20 years experience of Fontan Surgery in Malaysia
Tóm tắt
Background: Univentricular heart disease is a complex congenital heart
disease with only one functional ventricle and is linked to significant mortality and
morbidity. The emergence of Fontan surgery has improved the outcome of this condition.
This study examines post-Fontan outcomes in a single-ventricle population at Malaysia's
primary cardiac centre, National Heart Institute Malaysia.
Methods: We performed a retrospective review of medical data for all children with functionally univentricular hearts who were part of the National Heart Institute follow-up from January 1st, 2000 to December 31st, 2022. Of 854 univentricle patients, 429 of them underwent Fontan surgery and their diagnoses, methods of operation and outcomes wererecorded. The primary outcome was mortality.
Results: Among 429 post-Fontan patients, Tricuspid atresia, 123 (28.7%), double inlet left ventricle, 196 (21.7%), and imbalanced AVSD, 64 (14.9%) were the most prevalent group diagnoses for univentricular hearts. The majority type of Fontan surgery was extracardiac Fontan, performed on 383 patients (89.2%) followed by intra-extracardiac which was 30 patients (7%). The mean age at the time of Fontan's surgery was 6.5 years. Among the 429 patients, 41 (9.5%) deaths were recorded with the mean duration of death from Fontan surgery being 2.6 years and the mean age for death being 11 years old. The commonest cause of death was myocardial failure 15 (36.5%), Fontan failure 10 (24.4%) followed by intracranial haemorrhage 5 (12.2%). In comparison, the non-Fontan univentricular heart patients recorded 239 (56.2%) deaths out of 425 patients with a mean age of death of 3 years old.
Conclusion: Univentricular hearts with Fontan surgery have better outcomes with significantly lower mortality rates. However, the cardiac cause of mortality in post-Fontan patients remains substantial. Further study should be done to identify predictors for post-Fontan mortality. Univentricular hearts with Fontan surgery have better outcomes with significantly lower mortality rates. However, the cardiac cause of mortality in post-Fontan
patients remains substantial. Further study should be done to identify predictors for
post-Fontan mortality.
