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*Other causes include [[sarcoidosis]], [[histiocytosis X]], genetic diseases linked to known mutations<ref>Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000;67:737-44.</ref>,<ref>Blanpain C, Le Poul E, Parma J, Knoop C, Detheux M, Parmentier M, Vassart G, Abramowicz MJ. Serotonin 5-HT(2B) receptor loss of function mutation in a patient with fenfluramine-associated primary pulmonary hypertension. Cardiovasc Res 2003;60(3):518-28.</ref>,[[fibrosing mediastinitis]], [[pulmonary embolism]], and
*Other causes include [[sarcoidosis]], [[histiocytosis X]], genetic diseases linked to known mutations<ref>Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000;67:737-44.</ref>,<ref>Blanpain C, Le Poul E, Parma J, Knoop C, Detheux M, Parmentier M, Vassart G, Abramowicz MJ. Serotonin 5-HT(2B) receptor loss of function mutation in a patient with fenfluramine-associated primary pulmonary hypertension. Cardiovasc Res 2003;60(3):518-28.</ref>,[[fibrosing mediastinitis]], [[pulmonary embolism]], and
[[Thyroid disease|thyroid disease]].<ref>Curnock AL, Dweik RA, Higgins BH, Saadi HF, Arroliga AC. High prevalence of hypothyroidism in patients with primary pulmonary hypertension. Am J Med Sci 1999;318:289-292</ref>However thyroid disease is only am association and is not regarded as causative.
[[Thyroid disease|thyroid disease]].<ref>Curnock AL, Dweik RA, Higgins BH, Saadi HF, Arroliga AC. High prevalence of hypothyroidism in patients with primary pulmonary hypertension. Am J Med Sci 1999;318:289-292</ref>However thyroid disease is only an association and is not regarded as causative.


*The use of weight loss pills has also led to the development of PAH in the past <ref>Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, Higenbottam T, Oakley C, Wouters E, Aubier M, Simonneau G, Begaud B. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;335:609-16</ref>
*The use of weight loss pills has also led to the development of PAH in the past <ref>Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, Higenbottam T, Oakley C, Wouters E, Aubier M, Simonneau G, Begaud B. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;335:609-16</ref>


*Human herpesvirus 8, also associated with [[Kaposi's sarcoma]], has been demonstrated in patients with PAH, suggesting that this virus may play a role in its development<ref>Cool CD, Rai PR, Yeager ME, Hernandez-Saavedra D, Serls AE, Bull TM, Geraci MW, Brown KK, Routes JM, Tuder RM, Voelkel NF. Expression of Human Herpesvirus 8 in Primary Pulmonary Hypertension.N Engl J Med 2003;349:1113-22.</ref> Recent studies have been unable to find an association between human herpesvirus 8 and idiopathic pulmonary arterial hypertension.
*Human herpesvirus 8, also associated with [[Kaposi's sarcoma]], has been demonstrated in patients with PAH, suggesting that this virus may play a role in its development<ref>Cool CD, Rai PR, Yeager ME, Hernandez-Saavedra D, Serls AE, Bull TM, Geraci MW, Brown KK, Routes JM, Tuder RM, Voelkel NF. Expression of Human Herpesvirus 8 in Primary Pulmonary Hypertension.N Engl J Med 2003;349:1113-22.</ref> However, recent studies have been unable to find an association between human herpesvirus 8 and idiopathic pulmonary arterial hypertension.


*There also seems to be an association of idiopathic PAH (not only PAH caused by heart malformations) and [[Trisomy 21]].
*There also seems to be an association of idiopathic PAH (not only PAH caused by heart malformations) and [[Trisomy 21]].
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{|style="width:70%; height:100px" border="1"
{|style="width:70%; height:100px" border="1"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | [[Atrial Septal Defects]], [[Cor triatum]], [[Left heart Dysfunction or Failure]], [[Teralogy of Fallot]], [[Persistent fetal circulation]], [[Mitral Valve stenosis]],[[Mitral valve incompetence]], [[Ventricular Septal Defect]]   
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | [[Atrial Septal Defects]], [[Cor triatriatum]], [[Left heart failure]], [[Fallot tetralogy]], [[Persistent fetal circulation]], [[Mitral valve stenosis]],[[Mitral valve insufficiency]], [[Ventricular Septal Defect]]   
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Chemical / poisoning'''
| '''Chemical / poisoning'''
|bgcolor="Beige"| [[Coal worker pneumoconiosis]]
|bgcolor="Beige"| [[Coal workers' pneumoconiosis]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Drug amd Toxin Side Effect'''
| '''Drug amd Toxin Side Effect'''
|bgcolor="Beige"|  Definite: [[Aminorex]], [[Fenfluramine]], [[Dexfenfluramine]], [[Toxic rapeseed oil]], [[Benfluorex]], Likely: [[Amphetamines]], [[L-Tryptophan]], [[Methamphetamines]] Possible: [[Cocaine]], [[Phenylpropanolamine]], [[St.Johns wort]], [[Chemotherapeutic agents]], [[SSRI]], [[Pergolide]].
|bgcolor="Beige"|  Definite: [[Aminorex]], [[Fenfluramine]], [[Dexfenfluramine]], Toxic [[Rapeseed]] oil, [[Benfluorex]], Likely: [[Amphetamines]], L-[[Tryptophan]], [[Methamphetamine]] Possible: [[Cocaine]], [[Phenylpropanolamine]], St.Johns wort, [[Chemotherapeutic agents]], [[SSRI]], [[Pergolide]].


