pulmonary hypertension
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Introduction
Chronic disorder with mean pulmonary artery pressure of > 20-25 mm Hg at rest or > 30 mm Hg with exercise & a pulmonary capillary wedge pressure < 15 mm Hg measured during right heart catheterization.*
* see Differential diagnosis: below for causes of acute elevation in pulmonary artery pressure.
Etiology
- left atrial hypertension*
- valvular heart disease
- heart failure
- LV systolic dysfunction
- LV diastolic dysfunction (most common in elderly, USA)[13] (28%)
- left ventricular end-diastolic pressure (LVEDP) > 15 mm Hg[11]
- congenital heart disease
- hypoxemia*
- left to right shunts - atrial septal defect
- vascular diseases of the lung
- connective tissue disease
- HIV1 infection
- portal hypertension, portopulmonary hypertension
- schistosomiasis (world-wide most common cause)
- chronic hemolytic anemia
- sickle cell disease (older patients)[8][15]
- familial, congenital heart disease (see Genetics below)
- pharmaceutical agents: analeptics
- toxin exposure
- idiopathic (primary pulmonary hypertension, group 1) (14%)[13]
* left heart disease & hypoxemia due to chronic lung disease most common causes[3]
* see causes of pulmonary hypertension for more detail
Pathology
- vascular
- elevated mean pulmonary artery pressure due to increased pulmonary vascular resistance
- arterial intimal proliferation & arteriolar constriction secondary to alveolar hypoxia
- disease localized to small pulmonary arterioles resulting in high pulmonary vascular resistance is primary pulmonary arterial hypertension
- increased pulmonary blood flow from left to right shunts
- increased pulmonary venous pressure from left heart failure*
- > 50% of the vascular bed must be occluded to produce clinical pulmonary hypertension
- perivascular parenchymal changes resisting blood flow
- intravascular obstruction
- cor pulmonale may result from right ventricular overload
* increased mean pulmonary artery pressure with elevated pulmonary artery capillary wedge pressure indicates pulmonary venous hypertension
Genetics
Clinical manifestations
(also see primary pulmonary hypertension)
- exertional dyspnea
- fatigue
- syncope
- peripheral edema
- persistent unexplained hoarseness
- right ventricular heave
- loud pulmonic valve component of the 2nd heart sound P2
- widely split S2
- systolic murmur best heard at the left sternal border that increases with inspiration
- diastolic murmur of tricuspid regurgitation
- right-sided S3 gallop or S4 gallop heart sound
- S3 gallop best heard at the left sternal border with the patient supine
- listen during inspiration, murmur increases with inspiration
- S3 gallop best heard at the left sternal border with the patient supine
- jugular venous distention with a large a wave
- lungs are clear to auscultation
- chest pain
- Raynaud phenomenon suggesting connective tissue disease (see etiology)
Laboratory
- antinuclear antibody (ANA)
- HIV testing
- serum pro-BNP improves predictive value in patients with sickle cell disease[8]
- serum ALT, serum AST
- arterial blood gas (hypoxemia, hypercapnia)
Diagnostic procedures
- echocardiography (initial diagnostic test of choice)[3]
- elevated pulmonary artery pressure
- mean pulmonary artery pressure > 25 mm Hg at rest[3]
- normal range: 15-30/5-13 (mean 10-18) mm Hg
- echocardiography may underestimate true pulmonary artery pressure
- right ventricular hypertrophy, right ventricular dilation, right ventricular failure
- rule out mitral stenosis
- bubble-contrast or transesophageal echocardiogram for congenital heart disease
- elevated pulmonary artery pressure
- electrocardiogram: (EKG)
- right ventricular hypertrophy
- RVH by EKG found in only 1/3 of patients with COPD & cor pulmonale
- RBBB often occurs
- right axis deviation
- T wave changes[9]
- pulmonary function testing
- isolated decrease in DLCO with normal TLC, FEV & FEV1
- SaO2 during sleep (overnight pulse oximetry) & with exertion 6-minute walk may determine need for oxygen therapy[3]
- 6-minute walk improves predictive value in patients with sickle cell disease[8]
- polysomnography
- if suspected sleep disordered breathing[3]
- rule out severe nocturnal hypoxemia
- overnight pulse oximetry may suffice[3]
- pulmonary artery catheterization with vasodilator test
- indications
- suspected pulmonary artery hypertension suggested by clinical presentation not confirmed by echocardiography[3]
- allows measurement of mean pulmonary artery pressure > 20 mm Hg (diagnostic)
- most patients with confirmed pulmonary hypertension[3]
- not indicated in pulmonary hypertension due to heart failure[3]
- patients with connective tissue disease or other disorder associated with pulmonary parenchymal disease[3]
- IV vasodilator (adenosine, epoprostenol)
- inhaled nitric oxide
- vasoreactivity verified by
- reduction in mean pulmonary artery pressure of at least 10 mm of Hg to a value <= 40 mm Hg
- no decrease in cardiac output with vasodilation[3]
- indications
- acute vasoreactivity study
- assess response to vasodilator(s)
