World Journal for Pediatric and Congenital Heart Surgery

Background: Infants undergoing repair or palliation of congenital heart disease are at risk of renal insufficiency. Development of renal insufficiency increases mortality. This project seeks to determine whether intra- and postoperative renal near-infrared spectroscopy (NIRS) monitoring can reliably predict renal insufficiency after cardiac surgery in infants. Methods: In this prospective, observational cohort study 48 patients undergoing repair or palliation of congenital heart disease in the first 6 months of life were studied intraoperatively and on postoperative day 1 and 2. The NIRS mean and nadir were recorded for the 3 time periods, as were urine output, fluid balance, and serum creatinine. Renal insufficiency was defined as rise in creatinine ≥40% from baseline or oliguria for >4 hours. Near-infrared spectroscopy data were compared to creatinine increase, oliguria, and fluid balance on postoperative day 0, 1, and 2 by regression analysis. Results: Mean renal regional saturation on postoperative day 1 has a strong correlation with increase in creatinine (P < .001 and R ² = .6). Mean renal saturation less than 80% predicts renal insufficiency with a sensitivity of 100% and a specificity of 75% (P < .001). Conclusion: Monitoring of intra- and postoperative renal regional saturation may provide an early, noninvasive marker of renal insufficiency after cardiac surgery in infants. This would be clinically significant if interventions to improve renal regional saturation prevent renal insufficiency.

Objective: Repair of congenital heart defects involving the right ventricular outflow tract (RVOT) may require pulmonary valve replacement at the time of primary repair or reoperation. This study compares the outcomes of bovine jugular vein grafts (BJV, Contegra, Medtronic Inc.) with cryopreserved pulmonary homografts (PHs) in patients with RVOT obstruction at a single institution. Methods: We reviewed the outcomes of all BJVs and PHs implanted for RVOT reconstruction from 1999 to 2010. Echocardiographic data were reviewed to evaluate valve performance. Graft dysfunction is defined as RVOT obstruction with peak echo-Doppler gradient >40 mm Hg and/or grade III/IV conduit valve regurgitation. Graft failure is defined as need for conduit replacement or need for catheter or surgical reintervention. Results: A total of 216 patients who received BJVs (n = 153) and PHs (n = 63) were studied. There was no significant difference between the groups with respect to mean age, body surface area, conduit indication, or conduit diameter, though mean follow-up duration was longer in patients that received homografts. Conduit dysfunction and conduit failure and need for explantation were worse for homografts, albeit at longer follow-up interval. Distal stenosis and actuarial survival were similar. Conclusions: In the first ten years after pulmonary implantation of BJVs and PHs, survival and freedom from distal stenosis are statistically similar, but freedom from failure, dysfunction, and explantation are significantly better for BJV conduits. The BJV conduit is a good alternative in patients who require RVOT reconstruction.

Background: Early establishment of a two-coronary artery system has become the standard surgical approach in patients with anomalous origin of the left coronary artery from the pulmonary artery. After surgical repair, presentation of severely impaired ventricular function and mitral regurgitation is a common finding. Methods: We reviewed midterm outcome of 18 consecutive patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) undergoing surgical repair for establishment of dual coronary system operated on between September 1999 and July 2009. Mortality, morbidity, echocardiography assessment of left ventricular function, mitral valve regurgitation, and indications for reoperation were studied retrospectively. Results: The mean age at the time of surgery was 26 ± 44 months (range, 14 days-12.7 years), 14 patients were younger than 6 months. There was no in-hospital or late mortality. Recovery of left ventricular function was associated with improvement in the degree of mitral valve regurgitation. At the latest follow-up, mitral valve regurgitation was none or trace in 14 patients (78%), mild to moderate in 3 patients (16%), and remained severe in 1 patient (6%). Left ventricular function normalized in 16 patients and remained mildly impaired in 2 patients. Late postoperative echocardiograms demonstrated a patent left coronary artery in 17 patients. In 5 patients temporary left heart bypass (LHB) was needed. Conclusions: Early establishment of a 2-coronary artery system artery results in complete recovery of left ventricular function and without relevant mitral valve dysfunction. Reoperation rates are acceptable. Our results support the use of LHB in patients with refractory low cardiac output. The need of mechanical circulatory support was short and very effective in our patient cohort.

