Staged Biventricular Repair After Hybrid Procedure in High-Risk Neonates and Infants

Abstract

Background:

Single-stage biventricular repair remains a challenging and difficult decision in high-risk newborns and early infants with the presence of left ventricular outflow tract obstruction (LVOTO) or borderline hypoplasia of the left ventricle (LV).

Methods:

Six high-risk patients underwent the initial hybrid procedure (bilateral pulmonary banding + ductal stenting) for staged biventricular repair. Their median age was 17 days (range: 7-55 days). The diagnosis was interrupted aortic arch (IAA), ventricular septal defect (VSD), and LVOTO (n = 3); IAA and VSD (n = 1); and aortic annular hypoplasia, aortic arch hypoplasia, VSD, and LVOTO (n = 1). The last patient had borderline LV with large atrial septal defect (ASD) and aortic arch hypoplasia. The patient with borderline LV had also ASD closure with small fenestration.

Results:

One patient died of sepsis after the hybrid procedure. Other patients underwent biventricular repair 8 to 13 months later. Three patients had conventional repair with conal septum resection. The other patient with IAA, in whom LVOTO was considered nonresectable, underwent Yasui operation. The last patient with borderline LV had enough development of left heart structures during follow-up and underwent aortic arch repair. One patient who had conal septum resection died after biventricular repair. One patient needed a tracheostomy; four patients were discharged uneventfully and their clinical conditions were good on postoperative year 1.

Conclusion:

Staged biventricular repair with the initial hybrid procedure may be a feasible and safe alternative in high-risk neonates and early infants. Hybrid intervention may provide the development of cardiac structures in time and a better evaluation for the possibility of biventricular repair in borderline patients.

Keywords

hybrid procedure interrupted aortic arch left ventricular outflow tract obstruction borderline left ventricle congenital heart disease neonate

Introduction

The hybrid procedure, consisting of surgical bilateral pulmonary artery banding (bPAB) and interventional ductal stenting with or without atrial septal manipulation, introduced recently as an alternative to the Norwood stage I operation in the management of patients with hypoplastic left heart syndrome (HLHS) and left ventricular outflow tract obstruction (LVOTO). Potential advantages of this new approach are relatively noninvasive nature of the procedure, avoidance of cardiopulmonary bypass, ischemic injury, and inflammatory reaction.^1,2 Hybrid procedure minimizes the extent of initial palliative surgery and render possibility to postpone comprehensive surgery to a later stage, in which the patient is more mature and resistant to the undesirable adverse effects of long duration of cardiopulmonary bypass. Therefore, it may improve survival. Single-stage biventricular repair remains a challenging and difficult decision in newborns and early infants with the presence of LVOTO, hypoplastic aortic annulus, or borderline hypoplasia of the left ventricle (LV). Initial success of the hybrid procedure in high-risk neonates has prompted the increasing use of this approach and extended the indications in patients who are potential but borderline canditates for biventricular repair.^3
–6

In this study, we present six cases who underwent staged biventricular repair after the initial hybrid procedure as a first-step palliation.

Patients and Methods

Between January 2014 and January 2018, six consecutive patients (one boy and five girls) underwent the hybrid procedure with the intention of staged biventricular repair. This study was approved by the institutional ethics committee (ATADEK 2018-13/7). Informed consent was obtained. The age of the patients was between 7 and 55 days (median: 17 days). Their body weights were between 2,400 and 4,000 g (median: 3,000 g). The diagnosis of the four patients were interrupted aortic arch (IAA; type B, n = 3; type A, n = 1) and ventricular septal defect (VSD). Three of them had LVOTO due to posterior malalignment of the conal septum (Figure 1). Moderate aortic annular hypoplasia was also present. One of them had aberrant right subclavian artery. The fifth patient, who was 55 days old, had severe aortic arch hypoplasia, VSD, LVOTO, and aortic annular hypoplasia. The last patient had borderline hypoplasia of the LV with hypoplastic aortic and mitral valve annulus, aortic arch hypoplasia, and large atrial septal defect (ASD). No endocardial fibroelastosis was detected.

