Genetic and Extracardiac Anomalies Are Associated With Inferior Single Ventricle Palliation Outcomes

The Annals of Thoracic Surgery
Bahaaldin AlsoufiK R Kanter

Abstract

We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages. First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53%), shunt in 173 (33%), and band in 73 (14%). Outcomes were compared between those with GS/EC anomalies (121 [23%]) and without GS/EC anomalies (409 [77%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation). GS/EC anomalies varied among SV defects (range, 3% for double-inlet left ventricle to 100% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13% vs 11%, p = 0.552) and unplanned reoperation (16% vs 11%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24% vs 12%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60% vs 77%, p = 0.002) and lower 10-year survival (56%...Continue Reading

Citations

Dec 15, 2019·World Journal for Pediatric & Congenital Heart Surgery·Bahaaldin AlsoufiBrian Kogon
May 24, 2019·World Journal for Pediatric & Congenital Heart Surgery·Nicole L HerrickRaghav Murthy
Jan 8, 2021·World Journal for Pediatric & Congenital Heart Surgery·Omar ToubatS Ram Kumar

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