Isolated cardiomegaly in the second trimester as an early sign of fetal hydrops due to intracranial arteriovenous malformation

Abstract

We report the case of a 27-year-old pregnant woman in whom isolated mild fetal cardiomegaly, diagnosed prenatally on sonographic examination at 22 weeks' menstrual age, was the first sign of development of an arteriovenous malformation of the vein of Galen. The arteriovenous malformation was visualized on sonographic examination at 29 weeks' menstrual age; prenatal MRI at 32 weeks confirmed the diagnosis. At 34 weeks' menstrual age, repeat sonographic examination demonstrated polyhydramnios, cardiomegaly, and generalized hydrops with ascites and pleural effusion. After vaginal delivery, the male neonate died of respiratory distress. We present the findings of prenatal gray-scale, color Doppler, and power Doppler sonography; MRI; and postmortem examination.

Clinical relevance of fetal hemodynamic monitoring: Perinatal implications

“…Similar to fetal anemia, large vascularized tumors such as SCT or cerebral AVMs can cause high-output cardiac failure in the fetus, resulting in hydrops and fetal loss [63,64]. Serial evaluation of the combined CO via Doppler interrogation of the fetal outflow tracts and umbilical artery Doppler can help determine prognosis [65].…”

Management und Outcome der Vena-Galeni-Malformation im Kindesalter

“…Bereits intrauterin kann der Shunt ü ber eine VGM zu einer Belastung des Herzens und zur Herzinsuffi zienz f ü hren [29] . Oft ist jedoch pr ä natal das Shuntvolumen gering wegen des im Wesentlichen durch die Plazenta bestimmten niedrigen peripheren Gef ä ß widerstandes des Feten [20] . diagnostiziert werden [40] .…”

State of the art of antenatal diagnosis and management of vein of Galen aneurysmal malformations

“…However, a growing number of publications report diagnosis in the second trimester, 14–16 as in a recent Brazilian case series with an average age of 25 weeks of gestation at diagnosis 17 . Most diagnoses are made by direct visualization of the VGAM, but in rare instances the emergence of cardiac anomalies precedes the diagnosis of the VGAM by several weeks 18,19 …”

“…17 Most diagnoses are made by direct visualization of the VGAM, but in rare instances the emergence of cardiac anomalies precedes the diagnosis of the VGAM by several weeks. 18,19 Treatment with embolization has transformed the vital and neurodevelopmental prognosis of children with VGAM. 20 Prognosis is dependent on the cardiac and cerebral consequences induced by flow disorders.…”