Triple oral combination therapy in patients with idiopathic pulmonary arterial hypertension and recurrent vessel dissection of inoperable pulmonary artery aneurysm

Background: Pulmonary artery aneurysm is a rare condition related to the stress that the vascular wall undergoes with progressive dilation. A clear definition and its prevalence are not available. Pulmonary arterial hypertension is also a life-threatening and progressive condition which requires specific therapies. Case report: We describe the case of a 62-year-old Caucasian woman with idiopathic pulmonary arterial hypertension and inoperable giant pulmonary artery aneurysm associated with recurrent vessel dissection. Diagnosis was simple with the use of echocardiography and computed tomography. Conversely, deciding on the treatment was difficult, because surgeons declined to perform surgical repair of the aneurysm. We treated the patient medically with specific drugs for pulmonary arterial hypertension assuming to reduce the load pressure on the vascular wall of the pulmonary artery. Discussion: We describe a very rare and extremely serious condition characterized by the concomitant presence of pulmonary arterial hypertension appropriately treated with effective triple oral combination therapy and inoperable pulmonary artery aneurysm with recurrent dissection.