Four of the patients were referred to our center under mechanical ventilation and all patients were receiving prostaglandin E₁ infusion due to ductus-dependent systemic circulation. One of them had necrotizing enterocolitis, received intravenous total parenteral nutrition for nutritional support, and was treated with a full course of intravenous antibiotics for vancomycin-resistant Enterococcus sepsis, while being concomitantly treated with maximal medical therapy for severe pulmonary overcirculation. She had also multiple comorbidities, such as elevated international normalized ratio and persistent left lung atelectasis, accompanying with severe renal and liver insufficiency and the 22q11 deletion. Two of the patients had renal failure and high lactate levels; one needed peritoneal dialysis preoperatively.

For all of the patients, the single-stage biventricular repair was considered risky due to associated multiple comorbidities, bad general clinical condition, or uncertain morphologic characteristics for biventricular repair, and hybrid procedure was planned as first-stage palliation.

First Stage: Hybrid Procedure

Under general anesthesia, a median sternotomy was performed. The bands were fashioned by cutting a 2-mm ring from a 3, 5 or 4 mm Polytetrafluoroethylene (PTFE) tube graft (4 mm was used for 55-day-old patient). The standardized technique was used for banding as defined by Akintuerk et al, and a rise in systemic blood pressure and fall in oxygen saturation around 10-point each were considered enough for banding.⁵ No pressure measurement was performed. The patient with borderline LV additionally underwent cardiopulmonary bypass, and under a short period of cardioplegic arrest, ASD was closed with a fenestrated (4 mm) pericardial patch to promote the development of left heart structures.

A couple of days after bPAB, patients were brought to the cardiac catheterization laboratory. One patient who had sepsis preoperatively needed the long duration of prostaglandin E1 infusion. Ductal stenting was delayed until sepsis was controlled. Biplane angiography preferably was selected. After femoral venous access, an angiogram was performed and the lengths and the narrowest part of the ducts were measured. A stent is selected larger than the narrowest part of the ducts and longer than the length of the ducts. Peripheral stents were used for the stenting (Omnilink Abbot, California, USA). Stent was advanced through the femoral vein to the pulmonary artery and into the ductus arteriosus. After making sure the correct location, the stent was implanted. Echocardiographic examination was also performed to evaluate the location of the stent. After stent implantation, heparin infusion continued in 15 U/kg/h for 24 hours. Then aspirin was started 5 mg/kg in dose, up to the second operation.

All patients had successful ductal stenting, except 55-day-old patient, in whom ductal stent was not performed, because no ductal narrowing was detected despite cessation of prostaglandin E1 (PGE1) infusion. No atrial septal manipulation was needed in any of the patients, except the patient with borderline LV who had ASD closure with fenestration.

One patient who had IAA type A with multiple comorbidities died of sepsis in postoperative day 20. The remaining patients discharged uneventfully. Median intensive care unit and hospital stay after hybrid procedures were 20.5 days (range: 7-25 days) and 26 days (range 15-36 days), respectively.

Observations during interstage follow-up were unremarkable. Periodical (roughly bimonthly) echocardiographic controls confirmed nonrestrictive flow through the ductus, and mean gradient was approximately 50 to 60 mm Hg for each pulmonary artery band. One patient needed reintervention at four months due to narrowing of the ductal stent. A second ductal stent was inserted uneventfully. They have all been remained in balanced circulation with steady somatic growth. All remaining patients underwent biventricular repair 8 to 13 months after the initial palliation.

