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==Overview==
==Overview==
==Causes==
==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 in itself is not a life threatening condition, but it is progressive fatal if left untreated.
===Common Causes===
===Common Causes===
The most common cause of pulmonary hypertension is [[left heart failure]] leading to pulmonary venous hypertension. Common causes of pulmonary arterial hypertension (PAH) include [[HIV]], [[systemic sclerosis]], [[portal hypertension]], [[sickle cell disease]],<ref>Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95</ref> and [[congenital heart disease]]. Lung diseases that lower oxygen in the blood (hypoxia) are well known causes of pulmonary hypertension, including [[COPD]], [[interstitial lung disease]], [[Pickwickian syndrome]] or obesity-hypoventilation syndrome, and [[obstructive sleep apnea]].
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:


*[[HIV]]
*[[Systemic sclerosis]]
*[[Portal hypertension]]
*[[Sickle cell disease]]<ref>Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95</ref>
*[[Congenital heart disease]]
*[[COPD]]
*[[Interstitial lung disease]]
*[[Pickwickian syndrome]]
*[[Obstructive sleep apnea]]
*[[obesity-hypoventilation syndrome]]
* [[Idiopathic Pulmonary Hypertension]] (Diagnosis of exclusion).
* [[Idiopathic Pulmonary Hypertension]] (Diagnosis of exclusion).
* Familial Pulmonary Hypertension.
* [[Familial Pulmonary Hypertension]]
* [[Cor Pulmonale]]( Right heart failure due to pulmonary disease).
* [[Cor Pulmonale]]( Right heart failure due to pulmonary disease).
* [[Congestive heart failure]].
* [[Congestive heart failure]].
* Right sided valvular disease.
* [[Right sided valvular disease]]
* [[Mitral stenosis]].
* [[Mitral stenosis]]


====Idiopathic Pulmonary Arterial Hypertension====
====Idiopathic Pulmonary Arterial Hypertension====

Revision as of 20:55, 8 November 2013

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ralph Matar

Overview

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 in itself is not a life threatening condition, but it is progressive fatal if left untreated.

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:

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 Defects, 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 amd Toxin Side Effect Definite: Aminorex, Fenfluramine, Dexfenfluramine, Toxic Rapeseed oil, Benfluorex,
Likely: Amphetamines, L-Tryptophan, Methamphetamine
Possible: Cocaine, Phenylpropanolamine, St.Johns wort, Chemotherapeutic agents, SSRI, Pergolide.
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(due to thrombophilia), Sickle cell disease.
Iatrogenic No underlying cause
Infectious Disease Schistosoma japonicum and Schistosoma mansoni, HHV-8.
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 haemosiderosis, 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 Aterial Hypertension

  1. Idiopathic pulmonary arterial hypertension.
  2. Heritable( BMPR2,ALK-1,Endogin...)
  3. Drug and toxin induced.
  4. Connective tissue diseases
  5. HIV
  6. Portal Hypertension
  7. Congenital heart diseases
  8. Schistosomiasis
  9. Chronic hemolytic anemia.
  10. Persistent Pulmonary Hypertension of the newborn

Class 2: Pulmonary Venous Hypertension or pulmonary hypertension owing to left heart disease

  1. Systolic dysfunction.
  2. Diastolic dysfunction.
  3. Valvular disease.

Class 3: Pulmonary Hypertension associated with disorders of the respiratory system and/or Hypoxemia

  1. Chronic Obstructive Pulmonary Disease.
  2. Interstitial Lung Disease
  3. Pulmonary diseases with mixed restrictive and obstructive patterns.
  4. Obstructive sleep apnea.
  5. High Altitude(chronically).
  6. Developmental abnormalities.

Class 4: Pulmonary Hypertension due to chronic thrombotic and/or embolic disease

Class 5: Pulmonary Hypertension due to disorders directly affecting the pulmonary vasculature

  1. Hematologic disorders: Myeloproliferative disorders, splenectomy, polycythemia vera.
  2. Systemic disorders: Sarcoidosis, Langerhans cell histiocytosis, Neurofibromatosis, Vasculitis.
  3. Metabolic disorders: Glycogen storage diseases, Gaucher disease, thyroid disease.
  4. Miscellaneous: Tumor obstruction, fibrosing mediastinitis, chronic renal failure on dialysis.

References

  1. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95

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