining a pulsatile pulmonary blood flow, it represents an alternative to high risk biventricular repair and avoids the drawbacks of the Fontan circulation. We reviewed our institutional experience with 1.5 VR and analyzed early and long-term results.

Methods: All patients who underwent one and half ventricle repair were included. Baseline characteristics and operative data were obtained by institutional database and clinical charts review. Follow-up data were gained at last outpatient visit or by phone contact.

Results: Between 1994 and 2016, 28 consecutive patients underwent one and a half ventricle repair (M/F=15/13). Median age was 3.5 years (range 5 months – 66.8 years), with a median weight of 15 Kg (range 5.6-83 Kg). Indication for 1.5 VR was RV hypoplasia in 20 patients, RV dysfunction in 7 and subpulmonary LV hypoplasia in transposition of the great arteries in 1. Median tricuspid valve annulus Z-score was −3.5 (range 0.2 and −6.5). Five patients (19%) underwent BCPC at a previous operation. There were no operative deaths. Mean ICU stay was 6±7 days (median 3 days), with a mean hospital stay of 30±28 days (median 21 days). Postoperative complications occurred in 20 patients (74%), most frequent being cardiac or pleural effusion (41%). All patients were discharged home. At a mean follow up of 10 years (range 1 month – 22 years, FU completeness: 97%), there were 2 late deaths (1 non cardiac related). Twenty-two out of 26 survivors (85%) were asymptomatic in good clinical status. Actuarial survival rate was 95% at 10 years and 87% at 20 years. Reoperation were needed in 6 patients (23%); among these 2 patients were converted successfully to biventricular repair. Actuarial freedom from reintervention was 89% at 10 years and 68% at 20 years. Six patients (23%) underwent interventional procedures. Stratifying patients according to the anatomy (“simple” vs “complex”) there were no statistical significant differences in long-term results.

Conclusion: The 1.5V repair is a safe and valid option for surgical repair of hypoplastic or failing right ventricles, with good survival and acceptable rate of reintervention. The vast majority of patients in our series showed good clinical status at follow-up. Conversion from 1.5V to 2V repair is feasible, in selected cases.

¹University Hospital Leuven - Department of Cardiac Surgery Leuven BELGIUM, ²Abiomed Europe GmbH Aachen, GERMANY, ³Leiden University Medical Center - Department of Cardiothoracic Surgery, Leiden, NETHERLANDS, ⁴KU Leuven - Department of Cardiovascular Imaging and Dynamics, Leuven, BELGIUM, ⁵University Hospital Leuven - Department of Paediatric Cardiology, Leuven, BELGIUM

Objectives: The Fontan circulation is associated with distinctly abnormal hemodynamics and a significant risk of progressive failure. We hypothesized that mechanical support from the cavopulmonary connection to a single pulmonary artery could restore haemodynamic stability. Our study evaluated the effects of single pulmonary support in an ovine failing Fontan model.

Methods: A total cavopulmonary connection (TCPC) was established in nine sheep. The superior vena cava was connected to the main pulmonary artery (PA). The inferior vena cava was connected to the PA by an ePTFE conduit. The Impella RP (Abiomed Europe GmbH, Aachen, Germany) was modified to form a cavopulmonary support system. The modified Impella RP, inserted through the main PA, was placed with the inlet lumen in the main PA, distally from the TCPC, and the outlet lumen in the right PA, with flow adjusted to 2.8±0.7 L/min.

Results: The TCPC induced almost a doubling of the central venous pressure (p<0.0001). Five sheep died because of progressive failure of the Fontan physiology. After 12 weeks, implantation of the modified Impella RP in four sheep resulted in a significant increase in left atrial pressure (+163%, p=0.05). Central venous pressure decreased and arterial pressure increased during pump-supported Fontan circulation.

Conclusions: To our knowledge, this is the first chronic ovine model of a total cavopulmonary diversion which provides all hallmarks of the failing Fontan physiology. Mechanical support from the cavopulmonary connection to a single PA can reverse the substantial decrease in left atrial pressure associated with Fontan circulation and might be able to restore normal hemodynamics.

¹University of Michigan Medical School, Department of Cardiac Surgery, Ann Arbor, USA, ²University of Michigan Medical School, Department of Surgery, Ann Arbor, USA, ³University of Michigan, Departments of Biomedical and Mechanical Engineering, Ann Arbor, USA, ⁴University of Michigan Medical School, Department of Otolaryngology, Ann Arbor, USA

Objectives: Tracheobronchomalacia management remains a significant challenge. Current treatment options have high complication and failure rates. Severe forms carry mortality rates of 20-50%.

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Methods: This novel, 3-d printed, bioresorbable tracheobronchial splint is placed externally around the airway. The malacic segment is suspended to the device to maintain patency. The splint’s intended use is to treat severe cases of tracheobronchomalacia.

Results: The splint, custom-designed from each patient’s CT (Figure), has been utilized under FDA emergency-use provision in four patients with imminently life-threatening tracheobronchomalacia. All had dramatic improvement in clinical status and imaging studies (Table).

Conclusions: This novel, bioresorbable, custom-designed, 3-D printed airway splint has proven to be life-saving in this initial cohort of patients. Efforts at a formal trial are ongoing to allow broad availability.

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Table O-16

Patient	Age (months)	Pre-op Status	Segment(s) Addressed			Current Status
			Trachea	L Bronchus	R Bronchus
1	2	Tracheosotomy, PEEP 15, sedation/intermittent paralysis, multiple arrests, post-obstructive pneumonia		x		4 years post-implant, home, tracheostomy, off ventilator, normal bronchial growth
2	16	S/P repair of TOF absent pulmonary valve, tracheosotomy, PEEP 20, sedation/intermittent paralysis, desaturations requiring hand-ventilation, hospitalized since birth		x	x	26 months post-implant, home, tracheostomy, off ventilator during day, normal bronchial growth
3	5	S/P repair of TOF absent pulmonary valve and tracheoplasty, tracheosotomy, sedation/intermittent paralysis, desaturations requiring hand-ventilation, hospitalized since birth	x	x		22 months post-implant, home, tracheostomy, largely off ventilator, normal bronchial growth
4	10	S/P repair of hypoplastic arch/VSD, tracheosotomy, sedation/intermittent paralysis, desaturations requiring hand-ventilation		x		8 months post-implant, home, tracheostomy, off ventilator

¹Great Ormond Street Hospital, London, UNITED KINGDOM

Objectives: Congenital tracheal stenosis carries significant risk for postoperative mortality not only due to the tracheal procedure per se, but also due to associated cardiac anomalies. Combined cardiac procedures along with slide tracheoplasty on cardiopulmonary bypass (CPB) have been widely adopted. The aim of this study is to analyze the risk factors for tracheoplasty with concomitant cardiovascular repair.

Methods: 61 out of 100 consecutive children with congenital tracheal stenosis underwent concomitant cardiac procedures (median age 5 months, IQR 2.8-10.8, median weight 5.8 kg, IQR 4.18-7.74). Fifty-seven patients (57%) required preoperative mechanical ventilation. Eight patients (8%) had hypoplastic right lung and 7 (7%) had lung agenesis. CPB time was ranging from 37 to 371 minutes (median 90 minutes, IQR 65-113). The risk factors including age, weight, requirement of preoperative mechanical ventilation, presence of lung hypoplasia or agenesis, CPB time and RACHS-1 categories were analysed.

Results: There were 9 early deaths (9%) and 6 late mortalities (6%). 10 deaths were seen in those with concomitant cardiovascular procedures against 5 without any additional procedures (p = 0.39). 5, 11, and 45 patients were found in RACHS category-1, -2, and -3 respectively. There was no death in categories 1 and 2. In category 3, there were 5 early and 5 late deaths whereas there were 4 early and 1 late deaths in the group without cardiovascular procedures (p = 0.05). Multivariate analysis showed none of the risk factors was significantly affecting the outcome.

Conclusions: Concomitant cardiovascular procedures at the time of slide tracheoplasty can be performed with good surgical outcome. Although none of the risk factors analyzed were statistically significant, RACHS category 3 or beyond seemed to increase the risk compared other categories.

¹Emory University School of Medicine, Atlanta, USA

Background: Management of hypoplastic aortic arch (≤4 mm or z-value ≤-2) associated with coarctation (CoA) in infancy is challenging. Reverse subclavian flap aortoplasty (RSFA) plus CoA resection offers simplicity without foreign material or cardiopulmonary bypass. It is simpler and more reproducible than an extended end-to-end anastomosis for a hypoplastic arch.

Methods: Since 1988, 134 infants undergoing end-to-end CoA repair had hypoplastic arch enlargement with RSFA. Median age was 7d; mean weight was 3.2 kg. Eighty-seven patients (65%) had associated cardiac defects including single or multiple ventricular septal defects (VSDs, 44), aortic or mitral stenosis (8), transposition of the great arteries (TGA, 10), and atrioventricular septal defect (CAVSD, 9). Sixty patients had pulmonary artery banding (PAB); 15 had VSD closure or an arterial switch operation through a separate median sternotomy.

Results: Median clamp time for the arch repair was 15 minutes and for the coarctation repair was 16 minutes. There were six hospital deaths (4.5%) with no deaths in the 91 patients with no other heart defects or only associated VSD(s). Median hospital stay was 7 days. On follow-up, there were 9 recurrent obstructions: 7 at the CoA site treated with balloon dilatation and two at the arch site repaired surgically. Sixty-five of the 87 patients with associated heart defects (75%) have undergone subsequent repair or single ventricle palliation +/− PAB removal. There have been 7 late deaths (overall survival 90%).

Conclusions: RSFA is excellent for relief of arch hypoplasia and CoA in infants with low recurrence rates and acceptable operative survival.

¹Ann & Robert H Lurie Children’s Hospital of Chicago, Division of Cardiovascular-Thoracic Surgery, Chicago, USA, ²Ann & Robert H Lurie Children’s Hospital of Chicago, Division of Otolaryngology, Chicago, USA, ³Ann & Robert H Lurie Children’s Hospital of Chicago, Division of Medical Imaging, Chicago, USA

Objective: A vascular ring is caused by a right aortic arch has historically been treated by simple ligamentum division. The advent of extremely precise preoperative imaging with 3-dimensional reconstruction of the aortic arch vessels has led to a re-evaluation of this strategy.

Methods: We reviewed the operative notes and hospital records of all patients having an operation for a vascular ring caused by a right aortic arch. Improved preoperative imaging has led to a change in operative strategies.

Results: Between 1947 and 2015 172 patients were treated for a vascular ring caused by a right aortic arch. Of these 117 patients underwent division of a left ligamentum. In the past 15 years 49 patients have undergone in addition resection of a Kommerell diverticulum and transfer of the left subclavian artery to the left carotid artery. Six patients have undergone an aortic uncrossing operation for a circumflex aorta. There has been no operative mortality or significant postoperative morbidity.

