Simultaneous aortic valve replacement and pulmonary artery graft replacement in a patient with idiopathic pulmonary artery dilatation and severe aortic stenosis: a case report.

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 5 years of follow-up, mild AS progressed to severe AS, leading to exertional dyspnoea. A heart team decided on simultaneous AVR with Inspiris Resilia 23 mm and pulmonary artery replacement with an expanded polytetrafluoroethylene (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.