Characteristics of Patients with a Univentricular Heart Over the Age of 40 Years

Background: Patients with univentricular heart are often palliated, and their survival is described to be poor. There is still a lack of data regarding those patients who survive more than 40 years. The aim of this study was to analyze the characteristics of these patients.

Materials and Methods: We retrospectively analyzed 40 patients with univentricular heart who were seen in our department in the time period from 2005 to 2018. Data were extracted from the clinical reports.

Results: Clinical characteristics are shown in Table 1. Main diagnosis was double inlet left ventricle (n = 18, 45%), followed by tricuspid atresia (n = 13, 32.5%), Ebstein’s anomaly (n = 5, 12.5%), double outlet right ventricle (n = 3, 7.5%), and pulmonary atresia with tricuspid atresia (n = 1, 2.5%). Systemic ventricle was a morphologic left ventricle in 35 patients (87.5%). Up to 28 patients (70%) had a pulmonary stenosis, 1 patient had pulmonary atresia and 25 patients (62.5%) had malposition of the great arteries. Ventricular function at follow-up was normal or slightly impaired in 28 patients (70%). Twelve patients (30%) were uncorrected. The others had one or more palliative operations. Mean age at first operation was 11.8 ± 12.4 years. During a follow-up of 8.1 ± 6.5 years, cardiac decompensation requiring hospitalization was mean 0.98 ± 1 times; 26 patients had chronic cyanosis (65%), and 10 patients had arterial hypertension (25%). Sixteen patients (40%) had at least one cardioversion, and 11 had at least one ablation. Most common arrhythmia was atrial flutter. An epicardial pacemaker was present in 10 patients, whereas an endocardial pacemaker was found in 3 patients. Up to 29 patients underwent one or more cardiac catheterizations. Most extracardiac complications were liver (29 patients) and renal (12 patients) disorders. During follow-up, 10 patients died (25%). Mean age at death was 46.5 ± 3.7 years.

Conclusion: Most patients with univentricular heart who survive more than 40 years usually have a systemic left ventricle with pulmonary or subpulmonary stenosis and malposition of great arteries, and they are usually operated at adolescents. Most complications are rhythm disturbances. During follow-up, 25% of the patients died.