Conclusion: The advent of precise preoperative 3-dimensional imaging has led to a paradigm shift in treatment of patients with a right aortic arch. Currently the majority of patients with a right aortic arch undergo not only ligamentum division but also resection of a Kommerell diverticulum if present or conversion to the aortic uncrossing operation if the pathology is caused by a circumflex aorta.

¹Rikshospitalet University Hospital, Oslo, NORWAY

Objective: The approach for surgery of diseases of the aortic arch has changed during the years. We would like to see how different methods have changed the outcome, especially when the patients has grown up to an age of more than fifteen years.

Material: All patients from the start of our database in 1971 were included. No patient was lost to follow-up. The group includes both simple coarctation and arch hypoplasia or interruption. A total of 582 patients underwent surgery up to 2001. There were 335 boys and 247 girls. Early mortality was 9.3%, and mean follow-up was 35.8 years. Resection and direct anastomosis was used in 106 cases, patch aortoplasty in 340, subclavian flap in 7 and extended end-to-end in 120. Tube interposition was only used in nine patients. 230 patients were neonates, there were 129 infants and 223 children. Special attention was directed to patients operated between 1978 and 1981 using a double velour Dacron-patch.

Results: Mortality in patients less than one year was significantly higher than the older. 120 patients needed one reoperation, 19 needed two, and a stent was placed in five patients. Survival in different age groups were influenced by the type of procedure. However, in patients older than one year, there were no differences. Looking into reoperation-free survival, the same pattern was displayed, although there were no differences when the patients were >7 years at surgery. We found a very high incidence of patch aneurysms in the Dacron-double velour patch. We therefore conducted a special follow-up study in this group, looking into the adequacy of follow-up. In 11 out of 31 patients with inadequate follow-up, a patch aneurysm was detected, and in one patient both recoarctation and aneurysm was revealed.

Conclusion: Patients operated for aortic arch obstruction needs a life-long follow-up, especially if a patch procedure has been used. The extended end-to-end technique gives superior results for reoperation free survival, especially in the younger age group.

¹Pediatric Cardiac Surgery, Morgan Stanley Childrens Hospital of New York-Presbyterian, Columbia University, New York, United States Minor Outlying Islands, ²Laboratoire de psychopathologie et neuropsychologie, Université Paris 8 Saint Denis, FRANCE, ³Centre de Référence Malformations Cardiaques Congénitales Complexes - M3C, Centre Chirurgical Marie Lannelongue, Plessis Robinson, FRANCE, ⁴Centre de Référence Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Necker Enfants Malades, Paris, FRANCE

Objective: To determine the neuropsychological and psychosocial outcomes of adult patients who had a neonatal arterial switch operation (ASO) for Transposition of the Great Arteries (TGA) and determine factors associated with pejorative outcomes.

Methods: Sixty-seven ASO patients (22.9±3.3 year-old) and 43 healthy adults (23.8±2.8 year-old) were matched in age, gender and socio-cultural level. Neuropsychological outcome was evaluated using Wechsler Adult Intelligence Scale (WAIS- III), Wisconsin Card Sorting test and California Verbal Learning test. Psychosocial outcome and quality of life were assessed using Mini International Neuropsychiatric Interview, SF-36 survey and Qolibri.

Results: ASO patients had overall IQ scores within the normal range. But the mean full-scale IQ, verbal IQ and performance IQ scores were significantly lower (94.9±15.3; 96.8±16.2; 93.7±14.6 respectively) compared to healthy subjects (103.4±12.3; 102.5±11.5; 103.7±14.3 respectively). Working Memory (91.6±14.4), Perceptual Organization (93.1±15.9) and Processing Speed Indexes (95.4±13.9) were also significantly lower than normal. ASO patients showed poorer performance in 8/13 WAIS-III subtests evaluating attentional capacities, visuospatial abilities and executive functions. They had more depression (46% (n = 31) vs. 21% (n = 9), (p = 0.007) and social phobia (24% (n = 16) vs 7% (n = 3), (p = 0.022). ASO patients have an overall good quality of life. Lower parents’ educational level, longer aortic clamping time and the absence of prenatal diagnosis were significantly associated with lower patient’s IQ (p < 0.001), altered episodic memory (p = 0.021) and working memory (p = 0.023) respectively.

Conclusion: Adults who underwent neonatal ASO for TGA seem to present an increased risk of cognitive, psychological and social impairments. An early identification of emerging difficulties could be helpful.

¹The Hospital for Sick Children, University of Toronto Toronto CANADA

Objectives: We sought to determine if asymptomatic thrombi of unknown clinical significance detected through systematic or incidental ultrasound screening in children after cardiac surgery are associated with impaired neurodevelopmental outcome.

Methods: n = 130 children <5 years old at cardiac surgery were enrolled in a prospective study using systematic vascular ultrasound assessment of all vessels where cannulation was attempted/performed. Thrombi were classified as clinically significant (impaired flow, ischemia, risk of embolization) or of unknown clinical significance based on the expert judgment of at least 2/3 blinded reviewers. Almost all thrombi of unknown significance were non-occlusive, venous and were left untreated. Neurodevelopmental testing with the parent-reported Ages & Stages Questionnaire was performed 12-24 months after the operation.

Results: Enrolled children (55% males) were assessed at a median age of 2.2 years (IQR: 1.7-3.7) and 1.4 years (IQR: 1.1-1.8) after surgery; 92(71%) had no thrombosis, 21(16%) had only thrombi of unknown significance and 17(13%) had ≥1 clinically significant thrombus. Children with clinically significant thrombi had lower than normal scores for gross motor skills (EST: −10(5)/60 points, p = 0.05) and personal/social domains (EST: −10(4)/60 points, p = 0.03). Those with only thrombi of unknown clinical significance had similar neurodevelopmental scores for all domains as those with no thrombi .

Conclusions: We identified a subset of patients with post-operative thrombi that were not associated with an increased risk of acute complications or early risk of negative effects on neurodevelopment. These incidental thrombi without symptoms or signs and with low risk of negative neurodevelopmental consequences could likely be left untreated.

¹University Hospital Bern, Center for Congenital Heart Disease, Department of Cardiovascular Surgery, Bern, SWITZERLAND, ²University Hospital Bern, Center for Congenital Heart Disease, Department of Cardiology, Bern, SWITZERLAND, ³University Hospital Bern, Center for Congenital Heart Disease, Department of Anesthesiology, Bern, SWITZERLAND

Objective: A right axillary vertical mini-thoracotomy is the standard approach for correction of ASD, Sinus venosus defects and partial AV-canal defects at our institution and provides excellent clinical and cosmetic results. Here we report our experience by applying this approach to the correction of VSD and CAVC defects in infants and children.

Methods: Perioperative clinical data of all patients undergoing correction of VSD and CAVC through a right vertical mini-axillary thoracotomy were reviewed. The surgical technique involved a 3-5 cm vertical incision parallel to the anterior axillary fold, central arterial and bi-caval cannulation for institution of mild hypothermic CPB.

Results: A total of 32 patients underwent VSD closure (n = 22; median age: 7.5 (2–138) months; median weight: 6.5 (3.8–39) kg) and correction of CAVC (n = 10; median age: 4.7 (2–25) months; median weight: 5.4 (4.2–9) kg). There was no need for conversion to another approach in any patient. Median CPB and aortic cross-clamp times for VSD correction were 71 (42-139) min and 36 (23-67) min, respectively; for CAVC correction 107 (55-132) min and 71 (32-90) min, respectively. The median ICU stay was for VSD patients 3 days; hospital stay 7.5 days; and for CAVC patients ICU stay 6.5 days, hospital stay 14.5 days. Follow-up is complete with a median of 18 (3-45) months. One patient with severe pneumopathy died at 5 months after surgery due to sepsis and MOF. Follow-up echocardiography demonstrated a residual VSD defect in 1 VSD patient; mild left AV valve insufficiency in 6 and moderate in 1 CAVC patient. One CAVC patient demonstrated an AV-block 1°. All other patients are in sinus-rhythm. No wound infection and no thoracic deformity were observed until present.

Conclusion: By using standard techniques and equipment, and by avoiding peripheral vessel cannulation, the right vertical mini-axillary thoracotomy presents an attractive cosmetic access for safe and complete correction of VSD and CAVC-defects with good results in children.

¹Department of Cardiac Surgery, Boston Childrens Hospital, Boston, USA

Objective: Congenital aortic stenosis presenting in infancy often involves fusion of more than one commissure and dysplasia of more than one leaflet. In infants treated with valvotomy, late valvar regurgitation (AR) can present a complex challenge for the surgeon as options for treatment are limited and results with prosthetic replacement are less than optimal. We have evaluated a reconstruction technique described by Ozaki in young adults for replacing individual leaflets as compared to patch extension with autologous or bovine pericardium in children. We present our initial operative experience with this technique.

Methods and Results: From August 2015 to March 2016 15 children (median age – 11 yrs, IQR: 4-12) underwent aortic valve reconstruction using the Ozaki technique with pediatric and adult sizers and template. 9/15 (60%) had all 3 leaflets replaced, 1/15 (7%) had two leaflets replaced, and 5/15 (33%) had one leaflet replaced. There were no deaths. At discharge, echocardiography demonstrated 5/15 (33%) had none or trivial AR, 10/15 (66%) had mild AR. All patients had reduction in LV volume.

Conclusion: Aortic valve reconstruction remains a technically challenging procedure in infants and children. The Ozaki technique for aortic leaflet replacement is a simple and reproducible technique with promising early results.

¹Filatov Children’s Hospital, Moscow, RUSSIA

Objectives: This study analyzes the results of two-staged ASO in ‘simple’ TGA group of patients where an infection, neurological problems or necrotizing enterocolitis precluded neonatal surgery.

Methods: Transthoracic echocardiography data and outcomes of surgery were investigated in 19 patients (group 1) after two-staged ASO, and 67 patients (group 2) after a single-stage ASO. Age of patients at the first stage was 27±14.9 days and at the second stage 173±51 days. Patients in group 2 were 6.7±2.3 days old. All of them were followed up for 20.4±15,6 months (group 1) and 40.8±21.6 (group 2) after ASO.

Results: There was no operative mortality at both-stages in group 1. The duration of ASO stages were identical in both groups. The duration of the ventilation, length of ITU and hospital stay after surgery were considerably lower in group 1. Actuarial survival and freedom from reoperation at 4 years in group 1 were 100%, and at 5 years in group 2 was 98.5% and 94% respectively. The only difference detected by echocardiography was the aortic valve hinge points Z-score (3.9±1.4 vs 3.1±1.2) and sinuses of Valsalva (3.8±1.4 vs 2.8±1.9), which were slightly higher in 1st group. There was no aortic regurgitation >1 grade.

Conclusions: Two-staged repair of ‘simple’ TGA can be performed with excellent clinical outcomes. The tendency of group 1 patients to have a larger neo-aortic root does not appear to bear any clinical significance in mid-term follow-up.