- left heart catheterization & coronary angiography to exclude LV dysfunction[3]
- open lung biopsy may be indicated
Radiology
- chest radiograph
- enlarged right ventricle
- dilated pulmonary arteries[9]
- reduced peripheral pulmonary vascularity[3]
- CT angiography if acute pulmonary embolism suspected
- ventilation perfusion scan (V/Q scan) prior to pulmonary angiography in patients with
- suspected pulmonary embolism[3]
- suspected chronic thromboembolic pulmonary hypertension
- pulmonary angiography may be appropriate if VQ scan is equivoval
- findings on chest CT can identify patients with pulmonary hypertension due to left heart failure[24]
Complications
- hypoxia
- cor pulmonale
- even borderline pulmonary hypertension associated with 31% increased risk of all-cause mortality[23]
- complications associated with elective non-cardiac surgery include myocardial ischemia, venous thromboembolism, cardiogenic shock, & dysrhythmias[3]
* mean pulmonary artery pressure of 19-24 mm Hg
Differential diagnosis
- acute onset pulmonary hypertension due to pulmonary embolism
- non-collapsing inferior vena cava
- right ventricular dilation with septal bowing & preserved ejection fraction
Management
- treat underlying cause
- steroids for connective tissue disease, COPD
- treatment of left heart failure with vasodilator may overload left ventricle worsening congestive heart failure[3]
- treatment of lung disease with vasodilator may worsen VQ mismatch[3]
- see[17] for guidelines treating pulmonary hypertension in children
- supplemental oxygen in patients that are hypoxemic
- 6 minute walk & overnight pulse oximetry may qualify patients for oxygen therapy (SaO2 < 88%)
- anticoagulation with warfarin unless contraindicated[3]
- this recommendation in the absence of thromboembolism not well understood
- calcium channel antagonists
- invasive hemodynamic monitoring required prior to initiating therapy to verify vasoreactivity (see primary pulmonary hypertension)
- effective in some patients with primary pulmonary hypertension (pulmonary Raynaud's phenomenon)
- adverse effect of possibly worsening gas exchange
- nifedipine 120-240 mg QD
- diltiazem 540-900 mg QD
- phosphodiesterase-5 inhibitors
- sildenafil, tadalafil[3]
- sildenafil improves exercise capacity, WHO functional class, & hemodynamics[5] in patients with mild pulmonary hypertension
- riociguat in place of PDE-5 inhibitor may be of benefit
- useful for patients with negative vasoreactivity testing
- does not facilitate elective surgery in high-risk patients[3]
- endothelin receptor antagonists
- bosentan
- ambrisentan
- macitentan (Opsumit)
- macitentan/tadalafil (Opsynvi) improves exercise capacity
- useful for patients with negative vasoreactivity testing
- prostacyclin analogues
- treprostinil intravenous
- epoprostenol intravenous
- inhaled iloprost
- reserved for patients whose disease has progressed despite oral therapy with phosphodiesterase-5 inhibitor or endothelin receptor antagonist[3]
- triple therapy of PDE-5 inhibitor, endothelin receptor antagonist & prostacyclin analogue may be better than monotherapy
- other treatment options
- sotatercept (Winrevair) improves exercise capacity FDA-approved March 2024
- industry-sponsored study suggests benefit for initial therapy with ambrisentan (Letairis) 10 mg + tadalafil (Adcirca) 40 mg) PO QD[16]
- nitrates may have short-term benefit
- treatment with vascodilator not curative but improves survival[3]
- diuretics
- consider digoxin[3]
- chronic pulmonary embolism
- anticoagulation if pulmonary embolism suspected
- surgical thromboendarterectomy if indicated in patients with pulmonary embolism
- chronic anticoagulation
- inferior vena cava interruption (Greenfield filter)
- consider exercise training[3]
- delay or cancel elective surgery for patients with high risk pulmonary hypertension[3]
- patients with severe pulmonary hypertension or right ventricular dysfunction should be referred to pulmonary hypertension center[3]
- lung or heart/lung transplantation
- advise against pregnancy if severe; maternal mortality 30-50%
- screen for portal hypertension prior to liver transplantation
- screening:
- 1st degree relatives of patients with familial pulmonary hypertension
- congenital heart disease with systemic-to-pulmonary shunt
More general terms
More specific terms
- chronic thromboembolic pulmonary hypertension (CTEPH)
- portopulmonary hypertension
- primary pulmonary hypertension (PPH)
- pulmonary venous hypertension
Additional terms
- causes of pulmonary hypertension
- pulmonary artery pressure (PAP)
- right ventricular failure; right heart failure
- right ventricular hypertrophy (RVH)
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 256
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 778-81
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 3.17 3.18 3.19 3.20 3.21 3.22 3.23 3.24 3.25 3.26 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
McGlothlin DP, Granton J, Klepetko W, et al. ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery [Editorial]. J Heart Lung Transplant. 2022;41:1135-1194. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36123001 - ↑ Smith R., Jewish Home for the Aging, Reseda CA, 2001, unpublished
