Introduction
Congenital lobar emphysema (CLE) is a rare and life-threatening anomaly of the lung characterized by hyperinflation of at least one lung lobe. The incidence of CLE is approximately 1 in every 20,000 to 30,000 live births, with a 3:1 male preponderance1,2. The hyperinflated lobe compresses the surrounding normal lung tissue, causing atelectasis and leading to ventilation-perfusion mismatch and hypoxia. It usually presents 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 the right upper lobe (21%).
Congenital heart defects (CHD) can present in around 14–20% of CLE cases, with ventricular septal defect (VSD) being the most commonly associated anomaly1,4. The clinical presentation of CLE and most CHDs is similar and manifests in infancy.As CHD is more common, it is usually detected first.Often, the diagnosis of CLE may get overlooked in the presence of CHD5,6. Here, we present a case in which the presence of CLE was established after the corrective repair of a large perimembranous VSD with a moderate patent ductus arteriosus (PDA).