Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle

Abstract

ObjectiveThis tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.MethodsThis retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.Results160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration.ConclusionsIn this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

Postoperative Alanine Aminotransferase Levels Are Associated with Outcomes in Pediatric Patients Undergoing Total Cavopulmonary Connection

“…Moreover, it was reported that adverse outcome-free survival rates 10, 20, and 25 years post-TCPC were 89%, 60%, and 24%, respectively. Additionally, extracardiac TCPC was identified as an independent risk factor for adverse outcomes in a multivariate model [ 14 ]. Risk factors affecting long- and short-term outcomes after TCPC include, but are not limited to, the timing of TCPC operation [ 15 ], flow dynamics of bilateral superior cavopulmonary shunts [ 16 ], older age at stage-2 palliation [ 17 ], and systemic to pulmonary collateral blood flow [ 18 ].…”

Association of Body Mass Index With Clinical Features and Outcomes in Adults With Fontan Palliation

“… 1 Prior studies have identified various long‐term sequelae of the Fontan procedure, including arrythmias, heart failure (HF), thromboembolic events, cirrhosis, protein‐losing enteropathy, and death. 6 , 7 , 8 , 9 , 10 Researchers have previously identified several risk factors associated with these adverse cardiovascular events. However, these factors are mainly nonmodifiable, including preoperative conditions, type of operation, and timing of the procedure.…”

Editorial: cardiovascular anaesthesiology

“…Over the last 50 years, the Fontan procedure has become a mainstay for children born with a single functional ventricle. However, several disputes persist: What is the best configuration for total cavo-pulmonary connection (TCPC), lateral tunnel vs. extracardiac connection [7]? How best to prevent Fontan failure?…”