Hipoplastik sol kalp sendromunun prenatal tanısı ve yönetimi: tek merkez sonuçları

: Aim : hypoplastic left heart syndrome (HLHS) incidence is 2-3/1000, and the most common reason for neonatal deaths due to congenital heart defects. A numerous study 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 referral centre. Material-method: The records of patients were diagnosed with HLHS in our referral centre between March 2017-April, 2020. The diagnosis was confirmed with the collaboration of pediatric cardiologist in all cases. Detailed anatomy scan was performed, and karyotype analysis was recommended to all patients. Due to poor perinatal prognosis termination of pregnancy 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: 16 patients were recruited in our study. Mean maternal age was 25.6±5.2 years, and mean gestational age at diagnosis was 20.2±5.1weeks. %68.7 of cases were defined as classical type HLHS, and remaining %31.3 were determined as HLHS secondary to critical aortic stenosis. Karyotype analysis was performed in 8 (50%) cases and revealed normal karyotype in all cases. An extracardiac anomaly was detected in 2 (28.5%) cases, including unilateral renal agenesis and unilateral pes equinovarus. Foramen ovale restriction was detected in 12.5% of cases. Termination of pregnancy was performed in 9 (%56.7) patients. Mean gestational age at birth was 37.4±2.1 weeks, and mean birth weight was 2530±355 grams. Mean follow-up interval and neonatal intensive care unit stays were 16.4±4.7 months and 86±23 days, respectively. Urge septostomy was performed in 2 (28.5%) cases after birth due to foramen ovale restriction. 3 (42.8%) cases died before the first operation. Norwood procedure was performed in 4 (57.1%) cases. 2 cases died after this operation. Glenn shunt and Fontan procedure were performed in the remaining two offspring. Total survival rate was 28.5%. Conclusion: HLHS is a rare cardiac anomaly; however, it has high perinatal morbidity and mortality. Prenatal diagnosis allows family for the fate of pregnancy and planned delivery in a tertiary centre.