Introduction
Congenital Lobar Emphysema (CLE) is a rare and life-threatening anomaly of the lung characterized by hyperinflation of usually one or sometimes more of the lung lobes. It sincidence rate is around 1 in the 20,000 to 30,000 live births with 3:1 male preponderance1,2. The hyperinflated lobe compresses the surrounding normal lung tissue causing atelectasis leading to ventilation-perfusion mismatch and hypoxia. It is usually manifested in early infancy with respiratory distress3,4. The most common site of lobe involvement is the left upper lobe (43%), followed by the right middle lobe(32%) and right upper lobe (21%).Congenital Heart Defects (CHD) can present in around 14 – 20% of cases of CLE, and Ventricular Septal Defect (VSD) is the most commonly associated anomaly1,4. The clinical presentation of CLE and most CHDs are similar and overlapping in the infantile age, and CHD being far more common gets detected early.Often the diagnosis of CLE may get overlooked in presence of the CHD5,6. We encountered a case where the presence of CLE was established after the corrective repair of the large perimembranous VSD with moderate Patent Ductus Arteriosus (PDA).