[Infantile type Bland-White-Garland syndrome as a cause of death in Rh incompatibility].
The faulty origin of the left coronary artery from the pulmonary artery is with an incidence of 1:300,000 newborns a very rare heart defect. We report a case of a pregnancy with two intrauterine blood transfusions in the 30th and 32nd week of gestation because of Rh-incompatibility and fetal anaemia. Dopplersonographic and echocardiographic parameters were normal. In the 32nd week of gestation delivery was induced (birth weight 2240 g, cord pH value 7.35, Apgar-score 8/9/9). Under a therapy with respiration, blood exchange transfusion and cardiotonic drugs the newborn died in the second week. The autopsy showed a general immaturity, a haemosiderosis of spleen, liver and lungs, a marked cellular jaundice and signs of a multi-organ-failure. The sinus of the pulmonary valve was the origin of the left coronary artery. In case of a seriously impaired pumping action and after exclusion of other heart defects the Bland-White-Garland-Syndrome (BWGS) has to be considered. Although a prenatal diagnosis of BWGS is with high resolution-ultrasound possible, the early postnatal diagnosis seems to be more relevant.
Springer
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C
Frauenklinik der Universität Leipzig.
Horn
L C
LC
Möckel
A
A
Faber
R
R
D000328:Adult; D001805:Blood Transfusion, Intrauterine; D002423:Cause of Death; D003330:Coronary Vessel Anomalies / Q000000981:diagnostic imaging / Q000473:pathology*; D004452:Echocardiography; D004899:Erythroblastosis, Fetal / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D005260:Female; D005313:Fetal Death / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011651:Pulmonary Artery / Q000002:abnormalities / Q000473:pathology; D013577:Syndrome; D016216:Ultrasonography, Prenatal