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Alveolar Capillary Dysplasia with Congenital Misalignment of Pulmonary Vessels

Division of Cardiothoracic Surgery
¹ Department of Pathology, The Chinese University of Hong Kong, Prince of Wales Hospital Hong, Kong SAR, People’s Republic of China

For reprint information contact: Anthony PC Yim, MD Tel: 852 2632 2629 Fax: 852 2647 8273 Email: yimap{at}cuhk.edu.hk, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong SAR, People’s Republic of China.

Alveolar capillary dysplasia with misalignment of pulmonary vessels is an uncommon congenital cause of persistent pulmonary hypertension of the newborn. It is universally fatal, and diagnosis is entirely dependent upon surgical lung biopsy. We present a case of alveolar capillary dysplasia with misalignment of pulmonary vessels occurring in a full-term neonate, emphasizing that early involvement of the thoracic surgeon for a histological diagnosis allows expensive and ineffective treatments to be avoided.