Aortic coarctation associated with alveolar capillary dysplasia and misalignment of the pulmonary veins.
After surgical repair of an aortic coarctation a term infant presented with severe pulmonary hypertension and cyanosis unresponsive to treatment including extracorporeal membrane oxygenation. The atypical clinical course became apparent once the accompanying diagnosis of congenital alveolar capillary dysplasia with misalignment of the pulmonary veins had been established at autopsy. In infants with congenital heart defects and with refractory pulmonary hypertension unexplainable on anatomic findings, a lung biopsy at the time of cardiac repair should be considered to avoid further therapies that would not alter the uniformly fatal course of this rare lung disorder.
Gamillscheg
Andreas
A
Department of Pediatrics, Division of Pediatric Cardiology, Medical University of Graz, Auenbruggerplatz 30, Graz 8036, Austria. andreas.gamillscheg@meduni-graz.at
Zobel
Gerfried
G
Spuller
Ekkehard
E
Reiterer
Friedrich
F
Beitzke
Albrecht
A
D001017:Aortic Coarctation / Q000453:epidemiology* / Q000601:surgery; D004108:Dilatation, Pathologic; D015199:Extracorporeal Membrane Oxygenation; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D042601:Lymphatic Vessels / Q000473:pathology; D010547:Persistent Fetal Circulation Syndrome / Q000209:etiology* / Q000628:therapy; D011650:Pulmonary Alveoli / Q000473:pathology*; D011667:Pulmonary Veins / Q000002:abnormalities*