Stage-1 Hybrid Palliation for High-Risk 2-Ventricle Patients with Ductal-Dependent Systemic Circulation in the Era of High Prenatal Detection

Objective We reviewed our center's prenatal detection and surgical experience with high-risk, 2-ventricle patients, with complex congenital heart disease that underwent stage-1 hybrid palliation. Methods We retrospectively identified those born between March 2008 and March 2021 with 2-ventricle hearts, complex congenital cardiovascular malformations, and ductal-dependent systemic circulation that underwent stage-1 hybrid palliation consisting of surgical bilateral pulmonary artery banding and interventional catheterization placed ductus arteriosus stents. Results We identified 30 patients. Of the 30, 19 (63%) were male. For the 30, median gestational age was 35 weeks (29-39 weeks), and median birth weight was 2.2 kg (0.6-4.5 kg). Of the 30, 1 was transferred from an adjacent state, and 29 were born in Nevada. Of the 29 born in Nevada, overall statewide prenatal detection was 18 of 29 (62%); however, for 2008 to 2011 the prenatal detection rate was 3 of 10 (30%) and 15 of 19 (79%) for 2012 to 2021, P = .03. For the last 5 years, prenatal detection for Nevada-born patients was 8 of 8 (100%). Two full-term newborns, without a prenatal diagnosis, presented postnatally in extremis. For the 30 patients, there were 0 stage-1 hybrid palliation mortalities, 1 subsequent repair mortality, and 3 late nonsurgical deaths. Conclusions Stage-1 hybrid palliation may result in excellent surgical outcomes for high-risk, 2-ventricle patients. Additionally, high rates of population-wide prenatal detection are possible for high-risk congenital heart disease, allowing prenatal planning and possibly reducing postnatal extremis presentations.

Heart Transplantation of a Preterm Infant With HLHS

A 33-week gestation, 1.75-kg female infant with mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome and severe ventriculo-coronary connections underwent surgical septectomy and bilateral pulmonary artery banding at five weeks of age (2.10 kg). After separation from bypass, she developed hemodynamic instability requiring venoarterial extracorporeal membrane oxygenation support. She was listed for heart transplantation and transplanted after three days of support with an oversized heart (4.7:1 donor–recipient weight ratio).

Successful combination treatment with transcatheter balloon atrioseptostomy and bilateral pulmonary artery banding in a collapsed preterm neonate

There has been an increase in the use of extracorporeal membrane oxygenation for severe neonatal cardiac failure. However, the frequency of complications is high, particularly in preterm and low-birth-weight neonates. Herein, we present combination treatment with transcatheter balloon atrioseptostomy and bilateral pulmonary artery banding in a collapsed preterm neonate. This strategy can be an alternative to circulatory support using extracorporeal membrane oxygenation.

DOZ047.90: An interesting and extremely rare case report of PCD and HLHS

An antenatally diagnosed hypoplastic left heart syndrome boy from Kuwait was transferred in utero for postnatal surgical management. He was born to a nonconsanguineously married couple by spontaneous delivery at 33 + 5 weeks of gestation with a birth weight of 2.4 kg. He was on prostaglandin infusion after birth and shortly underwent bilateral pulmonary artery banding with gore-tex shunts (aorta to pulmonary artery shunts) to let him grow and get over his prematurity for his cardiac surgical palliative procedures. He had some right upper lobe collapse, which was treated with physiotherapy and suctioning to proceed for his first stage cardiac surgical repair (Norwood I). Following this, he had a rough course owing to his cardiac condition but was extubated without many problems in due course. He was persistently tachypnoeic, had two episodes of acute decompensations during his stay due to his lung segment collapse needing invasive ventilation, and was very fragile, needing careful management. He needed noninvasive positive pressure ventilation at other times even when stable, to keep his airways from collapsing. He underwent a bronchoscopy and CT scan when stable enough to do it that showed a normal airway but with thick, tenacious, and clear secretions on bronchoscopy while a CT showed some areas of atelectasis and pulmonary interstitial emphysema. He was investigated for cystic fibrosis and primary ciliary dyskinesia as other causes that could lead to persistent and recurrent atelectasis were ruled out and his acute episodes dealt with appropriately. His genetic testing results have confirmed the clinical diagnosis of primary ciliary dyskinesia and he is being discussed for further management from a multidisciplinary point of view.

Extracorporeal membrane oxygenation in a low-weight infant after cardiac surgery

A neonate was diagnosed with ventricular septal defect and aortic coarctation. Bilateral pulmonary artery banding was performed at 9 days of age (weight 1472 g). Because of ductal shock, emergency extracorporeal membrane oxygenation was initiated after surgery. Fine adjustment of the bilateral pulmonary artery banding sites using hemoclips was carried out to facilitate weaning from extracorporeal membrane oxygenation, and staged delayed sternal closure was achieved. Arch repair, ventricular septal defect closure, and tricuspid annuloplasty were undertaken when the patient was 4-months old (weight 1.8 kg). She was discharged at 11 months of age (3.0 kg).

Bilateral Pulmonary Artery Banding before Norwood Procedure: Survival of High-Risk Patients

Background This study reports midterm results of high-risk patients with hypoplastic left ventricle treated with initial bilateral pulmonary artery banding (PAB) before secondary Norwood procedure (NP). Methods Retrospective study of 17 patients admitted between July 2012 and February 2017 who underwent this treatment strategy because diagnosis or clinical status was associated with high risk for NP. Survival was compared with that of patients who underwent primary NP. Results Mean Aristotle comprehensive complexity score for NP would have been 19.7 ± 2.6. Risk factors included obstructed pulmonary venous return (n = 9), body weight < 2.5 kg (n = 7), total anomalous pulmonary venous connection (n = 3), and necrotizing enterocolitis (n = 1). Ten patients had a score ≥ 19.5. Early survival after PAB was 82.4% (14/17). NP was performed in 14 patients after improvement of clinical condition at a median age of 56 days and a weight ≥2,500 g. There was no 30-day mortality, but one interstage death. One patient died later after Glenn operation. One-year survival after primary PAB followed by NP was 70.6 ± 11.1%. During the same period, 35 patients with overall lower risk factors underwent primary NP; early postoperative survival and 1-year survival were 88.6 ± 5.4% and 68.6 ± 7.8%, respectively. There was no significant difference in survival between the two groups (p = 0.83) despite higher risk in the secondary Norwood group (p <0.0001). Conclusions PAB before NP in high-risk patients constituted salvage management. Primary PAB provided enough time for stabilization and control of most risk factors. It allowed midterm survival equivalent to the survival after primary NP in lower risk neonates.