Pulmonary hypertension causes

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Pulmonary Hypertension Microchapters


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ralph Matar; Mohamed Moubarak, M.D. [2]; José Eduardo Riceto Loyola Junior, M.D.[3]


Pulmonary hypertension may be caused by either left heart failure (the most common cause) or other somewhat common causes such as HIV, systemic sclerosis, portal hypertension, congenital heart disease, and sickle cell disease. The World Health Organization (WHO) has classified PH based on etiology into five distinct groups: Group 1 (pulmonary arterial hypertension), Group 2 (PH due to left heart failure), Group 3 (PH due to chronic lung disease and/or hypoxemia), Group 4 (PH due to chronic thromboembolic disease), and Group 5 (PH due to multifactorial mechanisms).


Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

  • Pulmonary hypertension itself is not a life-threatening condition, but it is progressively fatal if left untreated. Pulmonary embolism and acute left heart failure are two causes of pulmonary hypertension that can be quickly fatal.

Common Causes

The most common cause of pulmonary hypertension is left heart failure leading to pulmonary venous hypertension. Other common causes of pulmonary arterial hypertension (PAH) include:[1][2][3][4]

Idiopathic Pulmonary Arterial Hypertension

When none of the causes on this page can be found, the disease is termed idiopathic pulmonary arterial hypertension (IPAH).

Causes by Organ System

Cardiovascular Atrial septal defect, cor triatriatum, left heart failure, Fallot tetralogy, persistent fetal circulation, mitral valve stenosis, mitral valve insufficiency, ventricular septal defect
Chemical / poisoning Coal workers' pneumoconiosis
Dermatologic Neurofibromatosis, systemic lupus erythematosus
Drug and Toxin Side Effect Definite: Aminorex, Carfilzomib, benfluorex, dexfenfluramine, Diethylpropion, fenfluramine, toxic rapeseed oil, benfluorex,
Likely: Amphetamines, L-tryptophan, methamphetamine
Possible: Cocaine, Lorcaserin, Pegylated interferon alfa-2b, phentermine, phenylpropanolamine, pergolide,Protamine sulfate,St John's wort, chemotherapeutic agents, SSRI, Aprotinin
Ear Nose Throat Pickwickian syndrome
Endocrine Thyroid diseases
Environmental High Altitude (chronically)
Gastroenterologic Portal hypertension
Genetic Alveolar capillary dysplasia with misalignment of pulmonary veins, cholesterol ester storage disease, cystic fibrosis, Gaucher disease, Indian familial childhood cirrhosis
Hematologic Myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria, polycythemia vera, splenectomy, sickle cell disease
Iatrogenic No underlying cause
Infectious Disease HHV-8, schistosoma japonicum and schistosoma mansoni
Musculoskeletal / Ortho Idiopathic spinal scoliosis
Neurologic No underlying cause
Nutritional / Metabolic No underlying cause
Obstetric/Gynecologic No underlying cause
Oncologic No underlying causes
Opthalmologic No underlying causes
Overdose / Toxicity Diethylpropion, monocrotaline, phentermine, radiation exposure (fibrosing mediastinitis and pulmonary fibrosis).
Psychiatric No underlying causes
Pulmonary Idiopathic pulmonary hemosiderosis, sarcoidosis, primary pulmonary hypertension, pulmonary fibrosis, alveolar capillary dysplasia with misalignment of pulmonary veins, cystic fibrosis, pulmonary embolism, tropical pulmonary eosinophilia, pulmonary capillary hemangiomatosis, bronchiectasis,bronchopulmonary dysplasia, chronic obstructive pulmonary disease, pulmonary alveolar microlithiasis, pulmonary fibrosis, Langerhans cell histiocytosis,interstitial lung disease.
Renal / Electrolyte Chronic renal failure on dialysis
Rheum / Immune / Allergy Vasculitis, Churg-Strauss syndrome, sarcoidosis, systemic sclerosis, systemic lupus erythematosus.
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous Gaucher disease, glycogen storage diseases, idiopathic spinal scoliosis,

Causes in Alphabetical Order

Causes by Clinical Classification

Class 1: Pulmonary arterial hypertension

  1. Idiopathic pulmonary arterial hypertension
  2. Heritable (BMPR2, ALK-1, ENG, SMAD9, CAV1, KCNK3)
  3. Drug and toxin induced

Class 2: Pulmonary hypertension due to left heart disease

  1. Left ventricular systolic dysfunction
  2. Left ventricular diastolic dysfunction
  3. Valvular disease
  4. Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies

Class 3: Pulmonary hypertension due to lung diseases and/or hypoxia

  1. Chronic obstructive pulmonary disease
  2. Interstitial lung disease
  3. Other pulmonary diseases with mixed restrictive and obstructive pattern
  4. Sleep-disordered breathing
  5. Alveolar hypoventilation disorders
  6. Chronic exposure to high altitude
  7. Developmental lung diseases

Class 4: Chronic thromboembolic pulmonary hypertension

Class 5: Pulmonary hypertension with unclear multifactorial mechanisms

  1. Hematologic disorders: Chronic hemolytic anemia, Myeloproliferative disorders, splenectomy,
  2. Systemic disorders: Sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
  3. Metabolic disorders: Glycogen storage disease, Gaucher disease, thyroid disorders
  4. Miscellaneous: Tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH


  1. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K; et al. (2004). "Pulmonary hypertension as a risk factor for death in patients with sickle cell disease". N Engl J Med. 350 (9): 886–95. doi:10.1056/NEJMoa035477. PMID 14985486.
  2. Nayak NC, Chitale AR (2013). "Indian childhood cirrhosis (ICC) & ICC-like diseases: the changing scenario of facts versus notions". Indian J Med Res. 137 (6): 1029–42. PMC 3734708. PMID 23852284.
  3. Schultze AE, Roth RA (1998). "Chronic pulmonary hypertension--the monocrotaline model and involvement of the hemostatic system". J Toxicol Environ Health B Crit Rev. 1 (4): 271–346. doi:10.1080/10937409809524557. PMID 9776954.
  4. Kashyap S, Mohapatra PR (2013). "Pulmonary alveolar microlithiasis". Lung India. 30 (2): 143–7. doi:10.4103/0970-2113.110424. PMC 3669555. PMID 23741096.

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