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The Pathophysiology, Mechanism, Diagnosis, and Management of Pulmonary Arterial Hypertension: A Comprehensive Literature Review

Gangavelli A, Fabiano RC, Riad M, Kaur G, Rai D, Gomberg-Maitland M

US cardiology, 2026

Abstract

Pulmonary arterial hypertension is a rare group of diseases with distinct etiologies affecting all ages, with predominant prevalence in young women. The key common aspect of the disease process is pulmonary arterial remodeling and vasoconstriction, leading to right heart failure. The symptoms most commonly manifest as shortness of breath and progressively worsening exercise intolerance. Early diagnosis, identification of the underlying cause, and targeted treatments aimed at mitigating right heart failure are crucial in management. Although new therapies have emerged recently, it is still a condition with high morbidity and mortality that requires specialized multidisciplinary care. The aim of this study is to review the current literature on key aspects of pulmonary arterial hypertension.

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