Hypoplastic left heart syndrome – case report

We present a case of hypoplastic left heart syndrome (HLHS).

A 25- years-old primigravida was referred to our unit after routine dating ultrasound, that revealed the heart anomaly. The patient had no prenatal care prior to this time and was 20 weeks by dates. The family history was negative for congenital anomalies or genetic abnormalities and the patient denied exposure to drugs or toxins. Our ultrasound examination revealed a single fetus with BPD, HC, AC and FL measurements adequate with 20 weeks gestational age. We didn't observed any morphological anomalies, except the heart. The left side of the heart on the four chambers image are very small, especially the left ventricle and it was associated with aortic atresia. The right ventricle, right atrium and pulmonary artery were enlarged.

In M mode examination these data are confirmed. Doppler echocardiography has demonstrated the lack of antegrade flow into the left ventricule, abnormal flow through the foramen ovale, and retrograde flow in the ascending aorta.

We have performed and amniocentesis with fetal karyotype analise, that have revealed a trisomie 18. Because it was a severe anomalie incompatible with postnatal life, after counseling, the parents have decided upon therapeutical abortion.

The infant was female, dead during abortion. The results of autopsy confirmed diagnostic; the placenta and umbilical cord were unremarkable.