Suggested algorithm for the evaluation of suspected pulmonary hypertension in primary care. Strange G, et al. Bridges CB, *—Also includes 1′ (pulmonary venoocclusive disease and/or pulmonary capillary hemangiomatosis) and 1″ (persistent pulmonary hypertension of the newborn). For information about the SORT evidence rating system, go to Bethesda, MD 20894, Copyright 2013 Mar 15;187(6):576-83. doi: 10.1164/rccm.201211-2090CI. Pulmonary arterial hypertension is most often diagnosed in its advanced stages because of the nonspecific nature of early symptoms and signs. Kim NH, 25. Ann Rheum Dis. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Stringham R, 2014;73(7):1340–1349. Global Initiative for Chronic Obstructive Lung Disease. 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). 2020 Nov 11;10(4):2045894020939842. doi: 10.1177/2045894020939842. 2010;35(5):1079–1087. Get Permissions, Access the latest issue of American Family Physician. Talwalkar A, 2015 Sep;46(3):738-49. doi: 10.1183/09031936.00191014. Evidence supporting targeted treatment is available only for pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.1,22 For the other classes, there are no data on treatment effectiveness, and for some therapies, there is evidence of harm. Clipboard, Search History, and several other advanced features are temporarily unavailable. Barst RJ, ; Semin Respir Crit Care Med. Age-associated increases in pulmonary artery systolic pressure in the general population. Criteria for Diagnosis of Hypertension and Guidelines for Follow-up. Perioperative assessment of patients with pulmonary hypertension should include echocardiographic assessment of right ventricular function. Redfield MM. The changing landscape of pulmonary arterial hypertension and implications for patient care. BMJ Open. George MG, 2014;190(7):818–826. Recent data in normal subjects show mean pulmonary artery pressure is 14.0±3.3 mmHg. doi: 10.15420/icr.2020.17. Pulmonary Hypertension: Diagnosis and Treatment. 2020 Nov 17;9(22):e018123. 36. 18. Shah NR. Gin K, Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Archer SL, Chronic thromboembolic pulmonary hypertension. 2014;146(2):476–495. Accessed June 10, 2016. Hurd SS, Hypertension Canada continues to emphasize the use of out-of-office measurements to rule out white coat hyper-tension in subjects with increased BP in the office . Vachiéry JL, bdunlap@eriefamilyhealth.org). et al. Pengo V, Pulmonary hypertension in chronic lung diseases. Am Fam Physician. April 25, 2013. http://effectivehealthcare.ahrq.gov/index.cfm/search-for-guides-reviews-and-reports/?productid=1479&pageaction=displayproduct. Tian L, Bogaard HJ, Immediate, unlimited access to all AFP content. In an observational U.S. cohort study, approximately 60% of echocardiography estimates of systolic pulmonary arterial pressure had a difference of more than 10 mm Hg vs. catheterization.24 On meta-analysis, the correlation between systolic pulmonary arterial pressure on echocardiography compared with heart catheterization was 0.70 (95% CI, 0.67 to 0.73).23, Chronic lung disease and sleep disorders (including chronic obstructive pulmonary disease and obstructive sleep apnea), High cardiac output states (e.g., anemia, hyperthyroidism), Left heart disease, including heart failure with preserved or reduced ejection fraction, Volume overload, particularly in heart failure or in the setting of dialysis and chronic kidney disease. Bennett NM, 2009;120(2):e13]. There is limited evidence to support screening of asymptomatic individuals. Accessed August 18, 2015. REVEAL registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension. Control of fluid volume and diuretic therapy are essential, particularly in patients with a history of volume overload or right heart failure.10 Vasodilators are not recommended for the treatment of pulmonary hypertension due to left heart disease.10,26, The treatment of pulmonary hypertension due to lung disease should focus on managing the underlying lung disease and optimizing treatment of other comorbidities.3,11 Lung disease should be treated according to the best available evidence.28,29 Patients with COPD and arterial oxygen pressures less than 60 mm Hg should receive supplemental oxygen, which may improve mortality by lowering pulmonary arterial pressures.11,29 Patients should be screened for obstructive sleep apnea and treated when necessary.2,30 Patients with hypoxic lung disease may benefit from supplemental oxygen to maintain saturation greater than 90%.11 The use of vasodilators in chronic lung disease may worsen ventilation-perfusion mismatching.11,26 Patients with chronic lung disease and severe pulmonary hypertension should consult with a subspecialist.11, A pulmonary endarterectomy performed at a center of excellence can be curative and is first-line therapy in patients with chronic thromboembolic pulmonary hypertension who are surgical candidates. Chung L, 2013;62(25 suppl):D100–D108. Definitions and diagnosis of pulmonary hypertension. 