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.
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Acknowledgment
We are indebted to Prof. Dr. H.H. Popper, Head of the Pulmonary Pathology Working Group of the Department of Pathology, Medical University of Graz, for diagnosing this rare neonatal disorder and for the histological material for this publication.
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Gamillscheg, A., Zobel, G., Spuller, E. et al. Aortic Coarctation Associated with Alveolar Capillary Dysplasia and Misalignment of the Pulmonary Veins. Pediatr Cardiol 29, 191–194 (2008). https://doi.org/10.1007/s00246-007-9063-5
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DOI: https://doi.org/10.1007/s00246-007-9063-5