- ↑ 5.0 5.1 Galie N et al, Sildenafil citrate therapy for pulmonary arterial hypertension N Engl J Med 353(20):2148, 2005 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16291984
- ↑ Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical Therapy for Pulmonary Arterial Hypertension: Updated ACCP Evidence-Based Clinical Practice Guidelines. Chest. 2007 Jun;131(6):1917-28. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17565025 Corresponding NGC guideline withdrawn Jan 2013 http://www.chestjournal.org/cgi/content/full/131/6/1917
- ↑ McLaughlin VV 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. Circulation. 2009 Mar 30 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/19332472 <Internet> http://content.onlinejacc.org/cgi/content/full/j.jacc.2009.01.004
- ↑ 8.0 8.1 8.2 8.3 Parent F et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 2011 Jul 7; 365:44. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21732836
- ↑ 9.0 9.1 9.2 Geriatrics at your Fingertips, 13th edition, 2011 Reuben DB et al (eds) American Geriatric Society
- ↑ Brown LM, Chen H, Halpern S et al Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest. 2011 Jul;140(1):19-26 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21393391
- ↑ 11.0 11.1 Shah SJ. Pulmonary hypertension. JAMA. 2012;308(13):1366-1374. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23032553
- ↑ 12.0 12.1 Snomed :accession 697899000 Snomed :accession 697900005
- ↑ 13.0 13.1 13.2 Pugh ME et al. Causes of pulmonary hypertension in the elderly. Chest 2014 Jul; 146:159 http://journal.publications.chestnet.org/article.aspx?articleid=1819592
- ↑ ARUP Consult: Pulmonary Arterial Hypertension - PAH The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/pulmonary-arterial-hypertension
ARUP Consult: Pulmonary Arterial Hypertension https://arupconsult.com/ati/pulmonary-arterial-hypertension - ↑ 15.0 15.1 Klings ES, Machado RF, Barst RJ et al American Thoracic Society Ad Hoc Committee on Pulmonary Hypertension of Sickle Cell Disease. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014 Mar 15;189(6):727-40 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24628312
- ↑ 16.0 16.1 Galie N et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015 Aug 27; 373:834 PMID: https://www.ncbi.nlm.nih.gov/pubmed/26308684
- ↑ 17.0 17.1 Abman SH et al Pediatric Pulmonary Hypertension. Guidelines From the American Heart Association and American Thoracic Society. Circulation. Nov 3, 2015 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/26534956 <Internet> http://circ.ahajournals.org/content/early/2015/10/29/CIR.0000000000000329.full.pdf+html
- ↑ Galie N, Hoeper MM, Humbert M et al Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009 Oct;30(20):2493-537. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19713419 Free Article
- ↑ 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. J Am Coll Cardiol. 2009 Apr 28;53(17):1573-619 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19389575 Free full text
- ↑ Galie N, Corris PA, Frost A et al Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D60-72. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24355643 Free Article
- ↑ McGoon MD, Kane GC. Pulmonary hypertension: diagnosis and management. Mayo Clin Proc. 2009 Feb;84(2):191-207. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19181654 Free PMC Article
- ↑ Simonneau G, Gatzoulis MA, Adatia I et al Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D34-41. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24355639 Free Article
- ↑ 23.0 23.1 Assad TR, Maron BA, Robbins IM et al. Prognostic effect and longitudinal hemodynamic assessment of borderline pulmonary hypertension. JAMA Cardiol 2017 Oct 25; PMID: https://www.ncbi.nlm.nih.gov/pubmed/29071338
- ↑ 24.0 24.1 Aviram G, Rozenbaum Z, Ziv-Baran T et al. Identification of pulmonary hypertension caused by left-sided heart disease (World Health Organization group 2) based on cardiac chamber volumes derived from chest CT imaging. Chest 2017 Oct; 152:792. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28506612
- ↑ Thakkar V, Lau EM. Connective tissue disease-related pulmonary arterial hypertension. Best Pract Res Clin Rheumatol. 2016 Feb;30(1):22-38. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27421214
- ↑ Vonk Noordegraaf A, Groeneveldt JA, Bogaard HJ. Pulmonary hypertension. Eur Respir Rev. 2016 Mar;25(139):4-11. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26929415 Free Article
- ↑ Galie 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 Respir J. 2015 Oct;46(4):903-75. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26318161 Free Article
- ↑ Kato M, Atsumi T. Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects. Eur J Clin Invest. 2018 Feb;48(2). Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29285766
- ↑ Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023;61. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36028254