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Adults with Fontan circulation: insights from a Portuguese Adult Congenital Heart Disease center
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:
20.6 Congenital Heart Disease – Clinical
Session Type:
Cartazes
FP Number:
---
Authors:
Ana Isabel Pinho; Ana Filipa Amador; Cátia Oliveira; Luís Santos; Carla Sousa; Rui André Rodrigues; Cristina Cruz
Abstract
<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">Background: The Fontan procedure has significantly improved survival for patients with single-ventricle physiology, allowing many to reach adulthood. Following Fontan procedure, patients face substantial morbidity risk and require lifelong follow-up with a cardiologist experienced in the care of adult congenital heart diseases (ACHD).</span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">Aim: To understand the diversity of anatomical anomalies and therapy requirements of adult Fontan patients. </span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">Methods: We conducted a retrospective analysis of patients ≥18 years-old with Fontan circulation managed in an ACHD center. Data collected included demographic information, clinical outcomes and interventions. A time to adverse event analysis was performed.</span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">Results: Among 37 adult Fontan patients (mean age 27±6 years; 38% female), the underlying diagnosis were pulmonary atresia (35.1%), hypoplastic left heart syndrome (19%), tricuspid atresia (13%), double-outlet right ventricle (RV) (8%), unbalanced atrioventricular (AV) canal defects (5%), Ebstein anomaly (3%), and other anatomic variants with hypoplasia of either ventricle (16%). The most prevalent surgical technique was extracardiac conduit (76%), followed by lateral tunnel (19%) and atriopulmonary connection (5%). The systemic ventricle was morphologically left in 70%. A fenestration or residual shunt persisted in 43%. The mean basal oxygen saturation was 95%; 54% had desaturation with exercise. Most patients were asymptomatic (65% NYHA class I), with normal ventricular function in 62% of those with a systemic left ventricle and impaired function in 73% of those with a systemic RV; more than mild AV valve regurgitation was present in 16%.</span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">Rhythm disturbances were common, with atrial arrhythmias observed in 16% and ventricular arrhythmias in 11%. Basal ECG frequently showed AV or intraventricular conduction disturbances (73%); 5% required pacemaker implantation. Medical therapy included ACE inhibitors (46%), spironolactone (35%), SGLT2 inhibitors (24%), beta blockers (30%), diuretics (19%), aspirin (49%), anticoagulation with warfarin (22%) or DOAC (24%).</span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">Median follow-up was 6 years since transition to ACHD clinic, with regular multidisciplinary evaluations. More than 50% of patients were event-free during the first 5 years post-transition to adult care and 2 female patients achieved successful pregnancies. However, after 10 years of follow-up in adult care, more than 80% of patients suffered a Fontan complication or needed reintervention. One patient died of heart failure, 1 patient underwent a re-do Fontan and 2 patients underwent successful heart transplantation.</span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Aptos,sans-serif">Conclusions: Adult Fontan patients represent a heterogeneous group with diverse anatomical anomalies, comorbidities and clinical trajectories. This single-center experience highlights the importance of individualized long-term follow-up to address unique clinical needs and improve outcomes.</span></span></p>
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