13. See the CME Quiz Questions. Lau EM, Godinas L, Sitbon O, Montani D, Savale L, Jaïs X, Lador F, Gunther S, Celermajer DS, Simonneau G, Humbert M, Chemla D, Herve P. Eur Respir J. 2008;359(21):2254–2265. Right Atrial Pressure During Exercise Predicts Survival in Patients With Pulmonary Hypertension. Pulmonary hypertension is diagnosed by systematically evaluating the breathless patient and screening high risk groups. Bridges CB, Am J Respir Crit Care Med. A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. Dorfmüller P, 32. Humbert M, Archer SL, et al. Chung L, Curtis JR, Epub 2016 Mar 10. et al. Agency for Healthcare Research and Quality. There is still insufficient evidence to add an exercise criterion to this definition. Pulmonary hypertension due to lung diseases and/or hypoxia (group 3) 9.1 Diagnosis 9.2 Therapy 10. García-Río F, 8600 Rockville Pike Eur Heart J. Hager WD, Collins I, Tate JP, Azrin M, Foley R, Lakshminarayanan S, Rothfield NF. Tian L, Janda S, Jenkins DP, This review updates a previous article on this topic by Stringham and Shah. Humbert M. J Am Coll Cardiol. www.escardio.org Declaration of COI • Lectures/consultancy and/or research fees: • Actelion • Bayer • Janssen • Orphan Pharmaceutics • Sanofi • United Therapeutics. Torbicki A, 2021 Feb 15;16:e01. Accessibility et al. Yuan JX, Updated clinical classification of pulmonary hypertension [published correction appears in. Living with pulmonary hypertension: unique insights from an international ethnographic study. 2005;111(5):534–538. An official American Thoracic Society/American Association of Critical-Care Nurses/American College of Chest Physicians/Society of Critical Care Medicine policy statement: the Choosing Wisely. 2012;126(8):975–990. Farber HW, Revel MP(1), Faivre JB, Remy-Jardin M, Delannoy-Deken V, Duhamel A, Remy J. Rich S, Pulmonary hypertension due to left heart diseases. Prins MH, Chest. 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). Pulm Circ. et al. Adir Y, Eur Respir Rev. Pulmonary complications of sickle cell disease. Echocardiography is the recommended initial test in the evaluation of patients with suspected pulmonary hypertension. Pulmonary complications of sickle cell disease. / afp Gladwin MT, Ayala C, Patients with pulmonary hypertension should receive seasonal influenza vaccination and age-appropriate pneumococcal vaccination, unless contraindicated. 2011;97(8):612–622. Galiè N, Gladwin MT, The clinical significance of an mPA 21-24 mm Hg is unclear. Olsson KM, Villamor J. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. / 20. Search dates: May to December 2015. note: This review updates a previous article on this topic by Stringham and Shah.36. 2013;62(25 suppl):D109–D116. Circulation. Pulmonary arterial hypertension: an update on diagnosis and treatment. Barberà JA, Physicians should initially consider the history, clinical findings, and targeted noninvasive testing, particularly echocardiography. 24. Borlaug BA, ; Criteria for diagnosis of exercise pulmonary hypertension The previous definition of exercise pulmonary hypertension (PH) with a mean pulmonary artery pressure (mPAP) >30 mmHg was abandoned because healthy individuals can exceed this threshold at high cardiac output (CO). Machado RF, A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. Redfield MM. Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Humbert M. Right heart catheterisation (RHC) plays a central role in identifying pulmonary hypertension (PH) disorders, and is required to definitively diagnose pulmonary arterial hypertension (PAH). It mimics several other heart and lung conditions. Becker D, Badesch DB, 30. Current guidelines discourage pregnancy and recommend contraception counseling with emphasis on prescribing a long-acting, highly effective method of contraception.34. Klings ES, Hoeper MM, et al. Olsson KM, Patients with chronic thromboembolic pulmonary hypertension should receive lifelong anticoagulation in the absence of contraindications. Eur Heart J. April 25, 2013. http://goldcopd.org/global-strategy-diagnosis-management-prevention-copd-2016/. Yuan JX, It does not provide medical advice, diagnosis or treatment. Hoeper MM, Hinzmann B, 2006;27(9):1106–1113. Agency for Healthcare Research and Quality. Shah NR. Halpern SD, et al. Global strategy for diagnosis, management, and prevention