Background The characteristics of contemporary Fontan survivors are not well described. Objective We characterized a large cohort of children who had a Fontan procedure, using measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP). Methods We enrolled 546 children (6–18 years, mean 11.9 years) and compared them within pre-specified anatomic and procedure subgroups. History and outcome measures were obtained within a three month period. Results Predominant ventricular morphology was left (LV) 49%, right (RV) 34%, and mixed 19%. Ejection fraction (EF) was normal for 73% of subjects; diastolic function grade was normal for 28%. Child Health Questionnaire mean summary scores were lower than for controls; however, over 80% of subjects were in the normal range. BNP concentration ranged from <4–652 pg/mL (median 13). Mean percent predicted peak oxygen consumption was 65% and decreased with age. EF and EF z-score were lowest, and semilunar and atrioventricular (AV) valve regurgitation were more prevalent in the RV subgroup. Older age at Fontan was associated with more severe AV valve regurgitation. Most outcomes were not associated with a superior cavopulmonary connection prior to Fontan. Conclusions Measures of ventricular systolic function and functional health status, although lower on average in the cohort compared to controls, were in the majority of subjects within two standard deviations of the mean for controls. RV morphology was associated with poorer ventricular and valvar function. Effective strategies to preserve ventricular and valvar function, particularly for patients with RV morphology, are needed.

The degree of preoperative MR was predictive of outcome, whereas the severity of preoperative cardiac dysfunction and ventricular dilation were not. Mild and moderate MR tended to improve without mitral valvuloplasty. Complete recovery from myocardial dysfunction is expected after dual coronary repair of ALCAPA.

Background-Neo-aortic root dilation (ARD) and neo-aortic regurgitation (AR) may be progressive after arterial switch operation (ASO) for d-loop transposition of the great arteries (dTGA). We sought to identify predictors of ARD and AR after ASO. Methods and Results-335 patients were identified who underwent ASO for dTGA with intact ventricular septum or ventricular septal defect (VSD), including double-outlet right ventricle (DORV), before 2001 with at least 1 postoperative echocardiogram at our institution, at least 1 year after ASO, and no previous atrial switch procedure (median follow-up of 5.0 years). Probability of freedom from ARD was 97%, 92%, 82%, and 51%, from at least moderate AR was 98%, 97%, 96%, and 93%, and from neo-aortic valve or root surgery was 100%, 100%, 99%, and 95%, at 1, 2, 5, and 10 years, respectively. For patients in whom ARD developed, progressive dilation was not observed during late follow-up. By Kaplan-Meier method, independent predictors of ARD, with neo-aortic root z-score of Ն3.0, were previous pulmonary artery band (PAB) (Pϭ0.002, hazard ratio [HR]ϭ2.4) and later time period when ASO was performed (PϽ0.002, HRϭ19.0). Risk factor for at least moderate AR was age Ն1 year at ASO (Pϭ0.002, HRϭ5.8), which was closely related to VSD repair at ASO (PϽ0.001) and previous PAB. Conclusions-Significant ARD and AR continue to develop over time after ASO, but ARD does not tend to be progressive during late follow-up. Previous PAB was a significant risk factor for ARD. Older age at time of ASO, presence of VSD, and previous PAB were risk factors for AR.