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Late “failing” Fontan in a tertiary center in Portugal: 18 years follow-up
Session:
CO11 - Circulação Pulmonar / Congénitos
Speaker:
Ana Luísa Costa
Congress:
CPC 2019
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:
Comunicações Orais
FP Number:
---
Authors:
Ana Luísa Costa; Paulo Éden Santos; Jorge Casanova; António Rodrigues Sousa; Sofia Granja
Abstract
<p><strong>Background:</strong> The Fontan procedure represents the final surgical stage of the troublesome univentricular pathway. We aimed to describe some of the factors associated with “Late Failing” Fontan (FF) circulation.</p> <p><strong>Materials and Methods:</strong> Clinical medical records of children/young adult born after 1987 submitted to Fontan procedure and followed up at a tertiary centre in Portugal, were reviewed. Patients with evidence of early FF were excluded. Late FF was defined as: death/transplant, Fontan conversion, heart failure (HF) symptoms, protein-losing enteropathy (PLE) or plastic bronchitis.</p> <p><strong>Results:</strong> Fourty-six patients were included, 25 males (54,3%), with 4-29 yo at present, with complex CHD (table 1). Thirty patients had single LV anatomy, 12 single RV anatomy and four biventricular heart. Total cavopulmonary connection (TCPC) was performed in all patients and the median age was 5,7yo (P25-P75: 4,4-7,6yo). Forty-one patients had an extracardiac conduit (EC) and five a lateral tunnel (LT); 37 were fenestrated Fontan. Eleven patients (23.9%) developed late FF: five PLE, five HF and one death. No patient was submitted to cardiac transplant or Fontan conversion. The only deceased patient was 13yo (5,2yo after surgery) with malignant arrhythmia. The median time of FF presentation was 5.0yo (P25-75: 0.6-9.9) after the Fontan surgery. Comparing with the non-FF, FF patients more frequently had LT Fontan (4/5 vs EC conduit 7/41, p<0,001) and arrhythmias (4/8 vs no arrhythmia 5/30, p 0.049). All patients with ventricular dysfunction (5/46) developed FF (p<0,001). No other significant differences were found between the groups (ventricular morphology, size of pulmonary arteries, presence of fenestration, collateral vessels).</p> <p><strong>Conclusions:</strong> Less than 25% of our patients developed late FF. The lack of associations between some of the studied factors and FF might be due to the small sample. Future studies on the factors predicting the outcome of Fontan procedure are imperative to improve the management of these patients.</p>
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