Ask the Expert: Diagnosis of PAH from a Pulmonologist Perspective

Advances in Pulmonary Hypertension

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关键词: Cardiology;    Pulmonology;    Rheumatology;    Pediatrics;    WHO Group 1- Pulmonary Arterial Hypertension;    Echocardiography;    Pulmonary Function Tests;    Surgical Interventions;    Anatomy;    Physiology/Pathophysiology;    symptoms;    Research;    Genetics;    Associated Diseases;    Connective Tissue Diseases;    Scleroderma;    HIV Infection;    Patent Ductus Arteriosus;    hematologic disorder;    Non-invasive estimate of PVR;    pulmonary capillary wedge pressure;    Pulmonary vascular resistance;    Cardiac Output;    Dlco;    Spirometry;    endothelial dysfunction;    tricuspid regurgitation;    edema;    hypoxia;    patient-reported outcomes;    PHAROS;    Epidemiology;    Screening;   

DOI  :  

学科分类：医学（综合）

来源: Pulmonary Hypertension Association

【 摘 要 】

The axiom “diagnosis drives treatment” is especially important when evaluating patients with pulmonary hypertension (PH). Patients with PH, defined as a mean pulmonary arterial pressure (mPAP) greater than 25 mm Hg, are placed into 2 main categories based on hemodynamic measurements, pathophysiological mechanisms, clinical presentation, and therapeutic options. Patients with Group 1 disease, referred to as pulmonary arterial hypertension (PAH), have an mPAP >25 mm Hg with a normal pulmonary wedge pressure of 15 mm Hg or less and a pulmonary vascular resistance (PVR) greater than 3 Wood units. Group 1 consists of 3 main subgroups: idiopathic PAH, familial PAH, and PAH associated with known risk factors or associated conditions (APAH).1 The second category of patients with PH (Groups 2-5) includes patients with PH associated with left heart disease (Group 2), disorders of the respiratory system and/or hypoxemia (Group 3), chronic thromboembolic disease (Group 4), and obstruction of pulmonary vessels by processes including inflammation, mechanical obstruction, or extrinsic compression (Group 5).1 

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