Surgical Strategies for Unilateral Pulmonary Artery Agenesis: A case series

Unilateral pulmonary artery agenesis, typically affecting the right pulmonary artery, is a rare condition that may lead to congestive heart failure or pulmonary hypertension in infants and early surgical intervention improves outcomes. We report 5 cases from Muscat, Oman, of unilateral pulmonary artery agenesis who underwent surgical correction between November 2019 and August 2023, at an average age of 4.4 months. Most presented with cyanosis or respiratory distress and all had pulmonary hypertension confirmed by imaging. Surgical approaches included Gore-Tex conduit placement, autologous pericardial reconstruction and patent ductus arteriosus unifocalisation. All patients successfully restored pulmonary blood flow with no perioperative mortality. The median intensive unit care stay was 13 days and the median hospital stay was 20 days. Only 1 patient required repeat surgery for graft compression. Over a 27.6-month follow-up period, 4 patients showed regression of pulmonary hypertension and 2 underwent pulmonary angioplasty, reinforcing the benefits of timely intervention.