RESUMEN
Data on the characteristics and long-term outcomes of patients who underwent Fontan surgery and surviving into adulthood are limited. We aimed to describe our center's long-term experience with this unique patient population. Included were adult patients who had undergone Fontan surgery and were followed up at our Adult Congenital Heart Disease clinic between the years 1994 and 2021. We describe cardiac and noncardiac morbidities, medical treatment, laboratory data, echocardiographic characteristics, and all-cause mortality. The primary outcome was a composite of heart failure hospitalizations or death. A total of 107 patients who underwent Fontan surgery were followed up during the study period; 46.7% were male. The mean age at time of Fontan was 7.4 ± 6.2 years and the mean age at the last follow-up or at the time of an outcome event was 35.0 ± 8.0 years (range 21.1 to 62.8). At the last documented follow-up, 74.7% of the cohort were in New York Heart Association functional class I/II. The common morbidities included atrial arrythmias (37%) and stroke (17%). The primary outcome occurred in 17.7%. By the end of the study period, 9.3% of the patients in the cohort died. In a multivariate logistic regression analysis, controlling for gender, age, and Fontan type, worse functional class at the last follow-up (New York Heart Association III/IV vs I/II) was significantly associated with the risk of the primary outcome (odds ratio 34.57, 95% confidence interval 6.728 to 177.623, p <0.001). In conclusion, long-term outcomes of patients surviving into adulthood with a Fontan circulation is encouraging. Most of these patients achieve good functional cardiovascular status, despite the complex anatomy and a substantial burden of co-morbid conditions, specifically, atrial arrythmias and thrombotic events. Functional class was independently associated with heart failure hospitalizations and mortality.