Second Stage: Biventricular Repair

All patients had cardiac catheterization and computed tomography angiography to delineate the detailed morphology before proceeding the second stage (Figures 2 and 3). Echocardiographic measurements revealed that the aortic annulus diameters increased with age. All living patients, except patient with borderline LV, had subaortic stenosis due to posterior malalignement of conal septum. Echocardiographic dimension during first and second stage is demonstrated in Table 1. The patient with borderline LV, aortic, and mitral annulus diameters increased from 4.5 mm (z = −4.3) and 6.5 mm (z = −4.1) to 7.5 mm (z = −1.5) and 9.8 mm (z = −2.8), respectively. The same patient also underwent magnetic resonance imaging study to measure LV volume and function, before proceeding to the second stage. Magnetic resonance imaging findings confirmed adequate left ventricular development (LV end-diastolic volume 72.5 mL/m², end-systolic volume 20 mL/m², and Ejection fraction (EF) 72%; Figure 4).

Table 1.

Echocardiographic Dimensions During First- and Second-Stage Procedures for the Patients With Aortic Arch Anomaly, VSD, and LVOTO.

Patients	First Stage				Second Stage
Patients	BSA (m²)	Aortic Annulus Size (mm)	Z Score	Subaortic Area Size (mm)	BSA (m²)	Aortic Annulus Size (mm)	Z Score	Subaortic Area Size (mm)
Case 1	0.18	4	−3.44	2.9	0.31	6.1	−2.90	5.0
Case 2	0.21	5	−2.79	3.8	0.41	8	−2.06	6.5
Case 4	0.19	4	−3.68	3.0	–	–	–	–
Case 5	0.19	3.2	−4.70	2.7	0.36	5.0	−4.76	4.2
Case 6	0.25	5.2	−3.16	4.1	0.35	7	−2.38	5.8

The biventricular repair was performed with cardiopulmonary bypass under moderate hypothermia (26°C). Innominate artery cannulation was performed either directly or with a PTFE graft. All patients underwent aortic arch repair with ductus and/or ductal stent resection under selective antegrade cerebral perfusion. Autologous or bovine pericardial patch augmentation was used during arch repair. Pulmonary bands were removed and pulmonary arteries were reconstructed with autologous fresh pericardial patch. In three patients, in whom obstructive conal septum were seemed resectable preoperatively, conal septum resection via transaortic approach was performed and VSD was closed with a dacron patch. A small atrial septal fenestration (4 mm) was left. One patient with small aortic annulus underwent Yasui operation with 16 mm bovine jugular vein (Contegra) conduit between the right ventricle and pulmonary artery. The last patient with borderline LV underwent aortic arch repair, ductal stent resection, and debanding with the same technique. Her atrial fenestration was already closed at the time of biventricular repair. A new atrial septal fenestration was performed under short period of cardiac arrest. One of the patients with IAA had aneurysmal dilatation of the main pulmonary artery and it was resected during repair.

Results

One patient with IAA repair and LVOTO resection needed extracorporeal membrane oxygenation support due to low cardiac output on postoperative day 1. Her echocardiogram showed unobstructed aortic arch, and pulmonary arteries, but mild-to-moderate residual obstruction of the LVOT (40 mm Hg). Despite mechanical circulatory support, the patient died with disseminated intravascular coagulation (DIC) and multiorgan failure on postoperative day 5. Patients' characteristics and outcomes are summarized in Table 2.

Table 2.

Patient Characteristics and Outcomes.

