Outcomes of patients with hypoplastic left heart syndrome reaching adulthood after fontan palliation: Multicenter study

Hypoplastic left heart syndrome (HLHS) is a high-risk condition among the wider spectrum of single-ventricle physiology lesions, and few survivors have reached adulthood.1 As HLHS is one of the most common diagnoses for children undergoing Fontan palliation,2 identification of risk factors for adverse outcomes in this emerging population is of importance. We report results from a multicenter observational study that characterizes adults with Fontan palliation for underlying HLHS at entry into adulthood and defines factors associated with adverse outcomes. Adults (≥18 years by July 2014) with HLHS were studied. The diagnosis of HLHS was restricted to patients with mitral stenosis or atresia, aortic stenosis or atresia, and a diminutive left ventricle. Excluded were patients with other forms of dominant right ventricular lesions with univentricular physiology managed with a Norwood procedure (ie, unbalanced atrioventricular septal defect or double-outlet right ventricle) or those with heart transplantation. Available pediatric and adult cardiovascular data were reviewed. The primary end point (Fontan-specific major adverse cardiovascular event) was defined as death, transplantation listing, hospitalization (>24 hours) for heart failure requiring intravenous diuretics, sustained ventricular …