Background: The optimal management strategy for neonates with Ebstein's anomaly is unknown. This analysis was undertaken to assess current trends in the management and prognosis of neonates born with Ebstein's anomaly in the United States, as reflected in an administrative database. Methods: The Pediatric Health Information System database (40 children’s hospitals) was used to review the reported incidence and available data on neonates with Ebstein's anomaly treated in the United States between 2003 and 2007. Primary outcome was hospital survival. Of the 415 patients identified, 257 (62%) did not undergo initial surgical intervention as neonates. Aortopulmonary shunt only was done on 63 patients (15%), single-ventricle palliation on 36 (9%), two-ventricle repair on 16 (4%), heart transplantation in 3 (1%), and a catheter-based intervention or a hybrid palliative approach was applied in 40 (10%). Intergroup comparisons were done using chi-square analyses. Results: Mortality for the entire cohort was 24% (100 of 415). For medically treated patients, this was 22% (56 of 257). For surgically treated and hybrid patients, this was 30% and 23%, respectively (P = NS). Conclusions: The majority of patients born with Ebstein's anomaly currently do not undergo surgical intervention as neonates. Significant early mortality in this group suggests that certain subsets of patients may benefit from earlier surgical intervention. Among the severely symptomatic neonates who do undergo early surgical intervention, the mortality remains high, irrespective of the surgical approach taken. A multicenter trial may be appropriate to identify strategies to optimize care for these critically ill neonates. Further analysis of risk factors for early mortality is warranted.

Background. The purpose of this study was to evaluate the surgical outcomes and pulmonary artery (PA) development associated with a new strategy wherein the modified Norwood (N) procedure is performed at 1-2 months after bilateral pulmonary artery banding (PAB). Methods. Between January 2008 and February 2010, 16 patients underwent Norwood-type operation after previous bilateral PAB. For analysis, patients were divided into two groups. Group I (n = 11) underwent modified Norwood procedure with either right modified Blalock Taussig (RMBT) shunt (n = 4) or right ventricle to pulmonary artery (RV-PA) conduit (n = 7). Group II (n = 5) underwent Norwood procedure plus bidirectional Glenn anastomosis. Diagnoses were hypoplastic left heart syndrome in 6 and its variants in 10. Results. There was no surgical death and no late death. Pulmonary artery interventions were performed at the time of the Norwood procedure in 27% in Group I and in 100% in Group II (p < 0.05). Additional PA interventions were performed during the period of follow-up in 4 cases in Group I (36.4%), and in 4 cases in Group II (80.0%). Additional Blalock Taussig shunts were performed in 7 patients, resulting in significant increase in PA index. In all, four patients have reached total cavopulmonary connection, and one has undergone biventricular repair. Eight patients in Group I and one patient in II Group reached bidirectional Glenn anastomosis. In Gp II, two patients showed LPA narrowing or obstruction with PA index of 80 ± 12 mm²/m². Conclusions. Regarding the second-stage palliation after bilateral PAB, modified Norwood procedure with either RMBT or RV-PA conduit has some advantages compared with Norwood plus BDG with respect to subsequent pulmonary artery development. Additional BT shunt may contribute to PA development, even in the patients with Norwood procedure with RV-PA conduit.

Children born today with congenital heart disease (CHD) are likely to reach adulthood, even those with complex disease. As survival rates have increased, attention has focused on the longer-term morbidity associated with CHD and its treatment, but this has largely addressed the physical and physiological outcomes rather than psychosocial morbidity and health-related quality of life (QoL). The purpose of this article is to outline the arguments in favor of routine evaluation of health-related QoL, describe how such measurements might be used, and the barriers and challenges associated with the collection of the data. Finally, a strategy is suggested for the routine collection and use of health-related QoL data with children and adolescents with CHD.

Although interventions to improve outcomes for children with congenital heart disease may be designed and tested, the rarity of any one specific defect presents a barrier to using traditional statistical methods to measure the effects of these interventions. The purpose of this report is to describe the innovative statistical approach taken by the Joint Council on Congenital Heart Disease (JCCHD) National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) to measure outcomes for infants with hypoplastic left heart syndrome—a relatively rare disease. We report our experience with the application of statistical process control methods to generate measures capable of identifying statistically significant change in the incidence of early growth failure—a clinically important outcome in this relatively small patient population.