	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6
Body weight (g)	2,400	3,200	3,270	2,700	2,740	4,000
Diagnosis	IAA (type B), VSD, LVOTO	IAA (type B), VSD, LVOTO	BLV, ASD, AAH	IAA (type A), VSD	IAA (type B), VSD, LVOTO, aberrant RSCA	AAH, VSD, LVOTO
Comorbidities	Low body weight	Renal failure mechanically ventilated	Mechanically ventilated	Di George syndrome, NEC, sepsis, MOF, Mechanically ventilated	Anal atresia	Renal failure, peritoneal dialysis, mechanically ventilated
Age at bPAB (days)	13	30	13	17	15	55
Interstage follow-up time (months)	10	8	8	–	13	8
Interstage intervention	no	no	no	–	Ductal re-stent (4 months)	no
Second-stage biventricular repair	IAA repair, VSD closure, LVOTO resection	IAA repair, VSD closure, LVOTO resection	Debanding, PDA closure	–	Yasui operation	Aortic arch repair, VSD closure, LVOTO resection
Weight at biventricular repair (g)	5,600	8,000	6,900	–	6,500	6,500
Selective cerebral perfusion (minutes)	37	33	17	–	35	30
ACC time (minutes)	108	114	6	–	120	110
CPB time (minutes)	161	168	117	–	212	120
Delayed sternal closure	Yes	No	No	–	Yes	No
ECMO	Yes	–	–	–	–	–
Reintervention after biventricular repair	None	None	None	–	Distal aortic arch stenting	None
Outcome	Dead after biventricular repair	Alive	Alive	Dead after hybrid procedure	Alive	Alive

Abbreviations: AAH, aortic arch hypoplasia; ACC, aortic cross clamp; BLV, bordeline left ventricle; bPAB, bilateral pulmonary artery banding; CPB, cardiopulmonary bypass; ECMO, extracorporeal membrane oxygenation; IAA, ınterrupted aortic arch; LVOTO, left ventricle outflow tract obstruction; RSCA, right subclavian artery.

One patient needed long duration of mechanical ventilation and tracheostomy was performed. One patient had transient renal failure and mild neurological complication.

Control echocardiograms before hospital discharge showed no aortic sufficiency, unobstructed aortic arch. There was no residual branch pulmonary artery stenosis. One patient had mild residual LVOT obstruction (20 mm Hg). Their median intensive care unit and hospital stays were 27.6 days (range: 13-60) and 38 days (21-75 days), respectively. Their functional status was good and no increase in LVOT gradients was detected during echocardiographic examinations on postoperative year 1. The patient who had Yasui operation developed distal aortic arch stenosis and stent implantation was performed successfully at postoperative month 7.

Discussion

Encouraging results of the hybrid procedure in patients with HLHS has resulted in the expansion of the indications to include high-risk patients who have potential for biventricular repair, like our study group. For such patients, the initial hybrid palliation is an alternative to an extensive neonatal procedure with high risk of mortality and morbidity. For some patients, it defers the decision whether biventricular repair may be performed or not (which may be unclear in the neonatal period). The hybrid procedure avoids neonatal high-risk surgery in these patients and delays the difficult decision for biventricular repair to a later stage. Development of left heart structures with time may also facilitate the decision and enhance the possibility of biventricular repair.^7
–9 Besides, some concerns of hybrid procedure, such as retrograde aortic coarctation, ASD restriction, and risk of coronary ischemia due to lack of antegrade aortic flow, usually do not exist in this group of patients. But concerns still exist for the developmental delay of the pulmonary arteries which may complicate further operations.¹⁰

We have performed single-stage total repair for additional 12 patients with IAA (without LVOTO) at the same time period. One patient died in the early postoperative period (early mortality: 8.3%). All other patients with LVOTO underwent initial hybrid intervention, who are the patients of this article. Alsoufi et al conducted a study on 77 neonates who underwent IAA repair from 2002 to 2013. Of these, in 53 patients standard repair, in 7 patients LVOT enlargement, and in 17 patients LVOT bypass (initial Norwood [n = 14]; single-stage Yasui [n = 3]) were performed. This study reported that freedom from LVOT reoperation was lower after IAA plus a concomitant LVOT enlargement procedure than it was after standard repair or LVOT bypass, with a one-year freedom from LVOT reoperation of 84% (vs 97% for standard repair and 98% for LVOT bypass) and a five-year freedom from LVOT reoperation of 52% (vs 89% for standard repair and 93% for LVOT bypass). In this study, survival was comparable among the three surgical groups and was 90% after standard repair, 85% after IAA repair plus LVOT enlargement, and 76% after LVOT bypass procedures.¹¹