¹Children National Medical Center, Washington DC, USA, ²Boston Childrens Hospital, Boston, USA

Objectives: Young (<90 days) symptomatic infants with Tetralogy of Fallot are surgically managed with systemic to pulmonary artery shunt (SPS) followed by delayed repair or early primary repair (EPR). We compared outcomes between the two surgical groups.

Methods: The multicenter Pediatric Health Information System (PHIS) database was queried for patients less <90 days of age with a primary diagnosis of tetralogy of Fallot (excluding pulmonary atresia, absent pulmonary valve and atrioventricular septal defect), who underwent non-elective EPR or SPS between January 2008 and December 2014. The initial cohort of 821 patients (554 EPR and 267 SPS) was further propensity matched to account for differences in age and other confounding variables. The propensity matched groups (185 patients in each group) were analysed using linear and binary logistic regression models to determine differences in outcomes.

Results: Initial comparison of 267 SPS to 544 EPR patients, revealed younger age, higher incidence of extracardiac anomalies and prematurity in the SPS group (Table 1). Mortality was comparable between the two groups [EPR: 20/554 (3.6%) vs SPS: 11/267 (4.1%), p = 0.75]. Irrespective of the type of procedure, prematurity (OR = 3.3, 95% CI: 1.5-7.4, p = 0.007) and extracardiac anomalies (OR = 2.5, 95% CI: 1.2-5.3, p = 0.016) were independent risk factors for mortality. Propensity matched analysis revealed no difference in mortality (p = 0.65), duration of ventilation (p = 0.55), hospital length of stay (p = 0.5), ICU length of stay (p=0.15) or hospital costs (p = 0.45).

Conclusion: Outcomes of non-elective early primary repair are comparable to systemic to pulmonary artery shunt in symptomatic patients <90 days old. Barring exceptional anatomical issues, early primary repair should be the preferred approach in this group of patients, given the cumulative risk of a prior palliative procedure, interstage risk and the cost of subsequent repair.

¹Children’s Hospital Colorado, Aurora, USA

Objectives: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that is associated with ischemia and sudden death. Those with an intramural segment are typically managed with a modified unroofing procedure. Controversy exists over the management of AAOCA inter-arterial course and no or minimal intramural segment. For this variant, we have adopted a practice of coronary translocation and direct reimplantation (CTDR) into the appropriate sinus. The purpose of this study is to evaluate the short-term outcome of this cohort.

Methods: Fifty patients underwent surgical repair of AAOCA at our institution between January 2005 and December 2015. Fifteen (30%) underwent CTDR. Thirteen patients in this cohort had AAO of the right coronary artery, 1 had AAO of the left main coronary artery, and 1 had AAO of the left anterior descending coronary artery. Eleven had preoperative symptoms suggestive of myocardial ischemia.

Results: There was no operative mortality in the CRDR cohort. Post-operative coronary flow has been documented either by echocardiography alone in 80% of patients and echocardiography and CT scan in 20%. All patients have normal biventricular function and remain symptom free at a median follow-up of 13 months.

Conclusions: Coronary translocation and direct reimplantation is an acceptable method for repair of AAOCA without an intramural segment but inter-arterial course. Further follow-up is needed to determine long-term durability of this repair.

¹Children’s Mercy Hospital and Clinics, Kansas City, USA

Objectives: Mortality associated with correction of Type IV (Mixed) total anomalous pulmonary venous connection (TAPVC) is generally reported in combination with other anatomic types. The objective of this study was to analyze surgical outcomes associated with Type IV TAPVC utilizing a large multi-institutional cohort. We also analyzed patient specific variables, importantly obstruction to pulmonary venous return, which may contribute to mortality.

Methods: A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) identified patients that underwent repair of Type IV TAPVC between 1982 and 2007. Variables reviewed include gender, prematurity, age at primary repair, anatomic drainage patterns, presence of obstruction, and other major associated cardiac anomalies. The end point of the study was surgical mortality. An anatomic classification system was utilized based on the pulmonary venous drainage to the heart, which included Type IV A (“2+2” pattern), Type IV B (“3+1” pattern), and Type IV C (“bizarre”).

Results: Of the 2,248 patients with the diagnosis of TAPVC, 215 were Type IV (Mixed). The overall unadjusted mortality was 26%. There was no difference in mortality based on the particular anatomic drainage pattern. Twenty-eight percent had partial obstruction of the pulmonary venous return, with no patient having completely obstruction. Obstructed patients had a significantly greater mortality than those which were not obstructed (39% vs. 20%, p = 0.005). None of the cohort underwent an emergency repair secondary to pulmonary venous obstruction. Table I summarize the impact specific patient variables had on mortality.

Conclusions: Type IV TAPVC is a rare disease with a diverse anatomic presentation. Although the no patient with obstruction to the pulmonary venous connection underwent emergent repair, mortality remained significant. This likely represents the intrinsic lung pathology which must be considered in the post-operative period.

¹UCSF Benioff Children’s Hospital, San Francisco, USA, ²C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor, USA, ³Children’s Hospital of Minnesota, Minneapolis, USA, ⁴Ann and Robert H. Lurie Children’s Hospital, Chicago, USA, ⁵Children’s Hospital of Illinois, Peoria, USA

Objectives: The feasibility and impact of regionalization of congenital heart surgery (CHS) in the United States (US) has not been investigated on a national scale. Our study describes the current distribution of US CHS centers and simulates a regionalized system.

Methods: State Inpatient Databases from 2012 were concatenated to characterize the current US CHS network. These data were utilized as they are only available data where distribution of all CHS centers is represented. Centers performing only isolated PDA ligation in preterm infants were excluded (N = 126). Center volume was assessed by the annual volume of RACHS-1 cases. Simulations estimated the impact of regionalization of care to high volume hospitals.

Results: Data from 26 available US states demonstrated 97 total hospitals performing CHS during the study period. Median volume of RACHS-1 cases was 102 cases/yr (range 1–797). Of the 97 programs, 19% performed ≤20 cases/yr. Texas had the largest number of programs (N = 13), followed by Florida (N = 10) and New York (N = 10), many of which were located in close proximity to one-another. Using these states as examples, regionalizing care from CHS hospitals with ≤20 cases/yr to those performing ≥100 cases/yr resulted in a reduction in the number of hospitals in Texas from 15 to 5, in Florida from 10 to 7, and New York from 10 to 6.

Conclusions: Many US CHS hospitals are small volume programs located in close proximity to one another. Ongoing analyses will further evaluate the potential impact of regionalization on the number of programs, outcomes, and travel burden.

OPEN IN VIEWER

Table O-29

Variable	Odds Ratio	95% Confidence Interval	P-value
Gender	1.15	0.55-2.41	0.9651
Age at Surgery	0.18	0.07-0.43	<0.0012
Prematurity	2.89	0.81-10.36	0.1391
Obstruction	1.76	0.82-3.75	0.0051
Vein Pattern			0.531
2+2 vs. 3+1	1.46	0.65-3.25
Bizarre vs. 3+1	0.50	0.15 -1.71
Associated Anomalies			<0.0011
Complex 1 Vent vs. Simple/None	1.12	0.13-9.59
Complex 2 Vent vs. Simple/None	5.70	0.90-36.32
Heterotaxy vs. Simple/None	6.92	2.82-16.99

¹Pearson test, ²Wilcoxon test.

¹CardioStart International, Tampa, USA, ²Federal University of Maranhao, Cardiac surgery, Sao Luis, BRAZIL, ³Oregon Health & Sciences University, Cardiac Surgery, Portland, USA, ⁴Golden Jubilee National Hospital, Cardiac Anesthesia, Glasgow, UNITED KINGDOM, ⁵University College Hospital, Cardiac Anesthesia, Lagos, NIGERIA

Introduction: Setting up or advancing new pediatric heart programs in countries with severely restricted healthcare systems can be very perilous. During early surgical program development, priority is often given to sicker patients with late-presenting and advanced cardiac disease. Equipment and personnel issues, the team’s physical/emotional resilience and budget restrictions each create enormous pressures. We attempted to reduce the impact of un-anticipated complications and/or failure with simple “Dry Run” rehearsal strategies, applicable to any evolving unit.

Methods: Two simple Form Sets were developed, grouping routine, pediatric, peri-operative management into seven categories: these assess essential equipment, personnel standards; consider “cost-consciousness” throughout, and allow the new surgical team to prepare to a uniform state of readiness. On completing the clinical program’s preparation prior to the first surgical cut, a three-part Performance Scoring System (PSS) Form is introduced (Pre-operative, Discharge from Intensive Care, and Discharge home). For each patient the local team applies its own internal self-audit of performance standards (scored) over the first year of activity.

Results: These “Dry Run” and PSS Sets, successfully used in 9 international pediatric heart surgery missions, are now available in four languages. They have helped establish firm and reproducible platforms for the emergence of safe, peri-operative management. There have been no procedural lapses identified using this approach.

Conclusion: The development of a new pediatric and adult surgical program is an exciting but daunting journey, desirably involving highly expert team participation. These check-list tools may help reduce morbidity-mortality, unfair assessments, or medico-legal intrusion during a program’s early growth.

¹Johns Hopkins University School of Medicine, Baltimore, USA, ²Lurie Childrens Hospital, Chicago, USA, ³Columbia University, New York, USA, ⁴Mayo Clinic, Rochester, USA, ⁵Hospital for Sick Children, Toronto, USA

Objectives: Vascular rings in adults have been reported as either case reports or small institutional series, describing predominantly pediatric cohorts.

Methods: We reviewed the experience of five North American institutions with adult patients (age >18) undergoing surgical intervention for vascular rings.

Results: Ninety-nine adult patients undergoing surgical treatment of vascular rings over a 32-year period were identified. Seven cases were reoperations. There were no mortalities, complications were rare, and no patient required reintervention.

Conclusions: Vascular rings in adults are rare, and their surgical treatment yields excellent outcomes with low morbidity and mortality.

¹University of Iowa, Iowa City, IA, USA, ²University of Missouri, Columbia, MO, USA, ³Weis Center for Research; Geisinger Clinic, Danville, PA, USA

Objectives: Our goal is the development of surgical models that accurately phenocopy human congenital heart disease (CHD) as a means towards improving surgical outcomes. The use of Homo sapiens as the sole surgical training model is suboptimal for both patients and surgeons. We sought to develop relevant models for surgical practice using Sus Scrofa (pig) using both genetic and teratogenic approaches. Mice deficient for Islet1 (Isl1) have severe cardiac defects, and genetic variants of ISL1 confer susceptibility to human CHD. Thus, we chose to mutate ISL1; generating piglets with CHD. Retinoic Acid (RA) is a well-established teratogen; we determined the timing and dose required to create a range of CHDs in pigs modeling human CHD.

Methods: We used CRISPR/Cas mediated genome editing of ISL1 to create a mutant allele. Somatic cell nuclear transfer generated ISL1 deficient piglets. We performed a dose response curve to maternal administration of RA to generate piglets with CHD.

