Pulmonary hypertension causes

	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]

Overview

Pulmonary hypertension may be caused by either left heart failure (the most common cause); HIV, systemic sclerosis, portal hypertension, congenital heart disease and sickle cell disease.

Causes

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

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

Class 2: Pulmonary hypertension due to left heart disease

Left ventricular systolic dysfunction
Left ventricular diastolic dysfunction
Valvular disease
Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies

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

Chronic obstructive pulmonary disease
Interstitial lung disease
Other pulmonary diseases with mixed restrictive and obstructive pattern
Sleep-disordered breathing
Alveolar hypoventilation disorders
Chronic exposure to high altitude
Developmental lung diseases

Class 4: Chronic thromboembolic pulmonary hypertension

Class 5: Pulmonary hypertension with unclear multifactorial mechanisms

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

References

↑ 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.
↑ 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.CS1 maint: PMC format (link)
↑ 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.
↑ Kashyap S, Mohapatra PR (2013). "Pulmonary alveolar microlithiasis". Lung India. 30 (2): 143–7. doi:10.4103/0970-2113.110424. PMC 3669555. PMID 23741096.CS1 maint: PMC format (link)

Template:WikiDoc Sources

[pmid14985486-1] 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.

[pmid23852284-2] 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.CS1 maint: PMC format (link)

[pmid9776954-3] 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.

[pmid23741096-4] Kashyap S, Mohapatra PR (2013). "Pulmonary alveolar microlithiasis". Lung India. 30 (2): 143–7. doi:10.4103/0970-2113.110424. PMC 3669555. PMID 23741096.CS1 maint: PMC format (link)

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