הכינוס השנתי של החברה הישראלית לפדיאטריה קלינית - חיפ"ק 2022

Medium-term Outcomes and Risk Factors in Single Ventricle Congenital Heart Disease, Systemic Left Versus Systemic Right Ventricle: Single Center Cohort

Shani Shveky 1 Eldad Erez 2 Julius Golender 3 Merav Luria 2 Avital Rabinovitz 2 אסף תא-שמע 3
1School of Medicine, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, ישראל
2Cardiothoracic Surgery, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, ישראל
3Pediatric Cardiology, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, ישראל

Background: Single ventricle congenital heart defects (SV-CHD) are the most severe type of congenital cardiac anomalies. The aim of this study was to analyze current survival rates of patients with SV-CHD and compare the outcomes for single left ventricle (LV) versus single right ventricle (RV) as well as other risk factors.

Methods: We conducted a retrospective observational study based on patients` surgical and medical records. We included all infants diagnosed with SV-CHD who had their first cardiac surgery at our hospital before one year of age, between the years 2011-2018.

Results: 78 patients (43 males, 55%) met inclusion criteria. 45 (57.7%) had single RV. 30 (38.4%) had single LV and 3 (3.7%) had two ventricles not amenable for surgical septation. Median follow up was 6.95 years. Overall mortality during follow up was 36%. Survival rates were significantly higher for patients with single LV compared to patients with single RV (86.7% versus 51.1% p=0.002, Fig 1).

Higher lactate level prior to and during first cardiac surgery significantly correlated with higher mortality (p=0.011 and 0.001, respectively). We found statistically significance correlation between higher gestational age at birth, higher birth weight and shorter duration of the first operation with higher survival rates (p=0.04, 0.01 and 0.005, respectively).

Conclusions: Patients with a single RV have significantly lower survival rates compared to patients with a single LV. The data may assist physicians and parents during prenatal counseling. Higher lactate levels prior to and during the first cardiac surgery significantly correlate with higher mortality rates.