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The Failing Fontan: from the successful palliation to the unavoidable not-so-slowly progressive failure of the circuit
Session:
SESSÃO DE POSTERS 13 - CONGÉNITOS E HTP 1
Speaker:
Ana Isabel Pinho
Congress:
CPC 2025
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
20. Congenital Heart Disease and Pediatric Cardiology
Subtheme:
20.2 Congenital Heart Disease – Epidemiology, Prognosis, Outcome
Session Type:
Cartazes
FP Number:
---
Authors:
Ana Isabel Pinho; Ana Filipa Amador; Luís Santos; Cátia Oliveira; Carla Sousa; Rui André Rodrigues; Cristina Cruz
Abstract
<p>Background: Fontan procedure has expanded considerably as the most common operation performed in patients with a functional or anatomic univentricular heart. Despite its successful palliation for two to three decades, adult Fontan patients experience a unique spectrum of complications, requiring specialized care.</p> <p>Aim: This study examines the outcomes, complications, and management strategies of adult patients with Fontan physiology.</p> <p>Methods: We conducted a retrospective analysis of Fontan patients ≥18 years-old managed in a Portuguese congenital heart disease center. Data collected included demographics, clinical outcomes, comorbidities, and interventions. A time to adverse event analysis was performed.</p> <p>Results: The study cohort consisted of 37 adult Fontan patients (mean age 27±6 years; 38% female); the 2 oldest Fontan patients were 39 years-old. Median duration of Fontan circulation was 19 years (range 7-32 years) and median follow-up duration post-transition to adult care was 6 years (0-14 years), with regular multidisciplinary evaluations involving cardiology, hepatology, and imaging specialists.</p> <p>Fifty-four percent of patients presented with various degrees of hepatic congestion and liver fibrosis, including 1 patient with hepatocarcinoma. Pulmonary hypertension was observed in 8.1%. Common complications also included heart failure (32%), arrhythmias (19%), protein-losing enteropathy (13%), and thrombi in the Fontan circuit (5%).</p> <p>Long-term follow-up revealed stable hemodynamics without complications in 24% of patients, while 30% required additional surgical or catheter-based interventions. One patient died of end stage heart failure, 1 patient endured re-do Fontan, and 2 patients underwent successful heart transplantation. One patient is currently being studied for heart and liver transplantation. Three female patients got pregnant and 2 gave birth to healthy newborns.</p> <p>Regarding the time-to-adverse-events analysis, more than 50% of patents were event-free during the first 16 years of follow-up. However, after 19 years of follow-up, more than 70% of patients presented with a Fontan complication or need for reintervention. Patients with systemic right ventricle had a significantly higher likelihood of adverse events after transitioning to adult care compared to those with systemic left ventricle, with a hazard ratio of 3.56 (95% CI 1.26–10.02), indicating a more than threefold increase in risk (figure 1).</p> <p>Conclusions: The Fontan operation has transformed the prognosis for patients with single-ventricle physiology, allowing many to transition into adulthood. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. However, adult Fontan patients represent a heterogeneous group that faces considerable morbidity, underscoring the need for lifelong, multidisciplinary care.</p>
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