Absent aortic and dysplastic pulmonary valves associated with ventricular septal defect in fetal hydrops.
We describe worsening cardiac failure in a hydropic male fetus with a large ventricular septal defect associated with severe pulmonary and mild aortic regurgitation detected prenatally by echocardiography. The hemodynamic transition after emergency cesarean section at 31 weeks of gestation resulted in amelioration of the pulmonary incompetence but exacerbation of aortic insufficiency causing coronary steal. Death followed withdrawal of support 4 hours after delivery. Autopsy revealed absent aortic valve cusps, dysplastic pulmonary valve, and a large ventricular septal defect.
D000015:Abnormalities, Multiple / Q000175:diagnosis; D000328:Adult; D001021:Aortic Valve / Q000002:abnormalities*; D001344:Autopsy; D017809:Fatal Outcome; D005260:Female; D005865:Gestational Age; D006330:Heart Defects, Congenital / Q000175:diagnosis*; D006345:Heart Septal Defects, Ventricular / Q000175:diagnosis*; D006801:Humans; D015160:Hydrops Fetalis / Q000000981:diagnostic imaging*; D007231:Infant, Newborn; D011247:Pregnancy; D011256:Pregnancy Outcome; D011664:Pulmonary Valve / Q000002:abnormalities*; D016216:Ultrasonography, Prenatal