Results: ISL1 deficient pig hearts examined by a combination of gross dissection and computed tomography revealed double outlet right ventricle and right ventricular hypoplasia. Depending on the timing and dose of RA administered to pregnant sows, we produced double outlet right ventricle, persistent truncus arteriosus, transposition of the great arteries and pulmonary stenosis, and a variety of ventricular septal defects.

Conclusions: We have created the first genetic and teratogenic large animal models of surgically relevant CHD. These models provide myriad opportunities for surgical training, and for preclinical and translational applications to improve outcomes for patients with CHD.

¹Leiden University Medical Center, Leiden, Netherlands, ²Hospital La Paz, Madrid, Spain, ³Ospedale Papa Giovanni XXIII, Bergamo, Italy, ⁴Hospital Virgen del Rocio, Sevilla, Spain

Objective: To analyse outcomes of the original Nikaidoh technique.

Methods: Thirteen non-consecutive Nikaidoh procedures were performed for TGA/VSD/LVOTO. VSD’s were subpulmonary (6), doubly committed (2), outlet and inlet (1), non-committed (1) or outlet and very small (3) and multiple in 3. Mitral or tricuspid straddling occurred in 4. Coronary anatomy was 1LCx-2R (11) or 1L-2CxR (2). Average age was 27 (9-36) months. Twelve patients had 18 previous procedures. In all but one the aortic root remained attached under the LCA and was clockwise rotated into the LVOT after transecting the outlet septum. The aorta was never opened and coronary arteries remained in situ. The RCA was always mobilized. Six VSD’s were augmented, sometimes to great extent. Additional VSD’s were suture closed. Chordal translocation corrected AV-valve straddling. A bovine jugular vein graft (14-22 mm’s) was wrapped around the aorta, right-sided (10) or left- sided (3). Mean aortic clamp time was 125 (80-197) minutes.

Results: Further mobilization of the RCA was once needed to prevent RV ischemia. No mortality occurred. One patient was reoperated for endocarditis of the right-sided conduit. Residual VSD’s or AV-valve dysfunction did not occur. At last follow-up aortic insufficiency was absent or trivial in all. Bovine jugular vein obstruction was not observed.

Conclusions: Leaving the aorta intact and partly attached under the LCA facilitates posterior translocation and preserves aortic valve function. There is no need for aortic transection, Lecompte manoeuvre or coronary artery detachment. Wrapping the RVOT conduit around the aorta does not result in dysfunction.

¹Department of Surgery, University of Nebraska Medical Center, Omaha, Nebraska, USA

Repair of persistent truncus arteriosus with interrupted aortic arch is typically carried out at deep hypothermic temperature with some combination of selective cerebral perfusion and circulatory arrest. This video demonstrates the repair of this lesion in a newborn girl, at 32 deg C, with continuous full-flow cardiopulmonary bypass to the upper and lower body achieved through simultaneous cannulation of the innominate artery and the descending aorta above the diaphragm.

The author has previously shown the Society the performance of the Norwood operation at mild hypothermia, incorporating the descending aortic cannulation. In this video, the versatility of this cannulation and perfusion technique is demonstrated. Special emphasis is given to the easy exposure and secure cannulation of the descending aorta. Using the same cannulation technique, all operations involving reconstruction of the aortic arch can be performed. Hypoperfusion of the kidney and other abdominal organs is obviated; this patient was non-oliguric in the day following surgery and achieved negative fluid balance in the subsequent day. Potentially deleterious effects of profound cooling, e.g. on coagulation, are avoided. In addition to red cells in the bypass prime, this patient received a platelet transfusion, but no other blood products were required. Immediate chest closure is routine.

Aortic arch reconstruction with dual, innominate artery and descending aortic arterial cannulation and whole-body, continuous, full-flow cardiopulmonary bypass is a potentially important contribution in the continuing refinement of surgical techniques and the reduction of morbidity resulting from the most complex neonatal operations.

¹Center for Pediatric Palliative Care, Dept. of Woman and Child’s Health, University of Padova, Padova, ITALY, ²Pediatric Cardiology Unit, Dept. of Woman and Child’s Health, University of Padova, Padova, ITALY, ³Pediatric and Congenital Cardiac Surgery Unit, Dept. of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, ITALY

Objectives: Despite improvement in medical and surgical management, complex congenital heart disease (CHD) still presents consistent mortality. We report our experience in evaluating end-of-life (EOL) patterns of care and parental and caregivers’ perceptions.

Methods: This is a retrospective medical record review and a cross-sectional survey of cardiac surgeons, cardiologists, psychologists and parents of children who died in a tertiary care pediatric hospital between 2009 and 2012. Parents and all caregivers were asked to fill a questionnaire. Excluded were patients older than 18 years, absence of consent, and legal litigations.

Results: Among 32 patients, 28 cases were included. Ninety-one percent of deaths occurred before 1 year of age. Median length of terminal hospital stay was 23 days (range 1-130 d). Seventeen patients (53%) had univentricular heart. Thirty patients (94%) died in intensive care unit, 97% were ventilated, and 78% were on ECMO. Twenty-one patients (65.6%) experienced multi-organ failure (MOF). Only 23% of parents were present at the time of death. All (10/10) physicians and 18 of 19 contacted families responded to the survey. Parents reported feeling unprepared for the way their child died (72%), similar to caregivers’ perceptions. While 45% of parents realized the imminent death only in the last hours, 60% of caregivers were aware of approaching death about 3 days before death occurred.

Conclusion: Children with complex CHD frequently succumb in the intensive care setting with MOF and highly technical care. Both parents and caregivers perceive suffering at EOL. Caregivers have a precocious perception of death when compared to parents.

¹Pediatric and Congenital Cardiac Surgery Unit, Padua, ITALY, ²Pediatric Cardiology Unit, Padua, ITALY

Objectives: The interest in preserving pulmonary valve (PV) function has stimulated a few surgeons to apply valve-sparing techniques in repairing tetralogy of Fallot (TOF), with the aim of preserving long-term right ventricular function.

Methods: Since June 2007, we embarked into a program for preserving PV function in selected patients during TOF repair.

Results: We were able to preserve the PV in 50/86 consecutive patients (58%) who underwent repair of TOF (ranging from 21% during the initial 2 years of our experience to 100% during the last 3 years). At the beginning, together with our standard early transatrial/transpulmonary repair an intra-operative balloon dilation of the PV annulus was performed in selected patients with milder forms of TOF. Progressively we treated more severe forms (PV Z-score ≥-3), by combining additional PV plasty maneuvers, including the delamination plasty, to PV balloon dilatation. The delamination PV plasty is based on achieving more leaflet tissue for coaptation by delaminating with a fine scalpel the base of each PV cusp at the hinge point, occasionally extending the delamination into the RV myocardium. Subsequently, the extended cusps are re-suspended for creating a new PV commissure thus achieving an acceptable PV competence (Video 1-2). When leaflet tissue is lacking for PV cusp reconstruction, small triangles of prosthetic patch material can be added for extending the leaflets.

Conclusions: We believe that the preservation of the PV function during early repair of TOF, by combining different intra-operative surgical maneuvers, can be extended to almost all patients with classic TOF.

¹Department of Cardiac Surgery, Ukrainian Childrens Cardiac Center, Kyiv, UKRAINE, ²Department of Perfusiology, Ukrainian Childrens Cardiac Center, Kyiv, UKRAINE

Objective: We reviewed our experience with primary repair of truncus arteriosus, with focus on right ventricle-to-pulmonary artery (RV-PA) connection reconstruction approach.

Methods: We retrospectively reviewed a series of 87 consecutive patients, operated on between 1997 and 2016. The median age at operation was 1.3 months (range, 2 hours - 10 months) and the median weight was 3.9 kg (range, 2.4-5.9 kg). Patients cohort was subdivided into two groups and consisted of early patients managed with different technical approaches used for RV-PA outflow reconstruction: direct connection, pericardial tube, mono-, bicuspid Gore-Tex® conduits and allografts (n = 40, 1997-2007) and later patients originally managed intraoperatively with handmade trileaflet Gore-Tex® conduit (n = 47, 2008-16). Competency of these conduits was confirmed by echocardiographic assessment.

Results: Between 1997 and 2007 hospital mortality was 45%, and between 2008 and 2016, it dropped to 2.1% (p<0.001). There were five late deaths in the study with overall survival of 93% (95% CI 99.1 – 86.8) at 2 years and beyond. Hospital survivors were followed from 2.3 months to 14.2 years (mean 4.7± 2.2 years). The 1- and 10- year overall freedom from RVOT reoperation was 98% and 78%, respectively. The overall freedom from truncal valve reoperation was 95% and 94% at 1 and 10 years, respectively.

Conclusions: Since 2008 the use of handmade trileaflet Gore-Tex® conduit for RV-PA reconstruction for truncus arteriosus has become an option of choice in our Center with good early and late outcomes.

¹Department of Paediatric Cardiac Surgery - East Midlands Congenital Heart Centre, Leicester, UNITED KINGDOM, ²Department of Paediatric Cardiology - East Midlands Congenital Heart Centre, Leicester, UNITED KINGDOM

Objectives: The use of 3D modelling improves pre-operative understanding of anatomy and surgical decision-making in congenital cardio-thoracic surgery.

Methods: A series of patients with complex or unusual pre-operative anatomy was identified. MRI and CT scans datasets were optimised using Osiris (Pixmeo, Switzerland). 3-D interactive models were created using Mimics8 and 3-matic (Materialise NV, Belgium). Models were assessed and discussed at multidisciplinary team and surgical/interventional plan established in each case.

Results: This new technology was used in various cardio-thoracic situations, including:

Long-segment tracheal stenosis.

Conclusions: The use of 3D modelling, by providing a better understanding of the cardiac and airways anatomy, improves the decision-making process to choose the best surgical/interventional plan.

¹Queensland Paediatric Cardiac Services, Brisbane, AUSTRALIA, ²UQ School of Medicine, University of Queensland Brisbane, AUSTRALIA, ³Mater Medical Research Institute, Brisbane, AUSTRALIA, ⁴Cardiac Surgery, John Hopkins All Childrens Hospital, Florida, USA

Objective: To assess the mid-term performance of tissue-engineered bovine pericardium (CardioCel™, Admedus, Fremantle, Australia) for repair of congenital heart defects.

Methods: Between October 2012 and November 2014, CardioCel™ was implanted in 131 patients (135 procedures, 194 implants). Applications included closure of septal defects (98, 50.5%), repair of pulmonary arteries (62, 31.9%), intra-atrial/intra-ventricular baffles (10, 5.1%), repair of systemic arteries (15, 7.7%), valve repair (5, 2.5%), repair of systemic veins (3, 1.5%). There were 16 (11.8%) neonates, 76 (56.3%) infants and 43 (31.9%) children > 365 days. Instances were simple septation in 99 (51%) and more complex three-dimensional reconstruction in 95 (49%). Follow up is 98.5% complete (median duration 529 days) with 6 deaths (4.6%), none directly related to CardioCel™.

