Embolization of aortopulmonary collaterals in a patient with idiopathic pulmonary hypertension.

Abstract

Introduction: Idiopathic pulmonary arterial hypertension (IPAH) is a disease characterized by increased pressure in the pulmonary arteries, which can trigger the formation of aortopulmonary collateral arteries (MAPCAs) as a compensatory mechanism. These MAPCAs, anomalous vessels connecting the aorta to the pulmonary vascular bed, can contribute to disease progression. MAPCA embolization is a therapeutic strategy to reduce right ventricular overload through these alternative pathways, thus improving control of pulmonary hypertension.

Case Report: A 28-year-old woman was diagnosed with idiopathic pulmonary arterial hypertension (IPAH). She presented with dyspnea, hemoptysis, and asthenia. Imaging studies revealed the presence of multiple aortopulmonary collateral arteries (MAPCAs) and thyrocervical collateral to the right pulmonary artery, identified as the cause of hemoptysis. Right heart catheterization confirmed severe pulmonary arterial hypertension (mean pulmonary arterial pressure 66 mmHg), and aortography demonstrated two right MAPCAs. MAPCA embolization was performed using coils and an Amplatzer device, achieving occlusion. The patient showed a remarkable clinical improvement post-procedure, with resolution of hemoptysis and increased functional capacity.

Conclusions: Embolization of aortopulmonary collateral arteries (MAPCAs), combined with optimal medical treatment, is a promising therapeutic strategy in patients with idiopathic pulmonary arterial hypertension (IPAH). This intervention not only relieves symptoms associated with right heart failure but also controls complications such as hemoptysis, expanding its potential use in selected cases of IPAH.