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 "Classification and prognosis of pulmonary hypertension in adults" and "The epidemiology and pathogenesis of pulmonary arterial hypertension (Group 1)" 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 "Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Epidemiology, pathogenesis, and diagnostic evaluation in adults".)
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- Brown LM, Chen H, Halpern S, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest 2011; 140:19.
- Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet 2003; 361:1533.
- Peacock AJ. Primary pulmonary hypertension. Thorax 1999; 54:1107.
- Mesquita SM, Castro CR, Ikari NM, et al. Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension. Am J Med 2004; 116:369.
- Rich S, McLaughlin VV, O'Neill W. Stenting to reverse left ventricular ischemia due to left main coronary artery compression in primary pulmonary hypertension. Chest 2001; 120:1412.
- Kawut SM, Silvestry FE, Ferrari VA, et al. Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension. Am J Cardiol 1999; 83:984.
- Fishman AP. Pulmonary hypertension and cor pulmonale. In: Pulmonary Disease, Fishman AP (Ed), McGraw Hill, New York 1989.
- Tang KJ, Robbins IM, Light RW. Incidence of pleural effusions in idiopathic and familial pulmonary arterial hypertension patients. Chest 2009; 136:688.
- Weitzenblum E, Apprill M, Oswald M, et al. Pulmonary hemodynamics in patients with chronic obstructive pulmonary disease before and during an episode of peripheral edema. Chest 1994; 105:1377.
- CAMPBELL EJ, SHORT DS. The cause of oedema in "corpulmonale". Lancet 1960; 1:1184.
- Richens JM, Howard P. Oedema in cor pulmonale. Clin Sci (Lond) 1982; 62:255.
- Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37:67.
- McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation 2009; 119:2250.
- Bossone E, Paciocco G, Iarussi D, et al. The prognostic role of the ECG in primary pulmonary hypertension. Chest 2002; 121:513.
- Ahearn GS, Tapson VF, Rebeiz A, Greenfield JC Jr. Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. Chest 2002; 122:524.
- Bossone E, Bodini BD, Mazza A, Allegra L. Pulmonary arterial hypertension: the key role of echocardiography. Chest 2005; 127:1836.
- Hinderliter AL, Willis PW 4th, Long W, et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Primary pulmonary hypertension. Am J Cardiol 1999; 84:481.
- Mikami T, Kudo T, Sakurai N, et al. Mechanisms for development of functional tricuspid regurgitation determined by pulsed Doppler and two-dimensional echocardiography. Am J Cardiol 1984; 53:160.
- Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 1984; 70:657.
- Arkles JS, Opotowsky AR, Ojeda J, et al. Shape of the right ventricular Doppler envelope predicts hemodynamics and right heart function in pulmonary hypertension. Am J Respir Crit Care Med 2011; 183:268.
- Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC), European Respiratory Society (ERS), International Society of Heart and Lung Transplantation (ISHLT), et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2009; 34:1219.
- Fisher MR, Forfia PR, Chamera E, et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179:615.
- Rich JD, Shah SJ, Swamy RS, et al. Inaccuracy of Doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension: implications for clinical practice. Chest 2011; 139:988.
- Amsallem M, Sternbach JM, Adigopula S, et al. Addressing the Controversy of Estimating Pulmonary Arterial Pressure by Echocardiography. J Am Soc Echocardiogr 2016; 29:93.
- Jacobs W, Konings TC, Heymans MW, et al. Noninvasive identification of left-sided heart failure in a population suspected of pulmonary arterial hypertension. Eur Respir J 2015; 46:422.
- Berger M, Haimowitz A, Van Tosh A, et al. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 1985; 6:359.
- Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol 2003; 41:1028.
- Meyer FJ, Ewert R, Hoeper MM, et al. Peripheral airway obstruction in primary pulmonary hypertension. Thorax 2002; 57:473.
- Minai OA, Pandya CM, Golish JA, et al. Predictors of nocturnal oxygen desaturation in pulmonary arterial hypertension. Chest 2007; 131:109.
- Tunariu N, Gibbs SJ, Win Z, et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med 2007; 48:680.
- Leuchte HH, Baumgartner RA, Nounou ME, et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care Med 2006; 173:744.
- Fijalkowska A, Kurzyna M, Torbicki A, et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 2006; 129:1313.
- Blyth KG, Groenning BA, Mark PB, et al. NT-proBNP can be used to detect right ventricular systolic dysfunction in pulmonary hypertension. Eur Respir J 2007; 29:737.
- Leuchte HH, El Nounou M, Tuerpe JC, et al. N-terminal pro-brain natriuretic peptide and renal insufficiency as predictors of mortality in pulmonary hypertension. Chest 2007; 131:402.
- Tolle JJ, Waxman AB, Van Horn TL, et al. Exercise-induced pulmonary arterial hypertension. Circulation 2008; 118:2183.
- Kovacs G, Berghold A, Scheidl S, Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 2009; 34:888.
- Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54:S55.
- Grünig E, Lichtblau M, Ehlken N, et al. Safety and efficacy of exercise training in various forms of pulmonary hypertension. Eur Respir J 2012; 40:84.
- Mathai SC, Puhan MA, Lam D, Wise RA. The minimal important difference in the 6-minute walk test for patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 2012; 186:428.
- Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 62:D34.
- Halpern SD, Taichman DB. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest 2009; 136:37.
- Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013; 62:D42.
- Rich S (ed). Executive summary from the World Symposium on Primary Pulmonary Hypertension, Evian, France, September 6-10, 1998, co-sponsored by The World Health Organization.
- Herve P, Lau EM, Sitbon O, et al. Criteria for diagnosis of exercise pulmonary hypertension. Eur Respir J 2015; 46:728.
- 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
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS