Surgical Management of Giant Pulmonary Artery Aneurysm in Patients with Severe Pulmonary Arterial Hypertension

Patients with pulmonary arterial hypertension (PAH) may develop large pulmonary artery aneurysms (PAA) which may be complicated by rupture, dissection or intravascular thrombus formation. These patients were traditionally considered for heart-lung transplantation but more recently, there have been reports of successful lung transplantation with reconstruction of the pulmonary artery (PA). We present two patients who underwent successful transplantation for PAH with giant PAA. One patient had end stage PAH and right pulmonary artery atresia complicated by a giant main PAA. This patient underwent bilateral sequential lung transplantation with concurrent pulmonary artery reconstruction. Another patient had end stage PAH with giant PAA on a background of D-transposition of the great arteries who had a Mustard repair at 9 months of age. This patient underwent heart-lung transplantation. Both heart-lung transplantation and lung transplantation with reconstruction of the pulmonary should be considered as a treatment option for patients with PAH with PAA.