304 Agenesis of the ductus arteriosus: combination with pulmonary artery hypertension

Agenesis of the ductus venosus is a rare anomaly that may be responsible of cardiomegaly and hydrops in fetuses. We report 2 cases in combination with pulmonary artery hypertension (PAH). Agenesis of ductus venosus was diagnosed in 2 boys at the second term of pregnancy. None had associated hydrops. Chromosomal karyotyping was normal in both. Both of them had extracardiac malformation: one had oesophageal atresia (requiring surgery at day 1), the second had malformation of the ear and micropenis. Both had neonatal PAH. The first patient received bosentan at the second month of life. He had persistent PAH (PA pressure of 119/48, mean 77 mm Hg) and underwent closure of a small VSD at the age of 7 months. Two years and half after surgery, he remains in NYHA class II with similar PA pressure despite bosentan therapy. He is awaiting for combination therapy with sildenafil. The second presented pulmonary distress at birth requiring mechanical ventilation. He was placed immediately under a combination therapy of epoprostenol and NO for 4 days, received then treprostinil for 15 days and sildenafil. He could be weaned off the ventilator after 3 days. One month and half after birth, he is doing well under sildenafil but has persistent moderated PAH (tricuspid regurgitation velocity of 3 m/s). The antenatal existence of high grade arteriovenous shunt could explain high pulmonary artery pressure. However, the persistence of PAH in these patients remains unclear. In combination with research of chromosomal anomaly and congenital malformation, infants with agenesis of the ductus venosus should benefit after birth of serial ultrasound examination to rule out PAH.