TY - CHAP M1 - Book, Section TI - Pulmonary Hypertension A1 - Waxman, Aaron B. A1 - Loscalzo, Joseph A2 - Jameson, J. Larry A2 - Fauci, Anthony S. A2 - Kasper, Dennis L. A2 - Hauser, Stephen L. A2 - Longo, Dan L. A2 - Loscalzo, Joseph PY - 2018 T2 - Harrison's Principles of Internal Medicine, 20e AB - Pulmonary hypertension (PH) is a spectrum of diseases involving the pulmonary vasculature, and defined as an elevation in pulmonary arterial pressures (mean pulmonary artery pressure [PAP] >22 mmHg or an estimated systolic PAP >36 mmHg). Pulmonary arterial hypertension (PAH) is a relatively rare form of PH 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. There have been significant advances in this field in regard to understanding the pathogenesis, diagnosis, and classification of PAH. Despite these significant advances, there is still a substantial delay in diagnosis of up to 2 years. In many cases, patients whose primary complaint is exertional intolerance are frequently misdiagnosed with more common diseases such as asthma or chronic obstructive pulmonary disease. 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. A delay in diagnosis results in an obvious delay in the initiation of appropriate treatment. Clinicians should be able to recognize the signs and symptoms of PH and to complete a systematic workup in at-risk patients. In this way, early diagnosis, prompt treatment, and improved outcomes for patients become achievable. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/19 UR - accessmedicine.mhmedical.com/content.aspx?aid=1172696871 ER -