Aortic coarctation associated with alveolar capillary dysplasia and misalignment of the pulmonary veins.

Abstract:

: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.

journal_name

Pediatr Cardiol

journal_title

Pediatric cardiology

authors

Gamillscheg A,Zobel G,Spuller E,Reiterer F,Beitzke A

doi

10.1007/s00246-007-9063-5

subject

Has Abstract

pub_date

2008-01-01 00:00:00

pages

191-4

issue

1

eissn

0172-0643

issn

1432-1971

journal_volume

29

pub_type

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