Abstract Background Idiopathic pulmonary artery dilatation (IPAD) is a rare condition characterized by isolated dilatation of the pulmonary artery in the absence of congenital heart defects or other secondary causes. Here, we report a rare case of IPAD complicated by severe aortic stenosis (AS), managed with simultaneous aortic valve replacement (AVR) and pulmonary artery graft replacement. Case Summary A 74-year-old woman with type 1 diabetes was followed for IPAD. Initial evaluations ruled out shunt diseases and secondary causes of pulmonary artery dilatation. Over five years of follow-up, mild AS progressed to severe AS, leading to exertional dyspnea. A heart team decided on simultaneous AVR with Inspiris Resilia 23mm and pulmonary artery replacement with an ePTFE graft. Pulmonary regurgitation was also repaired. Postoperative recovery was uneventful, and the patient was discharged on postoperative day 22. This case highlights the importance of multidisciplinary approaches in managing rare vascular pathologies and concurrent cardiac diseases. Discussion This is the first report of IPAD with severe AS managed via simultaneous AVR and pulmonary artery graft replacement. This report underscores the importance of individualized management strategies in rare cases where standard guidelines are unavailable.
Naruse et al. (2026) studied this question.