Results: CardioCel™ implants failed in 9 patients (6.7%). 8 patients have required 11 reinterventions (5 catheter, 6 surgical) primarily related to CardioCel™. Mechanism of failure was related to neo-intima formation in 8 patients and thrombosis in 1 patient. There was no echocardiographic, radiological or macroscopic evidence of calcification in 17 reoperations for unrelated indications. The incidence of failure in neonates was 18.7%, compared to infants (6.6%) and children (2.3%) (p = 0.14). Incidence of failure was higher when CardioCel was used for indications other than simple septation [9/95 (9.4%) versus 0/99, p = 0.001].

Conclusion: CardioCel™ has acceptable mid-term results when used for repair of congenital heart defects. It appears to perform better when used as a simple patch. Caution is recommended in neonates.

¹Arnold Palmer Hospaital for Children, Orlando, USA

Objective: Bilateral bidirectional Glenn (b-BDG) anastomosis is technically more challenging and has been associated with greater degree of thrombosis and poor outcome. We devised a technique in which we banded the non-dominant superior vena cava (SVC) in preparation for a unilateral bidirectional Glenn (u-BDG) in patients with bilateral SVC and a diminutive bridging vein at birth.

Methods: From 2005 to 2015, 135 patients underwent BDG operation with 19 eligible for b-BDG. Since late 2014 we have modified our approach to performing BDG in select patients with bilateral SVC. Two patients with bilateral SVC and a diminutive (1-2 mm) bridging vein that were to undergo single ventricle palliation were identified by preoperative echocardiography. At first stage palliation, the non-dominant SVC was banded to 2 mm cephalad to the azygous/hemiazygous branch. Serial echocardiography and cardiac catheterization were performed prior to the Glenn anastomosis.

Results: By catheterization, both bridging veins were 5-6 mm in size within 3-4 months, but required balloon dilatation of stenotic segments. The patients underwent u-BDG with ligation and division of the banded SVC for their second stage palliation at 4 and 6 months of age. They were discharged in 6-7 days with saturations of 80% or greater.

Conclusions: Bilateral BDG anastomosis has been associated with stagnation of flow and increased risk of thrombosis and poor outcome. Non-dominant SVC banding in patients with a diminutive bridging vein allows for enlargement of the bridging vein in preparation for performing a u-BDG. It simplifies the technique and avoids the flow disturbances complicating b-BDG operation.

¹Childrens Memorial Health Institute, Warsaw, POLAND, ²University Medical Center Groningen, Groningen, NETHERLANDS, ³Athens Heart Surgery Institute, Athens, GREECE, ⁴Johns Hopkins All Childrens Heart Institute, St. Petersburg, USA, ⁵Hospital de Santa Marta, Lisbon, PORTUGAL

Objective: Patterns of treatment and early outcomes in children with Single Ventricle (SV) vary between units and countries. The aim of the study was to analyze the changes of outcomes that occurred during the last 15 years.

Methods: All children who underwent heart surgery between 1999 and 2015 with one of the diagnoses related to SV were included. 30 day and hospital mortality in all patients, age groups and associated with specific procedures were analyzed. Differences between two study periods (1999 – 2008, 2009 – 2015) were tested. The proportions of SV procedures in 6 countries (Australia, Belgium, France, Germany, Netherlands, Poland) were compared.

Results: 12,486 SV patients (6.98% of all) underwent 23,316 heart procedures (10.54% of all) with 30 day mortality 15.71%. In 5239 neonates 30 day mortality was 22.20%. In 5186 infants 30 day mortality was 8.93% and in children (6172) – 4.91%. Between two study periods 30 day mortality in neonates with SV decreased from 22.95% to 21.54% (p = 0.02), after Norwood (28.56 to 22.16%, p = 0.00004), after Glenn (3.95 to 2.77%, p = 0.03), after Fontan (4.3 to 3.05%, p = 0.01). The proportion of SV patients within the total of Congenital Heart Surgery varies between countries (6.8 to 12.8%), in neonates (9.74 to 27.54%), Norwoods (2.7 to 12.7%).

Conclusions: The proportion of children and neonates with SV varies significantly between countries. Early outcomes of CHS in children with functionally SV improved between two study periods, especially after Norwood procedure in neonates. The diagnosis of functionally SV remains associated with high risk of early mortality.

¹University Medical Center Groningen, Groningen, NETHERLANDS

Objective: The Du Bois and Du Bois formula for Body Surface Area (BSA) was published in June 1916 and is still the most widely used of several formulas for BSA. These formulas differ mainly in the balance between height and weight. The derivation of the formula is reconstructed and the appropriateness of its usage in the era of obesity is assessed.

Methods: As the raw data on their 9 subjects were published in the 1916 paper the formula’s derivation was reconstructed. The Du Bois’ formula was compared with all other available formulas for BSA. Its applicability in obese patients was assessed theoretically on the basis of perfusion rate of fatty tissue.

Results: Reconstruction of the formula was hampered because of limited information in the original paper on the methodology. The height and weight exponents could be reconstructed using the Du Bois methods, but current day analysis yields different exponents. The derivation of the constant could not be reproduced exactly. Comparison with other formula’s for BSA validates their basic concept of the formula’s form, but shows that era and location highly influence the formula’s parameters. Fatty tissue is much less perfused than muscle, so that normalisation by BSA overestimates cardiovascular processes and anatomical measures.

Conclusions: The Du Bois cousins deserve our deep respect for their ground breaking work and formula. The formula is less suited for children and in obesity. In children BSA is underestimated, while in obesity BSA is overestimated in its usage in normalising the circulation and relevant anatomy.

¹Indiana University, Indianapolis, USA

Objective: Scimitar syndrome is a variant of partial anomalous pulmonary venous connection (PAPVC) in which all or most of the right lung is drained by a single pulmonary vein that anomalously connects to the inferior vena cava. We illustrate a surgical approach to scimitar syndrome with direct anastomosis of the scimitar vein to the left atrium via right thoracotomy without cardiopulmonary bypass.

Case Video Summary: The patient is a seven-year-old girl who presented with pneumonia and her chest X-ray suggested scimitar syndrome. The diagnosis was confirmed by echocardiography. The scimitar anomaly was approached via an anterolateral right thoracotomy. The pericardium was opened widely posterior to the right phrenic nerve and the scimitar vein and right pulmonary artery were mobilized. All systemic collateral from the abdominal aorta was divided as it traversed the diaphragm into the right lung. The Scimitar vein was occluded and divided off the inferior vena cava using a partially occluding vascular clamp. The optimal anastomotic site on the left atrium (LA) was marked. The Scimitar vein anastomosis to the left atrium was done using running 5-0 monofilament suture. Intra-operative transesophageal echocardiography confirmed unobstructed flow of the Scimitar vein into the LA. The child made an uneventful recovery and was discharged on post-operative day 5 without complications.

Conclusion: This right thoracotomy, off pump approach to Scimitar syndrome is safe and effective and avoids the need for intra-atrial baffles and the use of the cardiopulmonary bypass. We have utilized this approached successful in more than 30 adults and children over the last 30 years.

¹University Hospital Leuven - Department of Cardiac Surgery, Leuven, BELGIUM, ²University Hospital Leuven - Department of Paediatric Cardiology, Leuven, BELGIUM

Objectives: Pulmonary artery banding continues to play an important therapeutic role in the management of congenital heart defects. Banding with a traditional non-stretchable band is straightforward and cheap, but carries the risk of surgical removal and re-banding if the band becomes too tight. We have developed a system of handmade dilatable pulmonary artery bands. We sought to describe our experience with this procedure.

Methods: We retrospectively reviewed all patients who had undergone dilatable pulmonary artery banding at our institution between 2003 and 2015. A non-resorbable nylon cord of 2 mm is placed around the main pulmonary artery and the ends are sutured together at the desired diameter. The ends of the cord are then folded back and fixed with two to four vascular clips, thus incorporating a stepwise dilatable restriction.

Results: A dilatable pulmonary artery band was placed in 14 patients at a median age of 3.7 weeks. Surgical repair could be successfully postponed (2 to 19 months) in seven children following progressive dilatation by balloon angioplasty. The VSD either closed spontaneously or became haemodynamically insignificant during progressive dilatation in six children, allowing for full dilatation and percutaneous release of the band, completely avoiding intracardiac surgery. One child who developed chronic respiratory insufficiency died 2 months after angioplasty.

Conclusions: We have developed a straightforward and cheap dilatable main pulmonary artery band. Dilatation of this dilatable pulmonary artery band is possible, effective and safe and allows to postpone or avoid intracardiac surgery.

¹Paediatric and Congenital Cardiac Surgery and Cardiology, Ospedali Riuniti, Ancona, ITALY

Objectives: The quest for the ideal valved conduit for right ventricular outflow tract (RVOT) reconstruction is still ongoing. In the last years, many new products have been proposed to surgeons ensuring good durability, good long-term functionality and good human compatibility. Herein we describe our experience with the Biopulmonic™ conduit (BioIntegral Surgical, ON, Canada).

Methods: In the last year, nine patients (range 1-58 years) received a Biopulmonic™ conduit for the RVOT reconstruction. Seven patients had previous conduit failure (4 presented regurgitation, 2 stenosis and 2 mixed lesions) and one patient had a previous palliation for a Tetralogy of Fallot.

Results: There was no operative mortality; neither major morbidity. The immediate post-operative echocardiographic assessment evidenced a good hemodynamic performance and a mean peak gradient of 21 mmHg with no regurgitation. At a mean follow-up of 6 months the mean peak gradient increased to 35 mmHg. Two patients (2 and 4 months follow-up) required conduit replacement for early severe re-stenosis. They developed a very thick fibrous tissue outside the conduit, causing extrinsic compression. An histologic specimen found the tissue to be of flogistic origin. One patient (10 months post-op) is currently in follow-up with a rapidly increasing gradient. In the others, the functionality of the conduit is currently satisfying.

Conclusions: The ideal pulmonary valved conduit should be durable, hemostatic and theoretically should increase in size with the patient. In the last year we used the Biopulmonic™ conduit with this aim. However early results have been discouraging. Although in some patients the conduit proved to be satisfactory, in three a severe early failure was observed.

¹Children’s Hospital of Georgia / Medical Collge of Georgia, Augusta, USA

Objective: Favorable management of atrial dysrhythmias and accelerated myocardial failure after atriopulmonary connection (APC) Fontan has been achieved with satisfactory early and mid-term outcomes with the conversion to total cavopulmonary connection (TCPC) and tailored arrhythmia surgery. Feasibility of a customized approach for patients with double-inlet single ventricle (DISV) using TCPC conversion with in-situ extracardiac conduit Fontan(iECF) and systemic venous atrial exclusion(SVAE) was assessed.

Methods: Between March 2013 and February 2016 8 consecutive Fontan conversions to TCPC with tailored arrhythmias surgery [right-sided Cox-maze cryoablation (CMC) for atrial flutter (AF) and biatrial for atrial fibrillation (Afib)] and dual-chamber pacemaker system were reviewed. Three (of 8); (group-A) had DISV where iECF and SVAE was used under beating heart normothermic cardiopulmonary bypass. All had NYHA class III or IV and refractory dysrhythmias. Patients with protein-losing enteropathy (PLE) were excluded.

