Outcomes of the Fontan Operation for Patients With Heterotaxy: A Meta-Analysis of 848 Patients

Abstract

Background. Heterotaxy is considered a risk factor for adverse events at all stages in the pre-Fontan pathway, and Fontan outcomes are expected to be worse in patients with heterotaxy. The aim of this study was to review existing literature reporting outcomes of the Fontan operation systematically in patients with heterotaxy.Methods. A systematic review and meta-analysis was performed to identify and synthesize early mortality and medium-and long-term survival in heterotaxy patients after the Fontan procedure. Subsequent outcome analyses were stratified by study period era, cohort size, and proportion of right versus left atrial isomerism to explore predictors of outcome.Results. A total of 21 studies were included for analysis, which were composed of 848 post-Fontan heterotaxy patients. Early mortality varied between 1% and 30% with a weighted event rate of 14% (95% confidence interval [CI], 10%-19%). Survival at 1, 5, and 10 years was 86% (95% CI, 79%-91%), 80% (95% CI, 71%-87%), and 74% (95% CI, 59%-85%), respectively. Stratification by study period highlighted that studies with a median study period year of 1995 or later had similar early mortality and 1-and 5-year survival, but superior 10-year survival (P [ .02) compared with earlier studies. Stratification by cohort size and right versus left atrial isomerism did not reveal subgroup differences.Conclusions. Compared with existing literature, in patients with heterotaxy, early mortality after Fontan is higher than for the overall Fontan population. Long-term survival is comparable to the overall Fontan cohort. When heterotaxy patients are successfully transitioned to Fontan, subsequent survival is acceptable and predictable. Long-term follow-up is lacking.

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China

“…This study reviewed our experience in the management of 86 patients with atrial isomerism in our center over the past 13-years. Our data showed that despite even greater clinical diversity, the intermediate outcomes were improving, and 10year survival rate reached 79.3%, which is comparable to many of the previous reports (5,6). Consistent with previous studies, we found that extracardiac TAPVC, more than moderate AVV regurgitation, and requirement for first-stage palliative surgery were independent risk factors for mortality.…”

“…Cardiovascular mortality in early childhood is estimated at about 10% or more, even in the current era (2,3). Fortunately, recent reports from developed countries showed that the survival of atrial isomerism with cardiac malformation is continuing to improve, and most patients can survive into adulthood (4)(5)(6)(7)(8).…”

Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study

“…[6][7][8][9][10] In addition, in our cohort of patients an associated syndrome did not increase the mortality and the survival of our cohort of patients with heterotaxy did not differ from that published in a recent meta-analysis. 11 Our study suggests that the presence of a genetic syndrome in association with single-ventricle AVSD does not preclude an excellent outcome for Fontan palliation.…”

Computed Tomography in the Evaluation of Fontan Circulation

“… 5) Studies have indicated that early mortality after the Fontan procedure is higher in heterotaxy patients, but the long-term survival is comparable to the overall Fontan population. 12) …”