Systolic dysfunction of systemic ventricle in patients who underwent a Fontan operation

Abstract

The aim of the study was to report the clinical and echocardiographic dataof patients who underwent a Fontan operation, and define the group withsystolic dysfunction in the systemic ventricle observed during postoperativefollow-up. The medical records of 183 patients [mean age: 10.93 ± 5.89 years(range: 2.5-45 years)] who were referred to our center and underwent a Fontanoperation were retrospectively reviewed. The clinical, echocardiographic, andpostoperative follow-up data of the patients were recorded. Preoperatively,68 (37.2%) patients experienced pulmonary stenosis, while 41 (22.4%) hadpulmonary atresia and 74 (40.4%) had pulmonary hypertension. The mostcommon pathology in patients who were ineligible for biventricular repairwas tricuspid atresia, seen in 51 patients (27.9%), followed by double-inletleft ventricle pathologies in 40 patients (21.9%). In total, 38 (20.7%) patientshad a biventricular structure; among those with a single ventricular structure,the systemic ventricle involved was the right ventricle in 51 (27.9%) patientsand the left ventricle in 94 (51.4%) patients. During follow-up, a total of 31(16.9%) patients underwent catheterization and ventricular systolic functionswere preserved in 168 (91.8%) patients. In Fifteen (8.2%) patients developedsystolic dysfunction (ejection fraction < 50%). The mean age of the groupdeveloping systolic dysfunction was 15.6 ± 2.63 years (median, 13 years;range: 5-45 years). Of this group, 10 patients had a left ventricular structureof the systemic ventricle, 14 had atrial situs solitus, and 4 had moderateatrioventricular valve insufficiency. Even if the systemic ventricle is in the leftventricular structure, systolic dysfunction in the systemic ventricle develops,especially after the first 10 years, and this makes us think rudimentaryventricle function should also be carefully monitored for intra-univentriculardiastolic asynchrony.