Reintervention and survival in 1428 patients in the Australian and New Zealand Fontan Registry

Abstract

ObjectivePatients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients.MethodsA retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival.ResultsOf the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%.ConclusionsA substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.

Heart failure related to adult congenital heart disease: prevalence, outcome and risk factors

“…Maybe more importantly, ACHD-HF prevalence in cyanotic patients, patients with a Fontan circulation and patients with a systemic RV was >30% from the age of 35 years and even >50% from the age of 50 years, consistent with prior studies. 25,26 Although ACHD-HF prevalence is lower in patients with a biventricular circulation and systemic LV, absolute numbers in these subgroups exceed the combined number of cyanotic patients, patients with a Fontan circulation, and patients with a systemic RV.…”

Commentary: You should occasionally look at the results!!

“…It has now been demonstrated that catheter intervention in the Fontan conduit can be successful with minimal morbidity. 3,4 The surprising findings of this manuscript should encourage us to investigate further even the simplest acts that we are performing. It should also open our minds to other innovative solutions.…”

“…These findings are consistent with previous computational modeling 2 and a retrospective review from South Korea. 3 It is insightful that no clear association was found between the implanted conduit size and the measured hemodynamic parameters. Surgeons frequently debate intraoperatively as to what size conduit to implant, with a bias toward trying to implant the largest conduit possible without causing distortion of surrounding structures.…”

Late Fontan Circulatory Failure. What Drives Systemic Venous Congestion and Low Cardiac Output in Adult Fontan Patients?

“…While the Fontan operation became the final palliation for thousands of patients with a single anatomical or functional ventricle worldwide, pediatric and adult congenital cardiologists are increasingly confronted with the limitations posed by the circulation created many years ago (3)(4)(5). There is no doubt that the surgical intervention has dramatically improved the longevity and quality of our patients' lives, but long-term morbidity and mortality remain high (4).…”