Case series have demonstrated excellent outcomes besides allowing adequate time for development and gradual adaptation of the LV and acted as a bridge to biventricular repair.^5,7
–9 There have been several reports of an increase in the rate of growth of the left heart structures documented with z-scores.^7,9 Ballard et al reported the results of seven patients with borderline left heart structures who had initial hybrid technique. According to serial echocardiograms, z-scores of left heart structures and aortic discriminant scores increased gradually and three of them could achieve an eventual biventricular repair.^7,8 Restriction of the atrial shunt is recommended in this patients to promote left heart filling and development.⁸ In our one patient with borderline LV, we closed the large ASD by leaving a 4 mm fenestration for this purpose.

Hoashi and colleagues presented their approach to three infants with persistent truncus arteriosus associated with aortic arch obstruction (Van Praagh type A4) as staged biventricular repair following bPAB. Although one patient has died from a noncardiac cause one month after the operation, the staged biventricular repair has been successfully completed in the remaining two cases.⁹ A similar report of three patients with aortic arch reconstruction and intracardiac repair following bPAB in moribund patients after birth was reported as with no postoperative neurological deficits and significantly reduced postoperative morbidity by Miyamoto et al.¹² Akintuerk et al reported recently their 15-year results for the hybrid approach in a single institute consisting of 154 patients. Forty of them eventually reached to biventricular repair with 89% survival rate.⁵

Although the future need of arch reconstruction in the presence of the ductal stent and pulmonary arterioplasty on both branch pulmonary arteries at the time of debanding may be a disadvantage of the hybrid procedure, the operative repair might be facilitated and better tolerated in a well-developed infant with a protected pulmonary vascular bed.^5,7,13

The Yasui operation can be performed either as a primary repair or as part of a staged strategy for patients with adequate-sized ventricles with VSD associated with aortic arch obstruction and LVOTO.^14,15 Yasui et al reported their results with initial palliation with bPAB. Eight of the 11 patients had Yasui operation later successfully. Hybrid intervention might be considered as simple and effective palliation to control congestive heart failure in symptomatic neonates. The same report also supports bPAB as useful, especially in patients with marginal LVOT size. Bilateral pulmonary artery banding provides delaying the decision-making for the Yasui operation or standard biventricular repair based on the time-related changes of the LV.¹⁴ Another option for these patients is making Norwood stage I operation first and completion to Yasui operation later. Kanter et al reported 21 patients who had Yasui operation. Fifteen of them had staged repair after initial Norwood procedure with no early mortality.¹⁵ Chosen primary or staged Yasui operation was usually based on clinical characteristics of the patient or surgeon preference. Those patients may be also good candidates for Norwood operation than patients with HLHS, thanks to the adequate size of ascending aorta.

Our three cases with LVOTO were seemed feasible for standard biventricular repair with the excision of the LVOTO. Although it was performed successfully, a moderate residual gradient was persisted in one patient, who died in the early postoperative period because of low cardiac output. Yasui operation could have been performed for that patient like our another patient who had Yasui operation successfully. After this experience, we consider Yasui operation more in patients with borderline aortic annulus and difficult subsets for LVOTO resection. Transpulmonary approach for LVOTO resection might be another alternative described by Luciani et al. Although we have no experience, a better view might be obtained from relatively large pulmonary annulus for conal septum resection.¹⁶

Neonatal Ross-Konno operation may be another good alternative in patients with IAA and LVOTO. Accord et al reported three neonates with good early and midterm results. But it needs long duration of cardiopulmonary bypass and may not be tolerated for critically ill neonates.¹⁷ This option may also be used as a second stage after hybrid operation, instead of Yasui procedure.

Limitations of the study are its retrospective design and the study consists of a small number of patients.

