Pulmonary hypertension: Difference between revisions
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#* Idiopathic or hereditary |
#* Idiopathic or hereditary |
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#* Drugs and toxins |
#* Drugs and toxins |
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#** Definitely appetite suppressants (e.g. aminorex, fenfluramine, dexfenfluramine, and diethylpropion), toxic rapeseed oil, and benfluorex |
#** Definitely appetite suppressants (e.g. [[aminorex]], [[fenfluramine]], [[dexfenfluramine]], and [[diethylpropion]]), toxic rapeseed oil, and [[benfluorex]] |
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#** Possibly amphetamines, L-tryptophan, methamphetamines, cocaine, phenylpropanolamine, St. John's |
#** Possibly amphetamines, L-tryptophan, methamphetamines, cocaine, phenylpropanolamine, [[St. John's wort]], [[dasatinib]], and [[interferon]] |
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#* Connective tissue disease |
#* Connective tissue disease |
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#** Systemic sclerosis |
#** [[Systemic sclerosis]] |
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#** Rheumatoid arthritis and systemic lupus erythematosus |
#** [[Rheumatoid arthritis]] and [[systemic lupus erythematosus]] |
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#* Infections |
#* Infections |
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#** HIV |
#** [[HIV]] |
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#** Schistosomiasis (most common cause worldwide) |
#** [[Schistosomiasis]] (most common cause worldwide) |
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#* Portal hypertension (portopulmonary hypertension) |
#* [[Portal hypertension]] (portopulmonary hypertension) |
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#* Congenital heart disease, including Eisenmenger syndrome |
#* [[Congenital heart disease]], including [[Eisenmenger syndrome]] |
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# Left heart disease, with an elevated left atrial (mean pressure >14 mmHg) and pulmonary venous pressure |
# Left heart disease, with an elevated left atrial (mean pressure >14 mmHg) and pulmonary venous pressure |
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#* Left ventricular systolic or diastolic dysfunction |
#* Left ventricular systolic or diastolic dysfunction |
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#* Mitral and aortic valve disease |
#* Mitral and aortic valve disease |
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#* Also, restrictive cardiomyopathy, constrictive pericarditis, left atrial myxoma, congenital or acquired in- or outflow tract obstruction and congenital cardiomyopathies |
#* Also, [[restrictive cardiomyopathy]], [[constrictive pericarditis,]] [[left atrial myxoma]], congenital or acquired in- or outflow tract obstruction and congenital cardiomyopathies |
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# Chronic lung disease or hypoxia |
# Chronic lung disease or hypoxia |
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#* Chronic obstructive pulmonary disease (COPD) |
#* [[Chronic obstructive pulmonary disease]] (COPD) |
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#* Interstitial lung disease (ILD) |
#* [[Interstitial lung disease]] (ILD) |
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#* Combined pulmonary fibrosis and emphysema (CPFE) |
#* [[Combined pulmonary fibrosis and emphysema]] (CPFE) |
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#* Obstructive sleep |
#* [[Obstructive sleep apnea]] (OSA) |
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# Chronic thromboembolic pulmonary hypertension (CTEPH) |
# [[Chronic thromboembolic pulmonary hypertension]] (CTEPH) |
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# Miscellaneous |
# Miscellaneous |
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#* Chronic hemolytic anemia (e.g. sickle cell disease [SCD], beta-thalassemia, or spherocytosis) |
#* Chronic hemolytic anemia (e.g. sickle cell disease [SCD], beta-thalassemia, or spherocytosis) |
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#* Myeloproliferative |
#* [[Myeloproliferative disorder]] |
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#* Systemic disorders (e.g. sarcoidosis) |
#* Systemic disorders (e.g. [[sarcoidosis]]) |
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#* Metabolic disorders (e.g. glycogen storage disease) |
#* Metabolic disorders (e.g. [[glycogen storage disease]]) |
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#* Chronic kidney disease |
#* [[Chronic kidney disease]] |
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== Clinical Manifestations == |
== Clinical Manifestations == |
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Latest revision as of 13:27, 18 November 2025
Definition
- Elevation of the mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest
- Normal pressures are 15-30 mmHg in systole and 4-12 mmHg in diastole, resulting in normal mPAP <20 mmHg
Etiology
- Pulmonary artery hypertension (PAH)
- Idiopathic or hereditary
- Drugs and toxins
- Definitely appetite suppressants (e.g. aminorex, fenfluramine, dexfenfluramine, and diethylpropion), toxic rapeseed oil, and benfluorex
- Possibly amphetamines, L-tryptophan, methamphetamines, cocaine, phenylpropanolamine, St. John's wort, dasatinib, and interferon
- Connective tissue disease
- Infections
- HIV
- Schistosomiasis (most common cause worldwide)
- Portal hypertension (portopulmonary hypertension)
- Congenital heart disease, including Eisenmenger syndrome
- Left heart disease, with an elevated left atrial (mean pressure >14 mmHg) and pulmonary venous pressure
- Left ventricular systolic or diastolic dysfunction
- Mitral and aortic valve disease
- Also, restrictive cardiomyopathy, constrictive pericarditis, left atrial myxoma, congenital or acquired in- or outflow tract obstruction and congenital cardiomyopathies
- Chronic lung disease or hypoxia
- Chronic thromboembolic pulmonary hypertension (CTEPH)
- Miscellaneous
- Chronic hemolytic anemia (e.g. sickle cell disease [SCD], beta-thalassemia, or spherocytosis)
- Myeloproliferative disorder
- Systemic disorders (e.g. sarcoidosis)
- Metabolic disorders (e.g. glycogen storage disease)
- Chronic kidney disease
Clinical Manifestations
Physical Examination
- Palpable P2 in MS has a LR+ 3.6 and LR– 0.05
- Loud P2 in cirrhosis has a LR+ 17.6 but only 38% sensitive
- Graham Steell murmur (high-pressure pulmonary regurgitation murmur) has LR+ 4.2
- Diastolic murmur best hear at the LUSB
- Similar to asortic regurgitation murmurs
- Can have fixed or wide splitting of the S
2hiv renal
Investigations
- Imaging of the chest to assess parenchymal lung disease
- V/Q scanning to assess potential chronic thromboembolic disease
- Pulmonary function testing with DLCO
- Serologic studies for connective tissue disease, liver disease, and HIV
- Sleep study to rule out OSA