Anesthesia practitioners have long been at the forefront of patient safety initiatives in the operating room and beyond. The Congenital Cardiac Anesthesia Society has partnered with the Society of Thoracic Surgeons Congenital Heart Surgery Database to develop a patient registry for patients with congenital heart defects in order to determine patient outcomes related to anesthesia in this high-risk population. A review of existing database efforts is also undertaken to determine their strengths and weaknesses.

We review our 16-year experience using the large, multi-institutional database of the University HealthSystem Consortium to study management and outcomes in congenital heart surgery for hypoplastic left heart syndrome, transposition of the great arteries, and neonatal coarctation. The advantages, limitations, and use of administrative databases by others to study congenital heart surgery are reviewed.

Congenital heart disease affects 0.8% of all live-born infants. Some of the malformed hearts can at best be palliated by conventional surgical or catheter interventions from the start. Others fail slowly from chronic overloading. Patients with congenital heart disease have been among the first transplant recipients since 1967. Primary therapy with infant heart transplant is a convincing concept from an immunological perspective but large-scale implementation is limited by donor organ shortages. Another growing area is rescue therapy for older patients with end-stage heart failure after palliative procedures, particularly those with single-ventricle hearts, systemic right ventricles, and associated arrhythmias.

Ventilating a child or newborn in the postoperative course after repair of congenital heart disease requires a solid basic understanding of respiratory system mechanics (pressure–volume relationship of the respiratory system and the concept of its time constants) and cardiopulmonary physiology. Furthermore, careful attention has to be paid to avoid damaging the lungs by potentially injurious mechanical ventilation. Optimizing ventilator settings during controlled and assisted ventilation, allowing as early as possible for spontaneous ventilation by still assisting mechanically the patient’s respiratory efforts are important features for lung protection, for minimizing potential hemodynamic side effects of positive pressure ventilation, and for early weaning from mechanical ventilation. In the search for being less invasive, the use of noninvasive ventilation in the cardiac intensive care setting is rapidly increasing despite still lacking evidence of its theoretical superiority and requires good knowledge of specific techniques and equipment available for this approach in this setting. This review will address many of these aspects and highlight the essentials to be known when ventilating a child in the Cardiac Intensive Care Unit (CICU).

Tremendous progress has been made in the science of assessing the outcomes of the treatments of patients with pediatric and congenital cardiac disease. Multi-institutional databases have been developed that span subspecialty, geographic, and temporal boundaries. Linking of different databases enables additional analyses not possible using the individual data sets alone and can facilitate quality improvement initiatives. Measures of quality can be developed, in the domains of structure, process, and outcome, which can facilitate quality improvement. Parents are an integral part of the health care team and are key partners with regard to quality improvement. The role of the parent in the process of health care delivery can be facilitated by enhancing the organizational culture and creating methods of transparency, empowering parents, and implementing effective strategies of communication. The professionals caring for patients with pediatric and congenital cardiac disease, in collaboration with the patients and their families, now have the opportunity to capitalize on the power of our databases and move beyond outcome assessment and benchmarking, to collaborative quality improvement.

A novel temporary aortopulmonary shunt, constructed between the aorta and main pulmonary artery with flexible cannulas, was used to facilitate right ventricular outflow tract reconstruction in one neonate and creation of a central aortopulmonary shunt in a second neonate. Although cardiopulmonary bypass is readily available in Turkey, the strategy described in the case report may prove especially useful in developing nations with limited access to cardiopulmonary bypass (CPB).

Heterotopic heart transplant (HHT) has traditionally been thought of as creating 2 parallel circulations. We present a case of using the donor heart as a “biologic left ventricular assist” (bio-LVA). The heterotopic technique used consisted of 4 anastomoses: the donor heart pulmonary artery (PA) to the native heart right atrium, the superior vena cava to superior vena cava, the left atrium to left atrium, and the aorta to aorta. A 9-year-old boy with restrictive cardiomyopathy, a PA pressure of 85/53 mmHg, received a HHT because he would probably not be able to tolerate an orthotopic heart transplant secondary to elevated PA pressure. He is currently alive 14 years post-transplantation.

Jacobsen syndrome (JS) is a rare chromosomal anomaly caused by deletions in the distal long arm of chromosome 11. Features of the syndrome include growth and developmental delays, a distinctive facial appearance, and a variety of physical problems including heart defects and bleeding disorders. Congenital heart defects occur in approximately 50% of children with JS. Hypoplastic left heart syndrome (HLHS) has been occasionally reported in association with JS. In such cases, the hematological abnormalities may influence the outcome from single-ventricle palliation through staged surgical reconstruction. Thrombotic obstruction or occlusion of the modified Blalock-Taussig (BT) shunt is a well-documented cause of interstage mortality following the Norwood operation. Although there is no consensus regarding the therapeutic value of antiplatelet therapy during the interstage period following the first stage of palliation, maintenance of shunt patency is critically important. For patients with JS undergoing single-ventricle palliation, decisions regarding antiplatelet therapy during the interstage period may be further complicated by the presence of thrombocytopenia and platelet dysfunction related to JS. We report the case of a patient with HLHS, JS, and thrombocytopenia who underwent the Norwood procedure, and we describe our strategy for prophylaxis against thrombosis of the BT shunt.

Left ventricular (LV) pseudoaneurysm is rare in children. This report describes the case of a 2-year-old previously healthy girl who was diagnosed with endocarditis and underwent resection of a large mitral valve vegetation from the posterior mitral leaflet. One month after this surgery, she was diagnosed with a large LV pseudoaneurysm based on echocardiography performed during routine outpatient follow-up. Cardiac surgery was performed urgently to address the pseudoaneurysm, given its potential to rupture spontaneously. This case emphasizes the need for a close follow-up of children with endocarditis for development of pseudoaneurysms, which is a rare but potentially fatal complication of endocarditis.

A one-stage repair was planned for an 11-year-old boy with tetralogy of Fallot. After initial attempts to wean from cardiopulmonary bypass were unsuccessful, an atrial septal defect and a ventricular septal defect were created in order to achieve hemodynamic stability. The boy recovered from the operation but had large volume of chest drainage. Transthoracic echocardiography revealed left to right shunting at both atrial and ventricular levels. Interventional catheter-directed devices were used to repair the residual shunts successfully in the catheterization laboratory.

Patients with vasoplegic syndrome (VPS) in the post-cardiopulmonary bypass setting usually require escalating vasopressor support. The utilization of methylene blue (MB) in the treatment of VPS in the adult population has been well described. We present a 5-year-old girl who developed vasodilatory shock due to VPS that was resistant to escalating doses of adrenergic agonists following cardiac transplantation. After receiving 1 mg/kg of MB, there was a significant improvement in the patient’s mean arterial pressure which allowed for progressive weaning of the vasopressor support. To date, there are limited data regarding the use of MB in pediatric patients with VPS following cardiothoracic surgery. The cellular mechanisms of MB in VPS are discussed and reports of its use in the adult and pediatric population are reviewed. Dosing regimens and potential adverse effects of MB are presented.

A 9-year-old boy with congenital atresia of the left main coronary artery underwent myocardial revascularization. Coarctation of the aorta and ventricular septal defect were diagnosed at the age of 1 year. At age 7 years, the child presented with syncope while exercising. Preoperative evaluation included cardiac catheterization which revealed the unexpected finding of congenital atresia of the left main coronary artery with origin of the circumflex artery from the right coronary artery. Surgical correction included myocardial revascularization by means of left internal mammary artery graft to the anterior descending coronary artery, coarctation resection, and ventricular septal defect repair. The patient recovered uneventfully. We report the details of this extremely rare case with successful concomitant surgical management of the congenital coronary artery anomaly and the associated structural heart disease.

From 2004 to 2008, 64 infants with large ventricular septal defects were managed using a two-stage surgical approach. In the first stage, banding of the main pulmonary trunk was performed. The defect was then repaired months later. Debanding of pulmonary artery was accomplished without the need for pulmonary artery reconstruction.

We present a newborn patient who had surgical correction of supracardiac total anomalous pulmonary venous connection repair with an unusual cause of residual postoperative pulmonary venous obstruction secondary to compression from the transesophageal echocardiography (TEE) probe.