Long-term outcomes of hypoplastic left heart syndrome with analysis of the Norwood procedure in infants following bilateral pulmonary artery banding

Abstract

Objective: To investigate the early and long-term outcomes of the deferred Norwood procedure by bilateral pulmonary artery banding (BPAB) versus the neonatal Norwood procedure.

Methods: This retrospective study examined 46 patients with hypoplastic left heart syndrome and its variants undergoing the Norwood procedure for single ventricle physiology between 2004 and 2022 at 3 institutions. The patients were divided into 2 groups: neonatal Norwood procedure (group N; n = 23) and staged Norwood procedure in infants following BPAB (group I; n = 23). Preoperative risk factors, surgical results, survival rates, Fontan candidacy, and long-term complications were compared.

Results: Early survival rates after the Norwood procedure were 91.3% (21 of 23) in both groups. Late survival rates after the Norwood procedure were similar at the 10-year follow-up (group N, 76.3%; group I, 68.7%; P = .63). Fontan completion rates also were comparable in the 2 groups (group N, 77.8%; group I, 85.7%; P = .67). Group N showed a higher median pulmonary artery (PA) index before bidirectional cavopulmonary connection (group N, 177 [interquartile range (IQR), 147-243] mm²/m²; group I, 152 [IQR, 146-163] mm²/m²; P = .03); this trend continued until 5 years after Fontan completion (P = .01). Group N also had a lower rate of freedom from protein-losing enteropathy (PLE) at 9.0 years after the Fontan operation (90.0% vs 52.5% for group I; P = .04), although the incidences of other Fontan-associated events were not significantly different.

Conclusions: Fontan candidacy and survival rates were similar regardless of the timing of the Norwood procedure. Early performance of the Norwood procedure may lead to lower rates of late Fontan-associated events, such as PLE.

Keywords: Norwood procedure; bilateral pulmonary artery banding; complications associated with Fontan circulation; hypoplastic left heart syndrome.

Graphical abstract

Late survival rates after the Norwood procedure were similar in the groups.

Figure 1

Flowchart of surgical pathways and outcomes in this cohort. HLHS, Hypoplastic left heart syndrome; BCPC, bidirectional cavopulmonary connection; HD, hospital death; BPAB, bilateral pulmonary artery banding; TCPC, total cavopulmonary connection.

Figure 2

Representative images of the pulmonary arteries on catheter angiography in the Group N and I. BCPC, Bidirectional cavopulmonary connection; TCPC, total cavopulmonary connection.

Figure 3

Representative computed tomography images of the aortic arch anatomy 1 year after the Fontan procedure in the groups N and I.

Figure 4

Summary of major findings. BPAB, Bilateral pulmonary artery banding; HLHS, hypoplastic left heart syndrome; BCPC, bidirectional cavopulmonary connection; TCPC, total cavopulmonary connection; PLE, protein-losing enteropathy.

Figure 5

Kaplan–Meier curves for survival and events after the Norwood and Fontan procedures. (A) Survival rates after the Norwood procedure. (B) Freedom from arrhythmias after the Fontan procedure. (C) Freedom from PLE after the Fontan procedure. (D) Freedom from Fontan-associated liver disease after the Fontan procedure. (E) Freedom from all Fontan-associated events after the Fontan procedure. CI, Confidence interval; PLE, protein-losing enteropathy.

Figure E1

Kaplan–Meier curves of survival and adverse events after the Norwood and Fontan procedures. (A) Survival rates after the Norwood procedure. (B) Freedom from arrhythmias after the Fontan procedure. (C) Freedom from PLE after the Fontan procedure. (D) Freedom from Fontan-associated liver disease after the Fontan procedure. (E) Freedom from all Fontan-associated events after the Fontan procedure. BPAB, bilateral pulmonary artery banding; CI, confidence interval; PLE, protein-losing enteropathy.