Decellularized Pulmonary Homograft Repair of Idiopathic Main Pulmonary Artery Aneurysm in SC Hemoglobinopathy

Pulmonary artery aneurysm is a rare and potentially fatal condition, often diagnosed incidentally. The lack of specific guidelines makes it difficult to define clear intervention criteria and the ideal reconstructive technique. Decellularized pulmonary homografts (DPHs) have emerged as a promising alternative, although they are still scarcely reported.

A 48-year-old previously healthy man presented with mediastinal widening on a routine chest x-ray. Work-up revealed an idiopathic main pulmonary aneurysm measuring 52 mm, associated with moderate pulmonary valve stenosis. He underwent aneurysm resection and valve replacement with a DPH (size 25). The immediate postoperative course was satisfactory, but he developed severe pneumonia and was incidentally diagnosed with SC hemoglobinopathy.

The use of a DPH was appropriate in this setting, particularly considering the patient's age and the presence of SC hemoglobinopathy, a condition associated with increased risk of pulmonary hypertension, thrombotic events, and vascular inflammation. Follow-up demonstrated a competent graft with no thromboembolic complications, reinforcing the potential benefit of this technique.

This case highlights the use of a DPH as a safe and effective surgical option for an idiopathic main pulmonary artery aneurysm, especially in patients with associated hematologic conditions, emphasizing the importance of strict follow-up and individualized therapeutic strategies.