Results: Mean age at APC Fontan and TCPC conversion was 5.7+/-2.2 and 33.8+/- 4.9 years (group-A) versus 6.01+/-1.8 and 32.5+/-5.6 years (group-B) respectively (p>0.05).All group-A patients had AF and received right-sided CMC versus three (of 5) for group-B (p> 0.05). There were no early deaths or 30-day readmissions for cardiac etiology in both groups. Chest tube removal occurred 6.4+/-2.0 (group-A) postoperatively versus 9.6+/-3.0 (group-B) (p<0.05). Intensive care unit (ICU) stay was 7.5+/-1.6 (group-A) versus 10.8+/-2.5(group-B) days (p<0.05). There were no atrial dysrhythmias at hospital discharge. Median follow-up was 16 months (IQR 2-27 months). There were no late deaths or heart transplantation. All patients maintained NYHA class I or II. None from group-A and 1 of group-B had recurrence of atrial dysrhythmia.

Conclusions: TCPC conversion with iECF, SVAE and tailored arrhythmia surgery in DISV is reproducible with favorable impact on mortality and quality indicators and might be associated with accelerated time to drain removal and ICU recovery.

¹BC Children’s Hospital, Department of Cardiothoracic Surgery, Vancouver, CANADA

Objectives: Successful use of the Berlin Heart EXCOR™ (Berlin Heart GmbH, Berlin, Germany) (BH™) ventricular assist device (VAD) as a bridge-to-transplant modality in pediatric patients has been demonstrated in recent years. Certain anatomic scenarios, such as hypoplastic left heart syndrome (HLHS) and non-compaction cardiomyopathy, can pose technical challenges for this type of VAD support. We have developed an alternative surgical technique that can overcome these challenges.

Methods: We report the cases of two infant patients who were successfully supported to heart transplantation after total cardiectomy and BH biventricular ventricular assist device (BiVAD) placement.

Results: A 5-day-old female child with HLHS (severe mitral stenosis and aortic atresia) and significant coronary sinusoids underwent a Norwood procedure with Sano modification. Postoperatively, she required ECMO support due to a massive sinusoidal shunt and could not be weaned. As a bridge to heart transplant, she underwent a cardiectomy and BH BiVAD insertion, utilizing reconstructed atrial cuffs for the inflow cannulae. She was successfully bridged to heart transplant 6 days later. A 4-month-old girl diagnosed with end stage idiopathic dilated cardiomyopathy underwent BiVAD insertion due to severe biventricular dysfunction. Her cardiac enlargement and hypertrophy made VAD cannulation impossible. A cardiectomy was performed and BH BiVADs were inserted. She underwent heart transplantation after 105 days of BiVAD support.

Conclusion: Utilization of the BH™ as a total artificial heart by performing a recipient cardiectomy can be successfully employed in certain infant with challenging anatomy.

¹TEH-Tube scientific coordinator, New York, United States Minor Outlying Islands, ²INSERM U970, PARCC & Laboratoire de Recherches Biochirurgicales, Hôpital Européen Georges Pompidou, Paris, FRANCE, ³UCL School of Pharmacy, Department of Electrospinning, University College London, London, UNITED KINGDOM, ⁴Statice SAS Besançon, FRANCE, ⁵Rescoll Bordeaux, FRANCE, ⁶Celyad SA Mont-Saint-Guibert, BELGIUM, ⁷HZG Berlin, GERMANY, ⁸APHP, Hôpital Saint-Louis, Laboratory of Cell Therapy; University Paris Diderot, Paris, FRANCE, ⁹APHP, Hôpital Européen Georges Pompidou, Department of Cardiovascular Surgery, Paris, FRANCE

Objective: Conduits currently used to reconstruct the right ventricular outflow tract have no growth potential and require reoperations. The TEH-TUBE program aims at creating an innovative tissue-engineered bioresorbable polymeric and biofunctionnalized valved tube.

Methods: This seven-partners project was funded 4,500,000€ by the European Union’s Seventh Framework Programme (GA N604049). It includes 3 steps: 1) to compare different electrospun bioresorbable polymers to generate the device; 2) to compare different types of biofunctionnalization; 3) to validate its growth and regenerative potential in a growing lamb model.

Results: The in vivo evaluation of polydioxanone and polyurethane stem cell-seeded or RGD-biofunctionalized patches in 84 rats confirmed the concept that appropriately peptide-functionalized polymers can be successfully substituted for cell-loaded materials. An advanced design of the valved tube was obtained using finite elements theory and computer-assisted designing techniques. The use of a rotating collector, specifically-designed toolings and optimized electrospinning parameters allowed for the generation of a second generation medical-grade polymeric valved tube with a highly anisotropic microstructure and an excellent reproducibility. Electrospun tubes possessed elastomeric mechanical properties close to the native heart valve. Functionalization methods using gelatin, RGD or plasma technology were optimized to improve the biocompatibility of the device. These results were obtained within a tightly controlled regulatory framework to ensure that the final product meets the current requirements for clinical studies.

Conclusion: These mid-term promising results of the TEH-TUBE program pave the way for a device that should decrease the risk of reoperative surgeries, improve the quality of life and ultimately decrease healthcare costs.

¹All India Institute of Medical Sciences, New Delhi, INDIA, ² Institute of Genetic Medicine, Newcastle University, Newcastle-upon-Tyne, UNITED KINGDOM

Background: The arterial switch operation (ASO) has evolved as the treatment of choice

for infants with transposition. The procedure is technically challenging when the aorta is left-sided, and requires surgical expertise and precision for a good surgical outcome. We review here our experience with such patients.

Methods: We carried out the arterial switch procedure in 20 patients, seen between January, 2002, and October, 2013, with usual atrial arrangement diagnosed as transposition on the basis of concordant atrioventricular and discordant ventriculo-arterial connections, but with left-sided and anterior aorta. Hospital records were analyzed for segmental anatomy, associated cardiac anomalies, the coronary arterial patterns, aortic cross clamp and cardiopulmonary bypass times, and specific surgical techniques used for transfer of the coronary arteries and reconstruction of great arteries. We recorded the outcomes in terms of in hospital survival and left ventricular function at most recent follow up.

Result: Age at the time of operation ranged from 3 days to 18 months, with a median of 75 days, and weight ranged from 3 kgs to 8.8 kgs, with a median of 3.85 kgs. All patients survived the operation. We did not perform the Lecompte maneuver in 9 patients. Mean cardiopulmonary bypass time was 157.5± 24.9 minutes, with a median of 161 minutes; mean aortic cross-clamp time was 101.2±23.8 minutes, with a median of 102 minutes. One patient died on the second postoperative day due to sudden onset of ventricular fibrillation. Follow-up ranged from 23 to 31 months, with a mean of 28.04±2.32 months, and a median of 28.4 months. At last follow-up, all 19 survivors are in NYHA class I, and none require cardiac medications. Serial echocardiograms have revealed normal biventricular function and no residual defects.

Conclusion: With appropriate technical modifications, patients with usual atrial arrangement, concordant atrioventricular, and discordant ventriculo-arterial connections with left-sided aorta can undergo successful anatomical repair.

¹Department of Cardiovascular Sciences, Cardiac Surgery Division, Mother Theresa Universitary Hospital, Tirana, ALBANIA, ²Department Of Pediatrics, Mother Theresa Universitary Hospital, Tirana, ALBANIA

Objectives: The aim of this study is to evaluate the outcome in a series of patients with Ebstein anomaly undergoing a new technique of tricuspid valve (TV) repair.

Materials and methods: Between May 2006 and May 2015, 11 consecutive patients with 2D-echo diagnosis of Ebstein anomaly underwent TV repair in association to a bidirectional cavopulmonary shunt. The mean age was 16.5 years. The most frequently found symptoms were cyanosis, dyspnea and arrhythmias. Our new peacock tail technique consisted in a total detachment of both the anterior and posterior TV leaflets and their rotation towards the septum and re-implanting onto the true annulus. The rudimentary septal leaflet was left untouched in its previous location.

Results: The hospital mortality was 9% (1 patient). TV repair was successful in 10 patients. At mean follow-up of 5.8±2.4 years, the indexed RV diameter was 26.4±7.2 mm/m² versus 38.4±8.7 mm/m² preoperatively (p = 0.003) and the indexed right RA volume was 109±37 cm³/m² significantly reduced versus the preoperative value 202±58 cm³/m² (p < 0.001). The postoperative mean TV regurgitation grade was 1.2±0.42 versus 3.9±0.3 preoperatively (p < 0.001) and postoperative indexed TV area was 1.6±0.6 cm²/m² versus 2.7±1 cm²/m² preoperatively (p=0.008). Mean NYHA, SaO2 and cardiothoracic ratio were significantly improved.

Conclusions: The peacock tail technique, is a new surgical approach to Ebstein anomaly which includes a novel type of valvuloplasty. Our strategy for these challenging patients has yielded a high rate of native TV preservation, a low incidence of recurrent regurgitation, an improved functional status and right ventricular function, and marked resolution of symptoms.

¹Joe DiMaggio Children’s Hospital, Hollywood, USA

Objectives: Since 2008 our institutional protocol for right ventricular outflow tract (RVOT) reconstruction has been an aggressive valve sparing approach. In those patients in whom the valve cannot be spared or is absent, an extracellular matrix monocusp or bicuspid valve is fashioned. The purpose of the study was to examine the fate of extracellular matrix valve reconstruction in the right ventricular outflow tract.

Methods: A retrospective chart review of all patients undergoing extracellular matrix valve creation in the right ventricular outflow tract was undertaken. Data from the most recent follow-up echocardiogram available is presented.

Results: Twenty two patients underwent extracellular matrix valve reconstruction in the right ventricular outflow tract from 2008 to 2016. Mean age at operation was 312 days (range 6-1999 days). Mean weight was 7.1 kg (range 2.7 – 13.7 kg). There were 20 monocusp valves, and 2 bicuspid valves. One patient underwent removal of his monocusp valve and replacement at another institution. Sixteen patients had follow-up echoes available. Mean follow up interval was 33 months (range 0.33-85.67). RVOT gradient was mild in 15, and moderate in one. Valvar insufficiency was trivial in 3, mild in 6, moderate in 2 and severe in 5 patients. Bicuspid valves had only mild insufficiency.

Conclusions: In patients requiring RVOT reconstruction, valvar reconstruction with extracellular matrix is feasible. Most patients have only mild residual gradients with less than moderate insufficiency. Bicuspid valve reconstruction may be preferable, with less insufficiency over the long-term. Long-term follow up is necessary to assess these results over time.

¹University of Wisconsin-Madison, Department of Surgery, Madison, USA

Median time-to-first readmission was 21 (IQR: 12-58) days. Median HLOS at readmission was 2 (IQR: 1-8) days. The 1-, 3-, 6- and 12-month freedom from first readmission was 98%, 96%, 95% and 88% respectively (Figure-1). There was no significant difference in survival between readmitted and non-readmitted patients (p = 0.68). Time-to-readmission and readmission frequency didn’t impact patient survival (p > 0.05).

OPEN IN VIEWER

Figure 1.

Kaplan-Meier plot demonstrating freedom from first unplanned readmission following CHS.

Conclusions: The first year after discharge remains a risk period for readmissions after congenital heart surgery (CHS). Viral infections were the leading cause of unplanned readmissions. Readmissions didn’t impact long-term survival of patients after CHS.

¹Yale University School of Medicine-Children’s Medical Center New Haven USA, ²Connecticut Children’s Medical Center Hartford, USA, ³Children’s Hospital Association Kansas City, USA

Background: Red blood cell transfusion is very common practice in pediatric cardiac surgery as the result of hemodilution, hemolysis, and defective hemostasis inherent to cardiopulmonary bypass. Transfusion of red blood cell has been shown to be associated with increase in morbidity in pediatric patients undergoing cardiac surgery. There is a vast variability in practice of blood utilization within and across different practices. The aim of this study is to demonstrate current variability and a decade trend in practice of blood usage amongst children’s hospitals performing pediatric cardiac surgery.

Methods & Results: A retrospective observational cohort study using the Pediatric Health Information System database from 43 participating pediatric hospitals in the United States was performed. All discharge billing for patients less than 18 years of age who underwent cardiac surgery using cardiopulmonary bypass between 2005 and 2015 was interrogated. There were ten index diagnoses and procedures which were investigated and analyzed based on age differences and the Society of Thoracic Surgeon & European Association for Cardio-Thoracic Surgery mortality scores. Our main outcome variable was the unit(s) of homologous red blood cell charged for the first 24-hour of the cardiac surgery. There was significant variability in current red blood cell usage for a given diagnosis and procedural code across all the centers despite age ranges and complexity scores. There was a vast variability in blood usage practice for different procedural codes with no discernable changes in the practice across all the participating centers for the last decade.

Conclusions: There is a major variability in current practice of blood utilization for a given procedure despite age and complexity with no changes in practice pattern for the last decade of pediatric cardiac surgery. The increasing costs, risk, and variability associated with the use of homologous red blood cell should mandate blood conservation practice as a quality indicator in pediatric cardiac surgery.

¹Childrens Memorial Health Institute, Warsaw, POLAND, ²Athens Heart Surgery Institute, Athens, GREECE, ³University Medical Center Groningen, Groningen, NETHERLANDS, ⁴Johns Hopkins All Childrens Heart Institute, St. Petersburg, USA, ⁵Hospital de Santa Marta, Lisbon, PORTUGAL

Objective: The early results of Congenital Heart Surgery (CHS) in neonates remain a challenge. Further improvements require identification of risk factors associated with outcomes. The aim of this study was to define risk factors of early mortality and morbidity in two study periods.

Methods: All neonates undergoing CHS at centers participating in the ECHSA Database were included. Only primary procedures stratified using STAT mortality score were analyzed. Patients below 2.5 kg at PDA ligation were excluded. The study time frame was divided into: 1999 to 2008 and 2009 to 2015.

Results: The dataset consists of 29289 procedures performed between 1999 and 2015. 13285 and 15286 procedures were performed in two study periods respectively. Overall hospital mortality was 12.16%; 12.96% and 11.5% in first and second period respectively, p < 0.001. Postoperative major complications occurred in 10.27% with no difference between periods (p = 0.58). Multivariate logistic regression showed that lower body weight, higher STAT mortality score, univentricular anatomy, non-cardiac abnormalities, gprf and CPB time were the risk factors for hospital death. The onset of major complications was associated with lower body weight, higher STS morbidity score (period 2), univentricular anatomy, non-cardiac abnormalities, gprf, and CPB time (period 2). Mortality for Norwood, ASO, IAA significantly decreased (p < 0.0005). There were no differences in mortality after ASO+VSD, TAPVC, CoA and B-T shunt. Mortality associated with PA banding increased from 16.10% to 18.99% (p < 0.0001)

Conclusions: In neonatal CHS the risk of hospital death decreased in the last years. Risk factors for hospital death and major complications remained unchanged.

¹Chukyo Hospital, Nagoya, JAPAN

Objectives: Aortic translocation procedure now gained more attention to expand the limitation of biventricular repair for Transposition of the Great Arteries (TGA) with left ventricular outflow tract obstruction (LVOTO). The issue of this complex procedure is technically demanding. One of the difficult parts is coronary translocation. We now used aortic free wall patch plasty for optimal repositioning of coronary artery.

Methods: A 9-month old boy with the diagnosis of TGA with LVOTO underwent anatomical correction. Median sternotomy was performed followed by initiation of cardiopulmonary bypass with bicaval cannulation. After the cardiac arrest, the ascending aorta was transected and the aortic root was harvested from the right ventricle, and the coronary artery buttons harvested from the aorta. The Lecompte maneuver was then performed. The harvested aortic root was rotated by 180 degrees and reimplanted into the pulmonary annulus. Coronary artery buttons were reimplanted in the hole of previous coronary button orifice. Aortic free wall patch was supplemented to place the coronary artery to optimal position to prevent kinking. An ePTFE patch was used for ventricular septal defect closure and reconstruction of the left and right ventricular out flow tract.

Results: The patient was weaned from the ventilator on third postoperative day, and discharged home on 14th postoperative day uneventfully. Postoperative coronary angiogram showed the non-obstructed coronary flow.

Conclusions: Aortic translocation provides excellent postoperative hemodynamics. Aortic free wall patch plasty for repositioning of coronary artery may be a good option to prevent coronary kinking and ease the technical difficulty of this procedure.

¹Children’s Hospital of Georgia / Medical College of Georgia, Augusta, USA

Objective: Surgical pulmonary valve insertion (SPVI) for re-entry right ventricular outflow tract intervention (rRVOTI) remains the gold standard. Introduction of transcatheter pulmonary valve insertion (TPVI) and a customized concept in patients undergoing rRVOTI as part of a comprehensive valve program at the Children’s Hospital of Georgia Heart Center targets early intensive care unit (ICU) and hospital discharge (Hd). Outcome analysis was undertaken.

Methods: From September 2014 to December 2015, 23 consecutive patients under 18 years of age who underwent rRVOTI [TPVI (percutaneous, hybrid) or SPVI (valve-only or conduit-valve)] were encountered. For hybrid TPVI small subxiphoidal access to the right ventricle was used. When SPVI was chosen, beating heart normothermic cardiopulmonary bypass (CPB) was deployed except for those with residual intracardiac lesions. Patients (1) mechanically ventilated preoperatively, and (2) neonates were excluded. All had preoperative advanced imaging. Intraoperative lung recruitment strategies, use of alpha-2 adrenergic-receptor agonist (Dexmedetomidine), early ICU mobilization and mediastinal drain removal were part of a standardized approach.

Results: All patients were intraoperatively extubated with no postoperative reintubation. Sixteen had SPVI (11 valve-only/5 conduit-valve) and 7 TPVI (6 percutaneous/1 hybrid). Mean age was 7.1+/−4.2 for SPVI versus 15.9+/−3.6 years for TPVI (p < 0.05). Mean CPB was 47.3+/−24.5 minutes. There was no early mortality or 30-day readmission for both groups. Early mobilization occurred within 6 hours after arrival in the ICU in all but 2(86%) patients. Mediastinal drain removal in SPVI occurred in 0.9+/-1.0 days. ICU length of stay was 1.2+/-1.1 for SPVI versus 1.0+/-1.3 days for TPVI (p > 0.05). Fifteen (94%) SPVI and 6(86%) TPVI had Hd in less than 48 hours (p > 0.05). Age, number of prior sternal reentry, CPB time, preexisting major risk factors (cardiac, chromosomal, neurodevelopmental) did not influence outcome.

Conclusion: Patients undergoing RVOTI can be safely stratified, based on a customized concept, towards SPVI or TPVI. SPVI, although it is delivered in younger patients, is associated with comparable to TPVI early outcomes and time to Hd irrespective of patient’s or procedural characteristics. Preoperative “roadmap” imaging, intraoperative lung recruitment strategies, standardized quality analgesia, early ICU mobilization and mediastinal drain removal can facilitate this concept.

¹Joe DiMaggio Children’s Hospital, Hollywood, USA

Objectives: The aim of this study was to determine the destiny of residual ventricular septal defects (rVSD) found on post-operative echocardiogram when detected in the immediate post-operative period.

Methods: Patients undergoing surgical repair of VSD with available intra-operative and discharge echocardiograms were reviewed. Presence of rVSD at discharge triggered review of the most recent follow up echocardiogram.

Results: One hundred VSD were analyzed. The average patient age was 783 days (rVSD = 538 days versus 956 days for no residual) with a mean weight of 9.5 kg (8.75 kg rVSD versus 10.1 kg) and BSA of 0.41 m² (rVSD = 0.38 versus 0.44). rVSD was present in 40% of patients on initial intra-operative echocardiogram with a mean size on TEE of 2.2 mm. The mean bypass time was 71 minutes (no VSD) versus 96 mins (rVSD), cross clamp time was 44 and 61 minutes, respectively. The average length of stay for patients with rVSD was 20.1 days, and no VSD was 7.5 days. At mean follow up of 27 months, 10% of patients (10/100) had persistent rVSD, but in patients with greater than 6 months of follow up, only 3 patients had rVSD.

Conclusions: Residual VSD after surgical repair is detected frequently on post-operative echocardiogram. Most rVSD are less than 4 mm in size. 97% of patients with greater than 6 months follow up are free from any rVSD. Residual VSD was associated with longer bypass and cross clamp times, younger age and lower weight. The presence of residual defect was associated with increased length of stay.

¹Arnold Palmer Hospital for Children, Orlando, USA

Objective: The aim of this study was to evaluate the outcome measures following the use of del Nido cardioplegia (dN) when compared with conventional multi-dose high-potassium cardioplegia (non-dN) in pediatric patients at a single center.

Methods: We retrospectively analyzed data in patients, less than one year of age, undergoing CPB from 1/2012 to 8/2015. We changed our cardioplegia protocol to dN administered in a single or infrequently-dosed strategy in 9/2013. The outcomes of the dN group (n=107) are compared with non-dN group (n=118). We analyzed variables for demographic, intraoperative, early postoperative (24-hours) and discharge, using the 2-tailed unpaired t test and Fisher’s exact test. Linear regression determined significant associations with the dependent variable.

Results: The two groups were similar in age, sex, weight, height, CPB and cross-clamp time, pre and postoperative echocardiographic systolic function, length-of-stay and mortality. The STAT category was significantly higher in the dN group (p=0.03). The cardioplegia dosing interval was lower for the non-dN group (non-dN, 28+7 min, vs dN, 68+23 min, p<0.001). The volume and doses of cardioplegia per patient were significantly higher in the non-dN group (p<0.001). The first 24-hours postoperative urine output was higher in the dN group (p<0.001) with no difference in the incidence of diuretic use (p=0.07). Linear regression showed belonging to dN group was the only variable significantly associated with higher first 24-hours urine output (p<0.001).

