Pulmonary hypertension - Revision history

Aidan: links

2025-11-18T13:27:50Z

links

← Older revision		Revision as of 13:27, 18 November 2025
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	#* Idiopathic or hereditary		#* Idiopathic or hereditary
	#* Drugs and toxins		#* Drugs and toxins
	#** 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]]
	#** Possibly amphetamines, L-tryptophan, methamphetamines, cocaine, phenylpropanolamine, St. John's ~~Wort~~, dasatinib, and interferon		#** Possibly amphetamines, L-tryptophan, methamphetamines, cocaine, phenylpropanolamine, [[St. John's wort]], [[dasatinib]], and [[interferon]]
	#* Connective tissue disease		#* Connective tissue disease
	#** Systemic sclerosis		#** [[Systemic sclerosis]]
	#** Rheumatoid arthritis and systemic lupus erythematosus		#** [[Rheumatoid arthritis]] and [[systemic lupus erythematosus]]
	#* Infections		#* Infections
	#** HIV		#** [[HIV]]
	#** Schistosomiasis (most common cause worldwide)		#** [[Schistosomiasis]] (most common cause worldwide)
	#* Portal hypertension (portopulmonary hypertension)		#* [[Portal hypertension]] (portopulmonary hypertension)
	#* Congenital heart disease, including Eisenmenger syndrome		#* [[Congenital heart disease]], including [[Eisenmenger syndrome]]
	# 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
	#* Left ventricular systolic or diastolic dysfunction		#* Left ventricular systolic or diastolic dysfunction
	#* Mitral and aortic valve disease		#* Mitral and aortic valve disease
	#* 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
	# Chronic lung disease or hypoxia		# Chronic lung disease or hypoxia
	#* Chronic obstructive pulmonary disease (COPD)		#* [[Chronic obstructive pulmonary disease]] (COPD)
	#* Interstitial lung disease (ILD)		#* [[Interstitial lung disease]] (ILD)
	#* Combined pulmonary fibrosis and emphysema (CPFE)		#* [[Combined pulmonary fibrosis and emphysema]] (CPFE)
	#* Obstructive sleep ~~apnoea~~ (OSA)		#* [[Obstructive sleep apnea]] (OSA)
	# Chronic thromboembolic pulmonary hypertension (CTEPH)		# [[Chronic thromboembolic pulmonary hypertension]] (CTEPH)
	# Miscellaneous		# Miscellaneous
	#* 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)
	#* Myeloproliferative ~~disorders~~		#* [[Myeloproliferative disorder]]
	#* Systemic disorders (e.g. sarcoidosis)		#* Systemic disorders (e.g. [[sarcoidosis]])
	#* Metabolic disorders (e.g. glycogen storage disease)		#* Metabolic disorders (e.g. [[glycogen storage disease]])
	#* Chronic kidney disease		#* [[Chronic kidney disease]]

	== Clinical Manifestations ==		== Clinical Manifestations ==

Aidan: Text replacement - "Clinical Presentation" to "Clinical Manifestations"

2020-07-15T00:15:27Z

Text replacement - "Clinical Presentation" to "Clinical Manifestations"

← Older revision		Revision as of 00:15, 15 July 2020
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	#* Chronic kidney disease		#* Chronic kidney disease

	== Clinical ~~Presentation~~ ==		== Clinical Manifestations ==

	=== Physical Examination ===		=== Physical Examination ===

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2020-07-04T01:17:52Z

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== 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
#** Systemic sclerosis
#** Rheumatoid arthritis and systemic lupus erythematosus
#* 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 obstructive pulmonary disease (COPD)
#* Interstitial lung disease (ILD)
#* Combined pulmonary fibrosis and emphysema (CPFE)
#* Obstructive sleep apnoea (OSA)
# Chronic thromboembolic pulmonary hypertension (CTEPH)
# Miscellaneous
#* Chronic hemolytic anemia (e.g. sickle cell disease [SCD], beta-thalassemia, or spherocytosis)
#* Myeloproliferative disorders
#* Systemic disorders (e.g. sarcoidosis)
#* Metabolic disorders (e.g. glycogen storage disease)
#* Chronic kidney disease

== Clinical Presentation ==

=== 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<s>2</s>hiv 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

[[Category:Respirology]]
[[Category:Cardiology]]