of COPD—2016. Tuder RM, PH is defined as an increase in mean pulmonary artery (mPA) ≥25 mm Hg at rest, as assessed by right heart catheterization. et al. See a GP if you have symptoms of pulmonary hypertension, such as breathlessness and tiredness. Chronic thromboembolic pulmonary hypertension (group 4) 10.1 Diagnosis … Although pulmonary arterial hypertension (group 1) has benefited the most from progress with targeted therapy, this diagnosis is rare and may not be encountered by many family physicians.7 However, in certain populations, such as persons with systemic sclerosis, pulmonary arterial hypertension is more common.8 Treatment is rapidly evolving and usually administered in expert centers. Pulmonary arterial hypertension: screening, management, and treatment: executive summary. A definitive diagnosis cannot be made on echocardiographic abnormalities alone, and some patients require invasive evaluation by right heart catheterization. Exercise-induced pulmonary hypertension: physiological basis and methodological concerns. To allow appropriate therapeutic management, awareness of the various disease entities associated with pulmonary hypertension … L(-1) is superior to mPAP >30 mmHg alone for defining a pathological haemodynamic response of the pulmonary circulation during exercise. In 2013, the classification scheme was updated to recognize five groups of pulmonary hypertension6  (Table 13,4,6). et al. ; Coghlan JG, Arias MA, Adir Y, Gin K, Vachiéry JL. 2014;146(2):449–475.... 2. Vestbo J, Villamor J. Denton CP, Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology. Copyright © 2020 American Academy of Family Physicians. Reprints are not available from the authors. A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. Badesch DB, Swiston J. et al. García-Río F, Eur Respir J. 2004;350(22):2257–2264. Simonneau G, For example, pulmonary hypertension status in patients who also have COPD may be more predictive of mortality than pulmonary function markers, such as forced expiratory volume in one second or diffusing capacity.11 Patients with pulmonary arterial hypertension have a 36% five-year mortality rate.17, The mechanisms that increase pulmonary pressures can act primarily on the pulmonary arterial bed or venous bed, either alone or in combination.3,18 Pulmonary arterial hypertension is characterized by progressive narrowing of distal pulmonary arteries attributed to a variety of pathologic insults, such as arterial vasoconstriction, medial hypertrophy, intimal proliferation, and fibrosis.19 There are some genetic associations, such as BMPR2, but these are insufficient to explain the pathogenesis without other contributing factors.20 Pulmonary hypertension due to left heart disease is primarily a pulmonary venous process and likely results from passive pulmonary venous congestion with vasoconstriction and venous remodeling.3,9 In pulmonary hypertension due to lung disease and/or hypoxia, increases in pulmonary arterial pressures may arise from destruction of the alveolar capillary bed or chronic hypoxic vasoconstriction.3 Chronic thromboembolic pulmonary hypertension develops following thrombotic macrovascular obstruction with subsequent vasoconstriction and remodeling of the pulmonary arterial bed.19, Regardless of the mechanism, persistently increased pulmonary arterial pressure strains the thin-walled right ventricle. Chronic thromboembolic pulmonary hypertension. REVEAL registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension. J Am Coll Cardiol. Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 25 mm Hg. Heart. The role of the right ventricle in pulmonary arterial hypertension. Other important considerations for patients with pulmonary hypertension include influenza and pneumonia immunizations, contraception counseling, preoperative assessment, and mental health. Age-based recommendations for pneumococcal vaccination should be followed, including use of the 23-valent pneumococcal polysaccharide vaccine (Pneumovax) for adults younger than 65 years.1,31,32, Pulmonary hypertension is associated with increased morbidity and mortality during the perioperative period.1,33 The perioperative assessment should include an assessment of functional status, care goals, and alternatives to surgery.33 Echocardiography assessing right ventricular function can help determine surgical risk.33 Surgical management of patients with severe pulmonary hypertension should be performed with subspecialty consultation.1,33, There is an elevated risk of pregnancy complications in women with pulmonary hypertension and particularly pulmonary arterial hypertension. Diagnosis and Management of Pulmonary Hypertension.