TY - CHAP M1 - Book, Section TI - Pulmonary Arterial Hypertension A1 - Kim, Nick H. A1 - Mandel, Jess A1 - Smith, Kimberly A. A1 - Yuan, Jason X.-J. A2 - Grippi, Michael A. A2 - Antin-Ozerkis, Danielle E. A2 - Dela Cruz, Charles S. A2 - Kotloff, Robert M. A2 - Kotton, Camille Nelson A2 - Pack, Allan I. PY - 2023 T2 - Fishman’s Pulmonary Diseases and Disorders, 6e AB - Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that results in right heart failure and excess morbidity and mortality without effective treatment. Although rapid progress has resulted in the availability of therapies that can improve the outlook for many patients, true full reversal of disease remains elusive. Furthermore, long delays in disease recognition are common, exposing patients to prolonged suffering and potentially irreversible harm. PAH should not be confused with other mechanisms leading to elevation of pulmonary arterial pressure, such as hypoxemic lung disorders (e.g., chronic obstructive pulmonary disease [COPD], interstitial lung disease [ILD]), left heart disease (e.g., systolic, diastolic, or valvular dysfunctions), or chronic thromboembolism.1,2 Prompt and accurate diagnosis of PAH followed by treatment based on the latest evidence-based guidelines are essential in successfully managing PAH. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2025/03/24 UR - accessmedicine.mhmedical.com/content.aspx?aid=1206603158 ER -