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Incidence of congenital cystic malformation of lungs ranges from 1 in 11,000 to 1 in 35,000 live birth. Concurrence of Congenital Pulmonary Airway Malformation (CPAM) with congenital heart disease is even infrequent. Even rarer, is the incidence of congenital pulmonary vascular and cystic parenchymal lesions with cardiac defects. The non functioning lung tissue is separated from bronchial tree and vascularised by aberrant artery from the systemic circulation. Simultaneous repair of cardiac and pulmonary defects, though challenging, but are preferable because of surgery being done in single stage with better cosmesis. A 10-yearold female child presented to the hospital with recurrent chest infections and respiratory distress while running since five years of age. The patient underwent atrial septal defect closure and right lower lobectomy for CPAM in one stage through midline sternotomy. We conclude that such complex cases can be managed successfully with meticulous planning and multidisciplinary team approach.