P27.07: Intrauterine intrafunicular treatment of nonimmune hydrops fetalis caused by fetal cardiomyopathia

The incidence of nonimmune hydrops fetalis (NIHF) is 1 : 1500 to 3800 deliveries. The perinatal mortality rate is 50 to 98 per cent, and the etiology is clarified only in 70 to 75 per cent of cases. Prenatal ultrasound (US) is highly important in the diagnosis and follow-up of NIHF. Cardiovascular etiology includes structural congenital heart defects (CHD), fetal arrhythmias, infectious causes, and cardiac tumors. With invasive intrauterine and appropriate postnatal treatment the outcome of pregnancies affected with severe NIHF might be improved. Authors report on a successful invasive intrauterine intrafunicular medical treatment of NIHF diagnosed at 27 weeks of pregnancy and caused by fetal cardiomyopathia. Fetal echocardiography ruled out structural CHD. Although maternal TORCH, HSV, HBV, and parvovirus B19 serology was negative, impaired systolic fetal cardiac function and AV-valve insufficiency raised the possibility of fetal myocarditis. Percutaneous fetal umbilical blood sampling verified normal fetal karyotype, negative fetal viral serology, normal fetal cord blood-gas values, and ruled out fetal anaemia. After unsuccessful maternal digoxin treatment for one week written consent had been obtained and direct intrafunicular intravenous combined dexamethasone + furosemide + digoxin therapy was administered to the fetus by cordocentesis and follow-up US revealed rapid improvement of NIHF for one week. Then, due to increased amount of fetal ascites, pericardial effusion, and maternal polyhydramnios the intrafunicular procedure was repeated within 7 days together with the drainage of the fetal ascites and fetal lung maturity test. The fetus was delivered for progressive NIHF within 24 hours by elective cesarean section. Gestational age at birth, birth weight, and 5-minute Apgar score were 29 weeks, 2300 g, and 5, respectively. The infant was discharged from the NICU at postpartum day 68 with the diagnosis of cardiomyopathia and is waiting for heart transplantation.