Severe pulmonary arterial stenoses in Conradi-Hünermann disease.
A 5-month-old male infant with chondrodystrophia punctata type Conradi-Hünermann showed electrocardiographically an increasing right ventricular hypertrophy. Echocardiographically an enormous enlarged right ventricle with signs of supra-systemic pressure was found. No other intracardiac defects could be seen. Angiography showed severe peripheral pulmonary arterial stenoses of both main arteries. Balloon-dilatation or surgical intervention was supposed to have no beneficial effect. At the age of 6 months the patient died.
D000792:Angiography; D002806:Chondrodysplasia Punctata / Q000145:classification / Q000150:complications* / Q000000981:diagnostic imaging; D003251:Constriction, Pathologic; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging