Congenital Pulmonary Airway Malformation of the Left Upper Lobe Associated With Dextrocardia in a Neonate: A Case Report

Congenital pulmonary airway malformation (CPAM) is a rare developmental lung anomaly that may present in the neonatal period with respiratory distress, and its association with dextrocardia is exceptional, potentially leading to diagnostic challenges. We report the case of a 26-day-old male neonate, born at term, admitted for cyanosis and respiratory distress. Clinical examination revealed tachypnea (68 breaths per minute), oxygen desaturation (SpO₂ 65% in room air), and signs of respiratory distress, with cardiac auscultation suggesting right-sided heart sounds. Imaging studies demonstrated a cystic lesion of the left upper lobe consistent with congenital pulmonary airway malformation, associated with cardiac dextroposition due to mediastinal shift, rather than true dextrocardia. The patient received supportive care and broad-spectrum antibiotic therapy; however, the clinical course was marked by progressive respiratory deterioration complicated by severe pulmonary infection, ultimately resulting in death before surgical intervention could be undertaken. CPAM results from abnormal airway development, and it is associated with cardiac dextroposition due to mediastinal shift, rather than true dextrocardia. This case highlights the importance of considering CPAM in neonates presenting with respiratory distress and emphasizes the crucial role of imaging, particularly computed tomography, in establishing the diagnosis and guiding management.