Prenatal diagnosis and management of hypoplastic left heart syndrome: Single center results

INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is the most common reason for neonatal deaths among congenital heart defects. Numerous studies showed that prenatal diagnosis improves prognosis. We aimed to review the prenatal assessment of associated extracardiac anomalies, postnatal outcomes, and surgical management in cases of HLHS that were detected in our center.
METHODS: The records of patients diagnosed with HLHS evaluated between March 2017 and April 2020. A detailed anatomy scan was performed, and karyotype analysis was recommended to all patients. Due to poor perinatal prognosis, termination of pregnancy (TOP) was offered an option to families. Serial ultrasonographic examinations every 2–4 weeks. Postnatal echocardiography was performed, and the prenatal diagnosis was confirmed in all offspring. Surgical outcomes were recorded.
RESULTS: Sixteen patients were recruited in our study. The mean gestational age at diagnosis was 20.2±5.1 weeks. About 68.7% of cases were defined as classical type HLHS, and the remaining 31.3% were determined as variant type HLHS. TOP was performed in 9 (56.7%) patients. The mean follow-up interval was 16.4±4.7 months. Urge septostomy was performed in 2 (28.5%) cases after birth due to foramen ovale restriction. Three (42.8%) cases died before the first operation. Norwood procedure was performed in 4 (57.1%) cases. Two cases died after this operation. Glenn shunt and Fontan procedure were performed in the remaining two offspring. The total survival rate was 28.5%.
DISCUSSION AND CONCLUSION: HLHS has high perinatal morbidity and mortality. Prenatal diagnosis allows the family for the fate of pregnancy and planned delivery in a tertiary center.