In conclusion, our experiences supports that hybrid procedure may be safe, feasible, and reproducible initial palliation alternative in neonates with the borderline condition for single-stage biventricular repair. Delaying of the decision for biventricular repair and gaining time for the development of left heart structures without subjecting critically ill babies to long duration of cardiopulmonary bypass are the main advantages of this approach.

Footnotes

Acronyms and Abbreviations

Declaration of Conflicting Interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

References

Gibbs

Wren

Watterson

Hunter

Hamilton

. Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome. Br Heart J. 1993;69(6):551–555.

Photiadis

Sinzobhamvya

Hraska

Asfour

. Does bilateral pulmonary artery banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second stage palliation? A review of the current literature. Thorac Cardiovasc Surg. 2012;60(3):181–188.

Pizarro

Davies

Woodford

Radtke

. Improving early outcomes following hybrid procedure for patients with single ventricle and systemic outflow obstruction: defining risk factors. Eur J Cardiothoracic Surg. 2015;47(6):995–1001.

Stoica

Alistair

Egan

. The retrograde aortic arch in the hybrid approach to hypoplastic left heart syndrome. Ann Thorac Surg. 2009;88(6):1939–1947.

Schranz

Bauer

Reich

. Fifteen year single center experience with the “giessen hybrid” approach for hypoplastic left heart and variants: current strategies and outcomes. Pediatr Cardiol. 2015;36(2):365–373.

Pieper

Mathes

Asfour

. A systematic review of the impact of volume of surgery and specialization in Norwood procedure. BMC Pediatr. 2015;14:198.

Karimi

Farouk

Golden

Gilkeson

. Hybrid palliation of interrupted aortic arch in a high-risk neonate. Ann Pediatr Card. 2010;3(1):74–76.

Ballard

Tibby

Miller

. Growth of left heart structures following the hybrid procedure for borderline hypoplastic left heart. Eur J Echocardiogr. 2010;11(10):870–874.

Hoashi

Kagisaki

Oda

Ichikawa

. Staged biventricular repair for persistent truncus arteriosus with aortic arch obstruction following bilateral pulmonary artery banding. Interact CardioVasc Thorac Surg. 2011;12(2):281–283.

10.

Nassar

Anderson

Hussain

. Second stage of hybrid pathway: have we reached a conclusion? J Thorac Cardiovasc Surg. 2016;152(4):1205.

11.

Alsoufi

Schlosser

McCracken

. Selective management strategy of interrupted aortic arch mitigates left ventricular outflow tract obstruction risk. J Thorac Cardiovasc Surg. 2016;151(2):412–420.

12.

Miyamoto

Sinzobhamvya

Wetter

. Twenty year’s experience of surgical valvotomy for critical aortic stenosis in early infancy. Eur J Cardiothorac Surg. 2006;30(1):35–40.

13.

Akintuerk

Michel-Behnke

Valeske

. Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart. Circulation. 2002;105(9):1099–1103.

14.

Nakano

Kado

Tatewaki

. The Yasui operation for patients with adequate-sized ventricles and ventricular septal defect associated with obstructions of the aortic arch and left ventricular outflow tract. Eur J Cardiothoracic Surg. 2014;45(5):166–172.

15.

Kanter

Kirshbom

Kogon

. Biventricular repair with Yasui operation (Norwood/Rastelli) for systemic outflow tract obstruction with two adequate ventricles. Ann Thorac Surg. 2012;93(6):1999–2005.

16.

Luciani

Ackerman

Chang

Wells

Starnes

. One-stage repair of interrupted aortic arch, ventricular septal defect and subaortic obstruction in the neonate: a novel approach. J Thorac Cardiovasc Surg. 1996;111(2):348–358.

17.

Accord

Schoof

Krings

Haas

. Ross-Konno for interrupted aortic arch: Simplified arch reconstruction using Swing-Back technique. Ann Thorac Surg. 2018;105(2):91–93.