Review Article

Pulmonary Arterial Hypertension: Evaluation and Management

Authors: Anne V. LaRaia, MD, Aaron B. Waxman, MD, PhD

Abstract

Pulmonary arterial hypertension (PAH), a rare disease involving the pulmonary vascular circuit, is defined as an elevation in pulmonary arterial pressures and is characterized by symptoms of dyspnea, chest pain, and syncope. If left untreated, the disease carries a high mortality rate, with the most common cause of death being decompensated right heart failure. Over the past 5 years, there have been significant advances in this field in regards to understanding the pathogenesis, diagnosis, and classification of PAH. The availability of newer drugs has resulted in a radical change in the management of this disease with significant improvement in both quality of life and mortality. Ongoing research promises to lead to a more comprehensive understanding of the genetics, etiology, and pathogenesis of pulmonary arterial hypertension, which may ultimately translate into more effective therapeutic options.


Key Points


* The typical signs and symptoms of patients presenting with unexplained dyspnea and suspected pulmonary hypertension are reviewed.


* The components of the clinical evaluation for patients with suspected pulmonary arterial hypertension are reviewed.


* The current therapeutic options in treating pulmonary arterial hypertension are reviewed.

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