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EP10.10: Large fetal pulmonary arteriovenous malformation with severe cardiomegaly and favourable postnatal outcome
Author(s) -
Guerra F.A.,
Vogt S.,
GarciaHuidobro T.,
Olate A.,
Solis N.,
Vega A.,
Ferrand P.,
Gajardo M.,
Rodriguez J.G.
Publication year - 2018
Publication title -
ultrasound in obstetrics and gynecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.202
H-Index - 141
eISSN - 1469-0705
pISSN - 0960-7692
DOI - 10.1002/uog.19939
Subject(s) - medicine , ductus venosus , cardiology , fetus , ductus arteriosus , umbilical artery , foramen ovale (heart) , pulmonary artery , patent foramen ovale , pregnancy , genetics , migraine , biology
Pulmonary arteriovenous malformation (PAVM) is a rare finding in a fetus and may lead to cardiac failure and hydrops. Because of the variable outcome of the few previously reported cases, perinatal management and parent counseling are difficult. The case presented a large PAVM complicated by severe cardiomegaly with a successful postnatal treatment. A 20-year-old woman was referred at 23w+1d of gestation due to suspicion of congenital heart malformation. US scan revealed normal fetal growth and amniotic fluid. The heart was in normal location with a severe cardiomegaly (CTAR: 0.628). Pulmonary trunk (PT) and right pulmonary artery (RPa) were dilated with retrograde perfusion through the ductus arteriosus. Turbulent flow was found in the right lung connecting the RPa with a dilated left pulmonary vein, and left-to-right shunt through the foramen ovale. Doppler of the PT revealed a velocity of 72 cm/s and high velocity at the fistula (260 cm/s) with low pulsatility. The descending aorta and umbilical artery showed high pulsatility but normal peak velocity, with no reversal end-diastolic flow. The ductus venosus had no reverse ‘‘a’’ wave. No signs of hydrops or other malformations were found. The fetus was monitored every 2 weeks, cardiac function remained unchanged. The mother was sent to a centre with pediatric cardiosurgery option. At 38 weeks a female fetus was vaginally delivered (weight 3270g; Apgar score 8-8 at 1-5 min) and then referred to a neonatal intensive care unit at a pediatric hospital. The image studies confirm a fistula, then, a surgical excision was performed and the fistula was closed. The newborn evolved favourably, without hemodynamic or neurological alterations at six months of age. This is one of the few published cases of prenatal diagnosis of a large PAVM with successful postnatal treatment resulted in survival with adequate life quality. Close monitoring of the fetus and planned multidisciplinary management seems to be essential for the outcome.