Pulmonary hypertension

From IDWiki


  • 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


  1. 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
  2. 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
  3. Chronic lung disease or hypoxia
    • Chronic obstructive pulmonary disease (COPD)
    • Interstitial lung disease (ILD)
    • Combined pulmonary fibrosis and emphysema (CPFE)
    • Obstructive sleep apnoea (OSA)
  4. Chronic thromboembolic pulmonary hypertension (CTEPH)
  5. 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 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 S2hiv renal


  • 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