Association Between the Degree of Severity of Pulmonary Hypertension With the Presence of Pulmonary Artery Aneurysm: A Brief Updated Review for Clinicians

Abstract

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure (mPAP) ≥20 mm Hg at rest as assessed by right cardiac catheterization. It has a median survival nowadays of 6 years, compared to 2.8 years in the 1980s. A pulmonary artery aneurysm (PAA) is the focal dilation of a blood vessel involving all 3 layers of the vessel wall; they have a diameter greater than 4 cm measured in the trunk of the pulmonary artery. PAAs can be classified into proximal (or central) and peripheral. The clinical manifestations of PAA are primarily nonspecific, and most patients remain undiagnosed, even those with large PAA, due to its silent course; however, clinical manifestations occur unless when there are complications such as bronchial or tracheal compression (leading to cough and dyspnea), dissection, or rupture (leading to hemoptysis). PAH is observed in 66% of patients with PAA. PA dissections are usually associated with PAH; 80% of dissections occur in the main pulmonary trunk. Although there is no clear guideline for the best treatment of PAA, surgery is indicated in patients with a pulmonary trunk aneurysm >5.5 cm. It has been observed that patients in the PAH group associated with congenital heart disease tend to develop PAA more commonly. Those with PAH associated with connective tissue disease have a smaller diameter of PA dilation. This report presents a comprehensive review of PAA, discussing critical aspects of the clinical and imaging diagnosis, hemodynamics, and treatment. A comprehensive updated literature review is included; we believe this article will interest cardiopulmonologists.