Conclusions: The single or infrequently-dosed del Nido cardioplegia with extended ischemic interval can be used safely and effectively in the pediatric cardiac surgical patients. The use of del Nido is associated with a significantly higher renal output with no significant difference in diuretic use between the two groups in the early postoperative period.

¹CardioStart International, Tampa, USA, ²Federal University of Maranhao, Sao Luis, BRAZIL, ³University of Kathmandu, Dhulikhel, NEPAL

Purpose: We sought outcome evaluation in building a previously well-planned, new pediatric and adult heart program during two 7.8/7.4 R earthquakes which devastated the entire Kathmandu region of Nepal (April/May 2015) causing more than 8800 deaths, 16,000 with severe initial injury, and thousands more with exacerbation of pre-existing conditions. Fifty international teams focused on trauma-related management, but most left within the week following the first quake.

Methods: To address the major, ethical, practical, personal challenges, the immediate and long-term issues for the new surgical program, our international medical team provided:

Specialist trauma assistance;

Results:

Fast-tracking of pediatric program building and ICU management skills evolved, by including adult surgical development simultaneously;

Conclusion: Pediatric cardiac program development emerged with positive hospital and rural outcomes following this natural disaster, despite the challenges of trauma management. Our team returned in November, 2015, and successfully launched the cardiac surgery program without mortality.

¹Paediatric and Congenital Cardiac Surgery and Cardiology, Ospedali Riuniti, Ancona, ITALY

Objective: The origin of the coronary arteries (CA) from the wrong aortic sinus, is a rare, under diagnosed defect, susceptible to sudden death. The surgical repair is often recommended and has to be tailored according to the anatomical pattern of presentation.

Methods: Seven patients (range 3.5-62 years) presented to our Institution with an anomalous origin of a CA and inter-arterial course. The diagnosis was made by echocardiography and confirmed using CT scan or MRI. Three patients had a CA reimplantation (single CA anomaly), 1 had a CA unroofing (intramural course), 1 had a pulmonary artery translocation (single CA ostium in the right aortic sinus) and one had a patch enlargement of the right coronary ostium. One young athlete arrived with a cardiac arrest and despite the use of the ECMO, the patient had a brain death.

Results: Among surgical patients there was no operative or late mortality. Evaluation of the results was accomplished with Echocardiography, MRI and ECG stress test. None of the patients but one (pulmonary artery translocation) presented recurrent signs or symptoms of heart ischemia.

Comment: Origin of the CA from the wrong sinus is a rare anomaly which carries a high risk when associated to an inter-arterial course. Various techniques have been described for the repair of the anomaly. We are far from believing that one technique is superior to the others, we suggest instead to tailor the surgical correction on the anatomical pattern. Results are satisfactory and surgery carries a low risk.

¹Athens Heart Surgery Institute, Athens, GREECE, ²Childrens Memorial Health Institute, Warsaw, POLAND, ³University Medical Center Groningen, Groningen, NETHERLANDS, ⁴Johns Hopkins All Childrens Heart Institute, St. Petersburg, USA, ⁵Hospital de Santa Marta, Lisbon, PORTUGAL

Objective: The arterial switch operation (ASO) is the gold standard for treatment of Transposition of the Great Arteries (TGA), associated with low mortality and morbidity. The aim of the study was to identify risk factors for early mortality and morbidity after ASO during two study periods.

Methods: All neonates listed in the ECHSA Congenital Database with TGA or TGA and Ventricular Septal Defect (VSD) who underwent ASO were included. The study was divided into two time periods, 1999 – 2008 and 2009 – 2015.

Results: The dataset consists of 5543 ASO and 1698 ASO/VSD repairs performed between 1999 and 2015. Hospital mortality (HM) after ASO was 5.12% and decreased from 5.97% to 4.38% between the two study periods (p=0.007). HM after ASO/VSD repair was 9.01% (8.79% and 9.20% in two study eras, NS). ASO/VSD repair caries significantly higher HM (p<0.001). Multivariate logistic regression showed that for isolated ASO, general preoperative risk factors (gprf), lower body weight, younger age and CPB time were the risk factors for mortality in the first period; in the second, only gprf, body weight and CPB time. For ASO/VSD repair only weight and CPB time were risk factors in first era, and CPB time in the second. Postoperative major complications occurred in 8.06% after ASO and in 14.19% (p< 0.001) after ASO/VSD repair, without differences between study periods.

Conclusions: ASO is associated with lower hospital mortality and morbidity than ASO/VSD repair. Hospital mortality after ASO decreased between the two study periods, but not after ASO/VSD repair.

¹Childrens Hospital of Georgia/Medical College of Georgia, Augusta, USA

Objective: Correction of a high partial anomalous pulmonary venous (PV) connection to the superior vena cava (SVC) may be complicated by conduction system disturbance, SVC or PV obstruction. A combined in-situ cavoatrial autologous reconstruction and atrial appendage advancement flap (CARAF) after distal SVC division was used when the anomalous PV connection entered at or adjacent to the junction between SVC and innominate vein. Mid-term term outcomes were reviewed.

Methods: Between March 2013 and Feb 2016, 5 patients with PV connection to SVC remotely from atriocaval junction underwent CARAF. Median age was 8.3 (IQR, 4.2 to 38.5) years. Four (of 5) (80%) had an atrial septal defect. None had persistent left SVC. Follow-up was complete in all patients (median 1.9; IQR 0.9-3.1 years)

Results: No early or late deaths occurred. No early or late reoperation for SVC or PV obstruction was encountered. No dysrhythmias or sinus node dysfunction occurred in the perioperative period. All patients remained in sinus rhythm at last follow-up.

Conclusions: CARAF technique appears to be effective and reproducible with no associated morbidity in cases where conventional strategies might carry risk for conduction system disturbance and caval or pulmonary venous obstruction.

¹TU, Manmohan Cardiothoracic Vascular and Transplant center, Kathmandu, NEPAL

Background: Atrial septal defect (ASD) is one of the most common congenital cardiac disorder requiring intervention. Here, we compare cost effectiveness of minimally invasive approach (group A) and median sternotomy (group B) for ASD closure.

Methods: The preoperative variables, intraoperative data and postoperative outcomes of patients of group A and group B were collected and analyzed.

Results: Between May 2014 to September 2015 we performed minimally invasive closure of ASD in 25 patients. In the same period we performed traditional median sternotomy in 25 patients for ASD closure. We compare duration of ICU and hospital stay. In Group A: There were 16 females and 9 males. The mean age at surgery was 23.4 years. The mean size of incision was 4.9 cm. Average CPB time was 44 minutes and cross-clamp time was 26.0 minutes. The mean length of stay in ICU was 1.8 days (range 1–3) days, and the length of stay in the hospital was 4.5 (range 4–7) days. In Group B: There were 14 females and 11 males. The mean age at surgery was 29.1 years. Average CPB time was 38.5 minutes and cross-clamp time was 22.7 minutes. The mean length of stay in ICU was 1.9 days (range 1–4) days, and the length of stay in the hospital was 6.8 (range 4–12) days.

Conclusion: ASD closure with mini invasive approach is safe and effective with very few perioperative complications, with shorter ICU and total hospital stay which is cost saving for our country.

¹Pediatric Cardiac Surgery, Morgan Stanley Childrens Hospital of New York- Presbyterian, Columbia University, New York, United States, Minor Outlying Islands, ²Pediatric Cardiology, Morgan Stanley Childrens Hospital of New York-Presbyterian, Columbia University New York United States, Minor Outlying Islands

Objectives: To present the case of a complex mitral valve reconstruction in a patient with a destructive endocarditis.

Methods: A 14 year-old girl presented in septic shock with a history of a recent Dilation & Curettage procedure. A Staphylococcus Aureus mitral and tricuspid endocarditis was diagnosed, complicated with multiple septic emboli to the brain, liver, kidneys and lungs. Preoperative echocardiography showed a severe mitral regurgitation, mild to moderate tricuspid regurgitation, mitral and tricuspid valvular vegetations and dilated left cavities. We performed an extensive mitral valve repair, through a mini-invasive sternotomy approach.

Results: Intraoperative inspection showed a complete destruction of A3, P3 with multiple ruptured chordea. An aggressive debridement of all infected tissue (remnants of A3, P3, part of A2, and posteromedial aspect of the annulus) was performed. The posteromedial parts of the free edges of A2 and P2 with intact chordea were sutured together and the defect corresponding to A3-P3 was closed with a pericardial patch. The annulus was reconstructed using a strip of autologous pericardium. Given the dilation of the mitral annulus, we also performed a ring annuloplasty. Tricuspid valve vegectomy and commissuroplasty were performed. Intraoperative echo showed good ventricular function, no residual MR, no significant MS and trace TR. Patient was discharged without complications 4 days later. Follow up echo at 3 months showed good function, trace MR, a mild MS (mean gradient 4 mmHg), trace TR.

Conclusions: We present the case of a successful complex mitral valve reconstruction in a patient with a destructive endocarditis.

¹Hannover Medical School - Cardiothoracic, Transplant, and Vascular Surgery, Hannover, GERMANY, ²Hannover Medical School - Pediatric Cardiology and Pediatric Intensive Care, Hannover, GERMANY

Objectives: Patients with complex congenital heart disease often require multiple re-operations during life, resulting in increased operative morbidity and mortality especially in double-valve replacement. Decellularized homograft valves (DHV) have shown reduced reoperation rates when used for pulmonary valve replacement (PVR) compared to current other valve substitutes and very auspicious early results in aortic valve replacement (AVR). DHV was approved by the competent authorities for PVR (in 2013) and AVR (2015).

Methods: Prospective follow-up of all patients, who received single-stage double semilunar valve replacement using DHV.

Results: Four patients underwent DHV double semilunar valve replacement since 2011 at our institution, 2 following Ross-procedure (31 y male, 38 y female) and 2 after repair of Truncus arteriosus communis (11 and 16 y female). Ross patients had fewer preceding valve procedures (3 and 1) than Truncus patients (5 and 4). There was no perioperative mortality despite challenging operations (bypass 373±114, x-clamp 224±79 min), 3 patients were extubated within 24 hours, one on day 2. During follow-up up good semilunar valve and biventricular heart function was present in all 4 patients, NYHA functional class was I for all at latest follow up (mean 14±20.2 months, range 5-41). Peak echocardiographic aortic DHV gradient was 11±2.5 mmHg and 14±5.9 mmHg for the DHV pulmonary valve. Valvular regurgitation was below I° for all DHV.

Conclusions: Single-stage DHV double semilunar valve replacement showed very promising early results in these four very complex cases and constitutes an additional surgical option after multiple preceding valve procedures in young patients.