Functional state following the Fontan procedure

Abstract

Background Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. Methods and Results We analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset. Conclusions Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes.

Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake

“…32 This finding is similar to those previously reported. 13 A larger study from the PHN showed a weak association between exercise performance and ventricular morphology, with the left-ventricle subgroup having a higher percentage of predicted maximum Vo 2 .…”

Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome

“…6, 7, 28-33 Although heart transplantation is an option for select patients with failing Fontan physiology, survival is significantly lower among patients with a prior Fontan operation compared to patients with other forms of congenital heart disease or cardiomyopathy. 34, 35 Thus, even if there are substantial on-going issues in patients with a BV circulation after FAV, the potential for improved long-term prognosis and survival compared to patients with a univentricular circulation is the real question.…”

Fetal interventions for congenital heart disease

“…Although early surgical survival has improved for infants with HLHS, staged univentricular palliation to a Fontan circulation carries significant lifelong morbidity and mortality [1][2][3][4]. A subset of patients with HLHS share a common pathophysiologic etiology; namely, severe aortic stenosis in utero with left ventricular (LV) dilation and dysfunction.…”