Fetal hypoplastic left heart syndrome and neonatal follow-up in Polish Mother's Memorial Hospital Research Institute report from January 2023 to January 2024: an improvement of survival rate.

Abstract

Objectives: In 2023 and 2024, our primary study group consisted of 39 fetuses with HLHS MATERIAL AND METHODS: From the final analysis, we excluded the following: 1 twin pair (twin A with normal heart anatomy and twin B with HLHS), 2 terminations of pregnancy, and 2 newborns without prenatal echocardiography (only ultrasound was performed, and HLHS was not detected prenatally). As a result, our study group comprised 34 fetuses. The mean gestational age at the detection of HLHS was 19 weeks (± 6 weeks). Extracardiac malformations were present in 7 fetuses out of 34. Prenatally diagnosed genetic problems included Turner syndrome, DiGeorge syndrome, and an abnormal Y chromosome.

Results: Most pregnancies ended at term, with a mean gestational age at delivery of 38.5 weeks (± 0.9). The mean birth weight was 3142 g, and the mean Apgar score was 8 at 1 minute. After birth, there were 2 neonatal deaths: one newborn with HLHS and a birth weight of 1900 g, and another with HLHS and lymphangiectasies. Norwood surgery was performed in 32 newborns: in survivors, at a mean age of 12 ± 5 days, and in the non-survivor group, at a mean of 17 ± 5 days (t-test p = 0.027). The overall survival rate from 2023 to 2024 was 87% for liveborn fetuses/neonates, significantly higher than the historical data from 2008-2015, which showed a survival rate of 64% (chi-square p = 0.015).

Conclusions: Prenatal echocardiographic data do not equate to cardiac surgery data, as they include deaths in utero, terminations of pregnancy, and deaths after delivery (presurgery mortality). In consultations with future parents following the prenatal detection of HLHS, the entire spectrum of possible outcomes should be discussed, including the survival rate after Norwood surgery at our center, which was approximately 90% from January 2023 to January 2025. The day of cardiac surgery may be an additional important risk factor.