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Right arrow Congenital - cyanotic

Ann Thorac Surg 2005;80:e17-e19
© 2005 The Society of Thoracic Surgeons

Case report

Staged Correction of Double Discordance, Situs Inversus, and Absence of True Pulmonary Arteries

Federico Brunelli, MD a , * , Andrea Amaducci, MD a , Giordano Tasca, MD a , Zen Mhagna, MD a , Bruno Amari, MD b

a Division of Cardiac Surgery, Brescia, Italy
b Division of Cardiac Anesthesia and Postoperative Intensive Care, Private Non-Profit Hospital Poliambulanza, Brescia, Italy

Accepted for publication June 7, 2005.

* Address correspondence to Dr Brunelli, Division of Cardiac Surgery, Private Non-Profit Hospital Poliambulanza, via Bissolati 57, Brescia, 25125 Italy (Email: cch-segreteria.poli{at}poliambulanza.it).

The case of a young boy with an extremely rare and complex cardiac malformation is described. Situs inversus, dextrocardia, double discordance with pulmonary atresia, absence of true pulmonary arteries, and systemico-pulmonary collateral arteries was the original diagnosis. Three preparatory operations were necessary to reconstruct and unifocalize the pulmonary vascular tree. At the age of 7 years complete correction with intraoperative stenting of the left neopulmonary artery was successfully performed. After more than 3 years from the definitive operation, the patient is alive with no physical limitations.






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