Long-term outcomes of single-ventricle palliation for unbalanced atrioventricular septal defects: Fontan survivors do better than previously thought

• Published in: The Journal of thoracic and cardiovascular surgery Vol. 153; no. 2; pp. 430 - 438
• Main Authors: Buratto, Edward, MBBS, Ye, Xin Tao, BBiomed, King, Gregory, BBiomed, Shi, William Y., MBBS, Weintraub, Robert G., MBBS, d'Udekem, Yves, MD, PhD, FRACS, Brizard, Christian P., MD, MS, Konstantinov, Igor E., MD, PhD, FRACS
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.02.2017
• Subjects: Abnormalities, Multiple; Adolescent; Australia - epidemiology; Cardiothoracic Surgery; Child; Child, Preschool; Echocardiography; Female; Follow-Up Studies; Fontan; Fontan Procedure - methods; Forecasting; Heart Septal Defects - diagnosis; Heart Septal Defects - mortality; Heart Septal Defects - surgery; Heart Ventricles - abnormalities; Heart Ventricles - diagnostic imaging; Heart Ventricles - surgery; Humans; Infant; Male; Palliative Care; Retrospective Studies; Risk Assessment; Risk Factors; single-ventricle palliation; Survival Rate - trends; Treatment Outcome; unbalanced atrioventricular septal defect; Young Adult; Australia; AVV; bidirectional cavopulmonary shunt; single-ventricle palliation; pulmonary artery; hypoplastic left heart syndrome; left ventricle; LV; PDA; SVP; HLHS; atrioventricular septal defect; atrioventricular valve; AVVR; TAPVD; PA; uAVSD; unbalanced atrioventricular septal defect; AVSD; atrioventricular valve regurgitation; patent ductus arteriosus; total anomalous pulmonary venous drainage; BCPS; Fontan
• ISSN: 0022-5223; 1097-685X
• DOI: 10.1016/j.jtcvs.2016.09.051

Abstract Background Single-ventricle palliation (SVP) for children with unbalanced atrioventricular septal defect (uAVSD) is thought to carry a poor prognosis, but limited data have been reported. Methods We performed a retrospective review of children with uAVSD who underwent SVP at a single institution. Data were obtained from medical records and correspondence with general practitioners and cardiologists. Results Between 1976 and 2016, a total of 139 patients underwent SVP for uAVSD. A neonatal palliative procedure was performed in 83.5% of these patients (116 of 139), and early mortality occurred in 11.2% (13 of 116). Ninety-four patients underwent stage II palliation, with an early mortality of 6.4% (6 of 94). Eighty patients (57.6%) underwent Fontan completion, with an early mortality of 3.8% (3 of 80). Interstage mortality was 11.7% (12 of 103) between stages I and II and 17.0% (15 of 88) between stage II and Fontan. Long-term survival was 66.5% (95% confidence interval [CI], 57.9%-73.9%) at 5 years, 64.4% (95% CI, 55.5%-72.0%) at 15 years, and 57.8% (95% CI, 47.5%-66.8%) at 25 years. Survival post-Fontan was 94.9% (95% CI, 86.9%-98.0%) at 5 years, 92.0% (95% CI, 80.6%-96.8%) at 15 years, and 82.4% (95% CI, 61.5%-92.6%) at 25 years. Risk factors associated with death or transplantation were aortic atresia (hazard ratio [HR], 5.3; P  = .03) and hypoplastic aortic arch (HR, 2.5; P  = .02). Atrioventricular valve operations were required in 31.7% of the patients (44 of 139), with 31.8% of them (14 of 44) requiring a further operation. Conclusions Children undergoing SVP for uAVSD have substantial mortality, with <60% survival at 25 years. However, survival of children who achieve Fontan completion is better than has been reported previously.