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

• Published in: JTCVS open Vol. 16; pp. 675 - 688
• Main Authors: Matsunaga, Yoshikiyo, Shikata, Fumiaki, Oka, Norihiko, Okamura, Toru, Tomoyasu, Takahiro, Kaneko, Masahiro, Inoue, Takamichi, Matsui, Kenta, Miyaji, Kagami
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Inc 01.12.2023; Elsevier
• Subjects: bilateral pulmonary artery banding; complications associated with Fontan circulation; Congenital: Hypoplastic Left Heart Syndrome; hypoplastic left heart syndrome; Norwood procedure; ECMO; bilateral pulmonary artery banding; BTT; hypoplastic left heart syndrome; TCPC; PLE; BCPC; HLHS; Norwood procedure; BPAB; PA; FALD; CPB; BW; HFRCP; complications associated with Fontan circulation; CVP
• ISSN: 2666-2736; 2666-2736
• DOI: 10.1016/j.xjon.2023.07.023

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