Combined Heart-Lung Transplant for Pulmonary Artery Aneurysm Secondary to Pulmonary Arterial Hypertension

BACKGROUND: Pulmonary artery aneurysms (PAAs) are rare occurring in approximately 1 in 14,000 cases. While PAA is frequently associated with pulmonary arterial hypertension (PAH), the optimal management strategy remains undefined due to the rarity of the disease. CASE SUMMARY: We report a case of a 58-year-old female with longstanding idiopathic PAH who underwent combined heart-lung transplantation to treat a rapidly expanding 8.95-cm PAA with associated cor pulmonale and severe pulmonic valve regurgitation. Graft function remained excellent; however, an ischemic stroke in the postoperative period led to significant morbidity. DISCUSSION: Despite the known association of PAA with PAH, specific treatment guidelines are lacking. Delay in intervention can lead to poor surgical candidacy and adverse outcomes. TAKE-HOME MESSAGES: PAA may progress despite stable PAH hemodynamics. Combined heart-lung transplantation can be a viable option for select patients with severe PAH, giant PAA, cor pulmonale, and severe pulmonic valve regurgitation.