Clinical features and diagnosis of pulmonary hypertension in adults
- Lewis J Rubin, MD
Lewis J Rubin, MD
- Adjunct Professor of Medicine
- College of Physicians and Surgeons, Columbia University
- William Hopkins, MD
William Hopkins, MD
- Associate Professor of Medicine
- University of Vermont College of Medicine
Patients suspected of having pulmonary hypertension (PH) undergo extensive diagnostic testing. The purpose of diagnostic testing is to confirm that PH exists and identify the underlying cause.
The clinical features, diagnostic evaluation, and diagnostic criteria for PH are reviewed here. Classification, epidemiology, etiologies, pathogenesis, natural history, treatment, and prognosis are discussed separately. (See "Overview of pulmonary hypertension in adults" and "Pathogenesis of pulmonary hypertension" and "Treatment of pulmonary hypertension in adults".)
Symptoms and signs of pulmonary hypertension (PH) may be difficult to recognize because they are nonspecific. Initially, patients present with exertional dyspnea and fatigue. Because PH is progressive, the presentation evolves over time so that patients may eventually develop the signs and symptoms of severe pulmonary hypertension with overt right ventricular failure (eg, exertional chest pain or syncope and congestion including peripheral edema, ascites, and pleural effusion). The diagnosis is often delayed because the presenting features of PH are frequently attributed incorrectly to age, deconditioning, or a coexisting or alternate medical condition. As a result, PH is often not suspected until symptoms become severe or serious. It has been estimated that more than 20 percent of patients have symptoms of PH for longer than two years before it is recognized . This is particularly prevalent among patients younger than 36 years and in those with co-existing medical conditions.
Symptoms — The initial symptoms of PH are the result of an inability to adequately increase cardiac output during exercise [2,3]. These include exertional dyspnea, lethargy, and fatigue. Additional symptoms emerge as the PH progresses and right ventricular hypertrophy and failure develop. (See "Cor pulmonale", section on 'Symptoms'.)
These include the following:
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- CLINICAL MANIFESTATIONS
- DIAGNOSTIC EVALUATION
- Our approach
- Diagnostic tests
- - Chest radiograph
- - Electrocardiography
- - Echocardiography
- Estimating pulmonary artery pressure
- Distinguishing group 2 pulmonary hypertension
- - Pulmonary function tests
- - Overnight oximetry
- - Polysomnography
- - V/Q scan
- - Laboratory tests
- - Exercise testing
- - Right heart catheterization
- DIAGNOSTIC CRITERIA
- Group 1 - Pulmonary arterial hypertension
- Group 2 - PH due to left heart disease
- Group 3 - PH due to chronic lung disease and/or hypoxemia
- Group 4 - PH due to chronic thromboembolic pulmonary hypertension
- Group 5 - PH due to multifactorial mechanisms
- Exercise-induced pulmonary hypertension
- DIFFERENTIAL DIAGNOSIS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS