A huge fistula between the right pulmonary artery and the left atrium was found prenatally in the 27th week of gestation and associated with cardiomegaly related to left heart enlargement (Supplemental video 1). The fistula presented proximal narrowing close to the right pulmonary artery and partially limited the inflow. The maximum pressure gradient was initially 36 mmHg and gradually increased to 49 mmHg during pregnancy. The small and tortuous ductus arteriosus resulted in limited abnormal, retrograde flow from the aorta to the pulmonary arteries. During pregnancy, the foetus received transplacental treatment with digoxin. After birth (37 weeks of gestation, caesarean section, body weight 4000 g), the child was initially in good condition but rapidly deteriorated within hours; because of respiratory failure and increasing cyanosis, the child was given continuous positive airway pressure support and eventually mechanical ventilation. Postnatal echocardiography confirmed the presence and anatomy of a fistula that exhibited mildly restricted inflow through its narrowest region, with a maximum pressure gradient of 28 mmHg (Supplemental video 2). In addition to these findings, enlargement of the left atrium and the left ventricle was observed, and mildly impaired left ventricle systolic function was noted. All four pulmonary veins were properly connected with the left atrium.
2. Department of Gynecology, Fertility and Fetal Therapy, Polish Mother’s Memorial Hospital, Lodz, Poland
3. Department of Cardiology, Polish Mother's Memorial Hospital, Lodz, Poland
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