|-
|-
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Endocrine'''
| '''Endocrine'''
|bgcolor="Beige"| [[Thyroid disorders]]
|bgcolor="Beige"| [[Thyroid diseases]]
|-  
|-  
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Environmental'''
| '''Environmental'''
|bgcolor="Beige"| [[Altitude sickness,chronic]]
|bgcolor="Beige"| [[ High altitude sickness|High Altitude(chronically)]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Genetic'''
| '''Genetic'''
|bgcolor="Beige"| [[Alveolar capillary dysplasia with misalignment of pulmonary veins]], [[Cholesterol ester storage disease]], [[Cystic fibrosis]], [[Gaucher disease]], [[Indian familial childhood cirrhosis]]
|bgcolor="Beige"| Alveolar capillary dysplasia with misalignment of pulmonary veins, [[Cholesterol ester storage disease]], [[Cystic fibrosis]], [[Gaucher disease]], Indian familial childhood cirrhosis
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Hematologic'''
| '''Hematologic'''
|bgcolor="Beige"| [[Myeloproliferative discorders]], [[Paroxysmal nocturnal haemoglobinuria]], [[Polycythemia Vera]], [[Splenectomy(Thrombophilia)]],[[Sickle cell disease]].
|bgcolor="Beige"| [[Myeloproliferative disorders]], [[Paroxysmal nocturnal haemoglobinuria]], [[Polycythemia Vera]], [[Splenectomy]](due to thrombophilia),[[Sickle cell disease]].
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Infectious Disease'''
| '''Infectious Disease'''
|bgcolor="Beige"| [[Shistosoma japonicum and mansoni]],[[HHV8 also associated with Kaposi's sarcoma]]
|bgcolor="Beige"| [[Schistosoma japonicum]] and [[Schistosoma mansoni]], HHV8 also associated with [[Kaposi's sarcoma]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Musculoskeletal / Ortho'''
| '''Musculoskeletal / Ortho'''
|bgcolor="Beige"| [[Idiopathic spinal scoliosis]]
|bgcolor="Beige"| [[Scoliosis|Idiopathic spinal scoliosis]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Overdose / Toxicity'''
| '''Overdose / Toxicity'''
|bgcolor="Beige"| [[Diethypropion]], [[Monocrotaline]], [[Phentermine]], [[Radiation exposure(Fibrosing mediastinitis and pulmonary fibrosis)]]
|bgcolor="Beige"| [[Diethylpropion]], Monocrotaline, [[Phentermine]], Radiation exposure [[Fibrosing mediastinitis]] and [[pulmonary fibrosis]]).
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Pulmonary'''
| '''Pulmonary'''
|bgcolor="Beige"| [[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]], [[Langerhan cell Histiocytosis]],[[Interstitial Lung Disease]].
|bgcolor="Beige"| [[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]].
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Renal / Electrolyte'''
| '''Renal / Electrolyte'''
|bgcolor="Beige"| [[Chronic renal failure on Dialysis]]
|bgcolor="Beige"| Chronic [[renal failure]] on [[dialysis]]
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Rheum / Immune / Allergy'''
| '''Rheum / Immune / Allergy'''
|bgcolor="Beige"|  [[Vasculitis; Churg-Strauss syndrome]], [[Sarcoidosis]], [[Systemic sclerosis]], [[Systemic lupus erythematosus]].
|bgcolor="Beige"|  [[Vasculitis]], [[Churg-Strauss syndrome]], [[Sarcoidosis]], [[Systemic sclerosis]], [[Systemic lupus erythematosus]].
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Miscellaneous'''
| '''Miscellaneous'''
|bgcolor="Beige"| [[Gaucher disease]], [[Glycogen storage diseases]], [[Idiopathic spinal scoliosis]],   
|bgcolor="Beige"| [[Gaucher disease]], [[Glycogen storage diseases]], [[Scoliosis|Idiopathic spinal scoliosis]],   
|-
|-
|}
|}
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(In Alphabetical Order)
(In Alphabetical Order)
* [[Altitude sickness, chronic]]
* [[ High altitude sickness|High Altitude(chronically)]]
* [[Alveolar capillary dysplasia with misalignment of pulmonary veins]]
* Alveolar capillary dysplasia with misalignment of pulmonary veins
* [[Atrial Septal Defects]]
* [[Atrial Septal Defects]]
* [[Bronchiectasis]]
* [[Bronchiectasis]]
* [[Bronchopulmonary Dysplasia]]
* [[Bronchopulmonary dysplasia]]
* [[Cholesterol ester storage disease]]
* [[Cholesterol ester storage disease]]
* [[Chronic hemolytic anemia]]
* Chronic [[hemolytic anemia]]
* [[Chronic obstructive pulmonary disease]]
* [[Chronic obstructive pulmonary disease]]
* [[Chronic renal failure on dialysis]]
* Chronic [[renal failure]] on [[dialysis]]
* [[Churg-Strauss syndrome]]
* [[Churg-Strauss syndrome]]
* [[Coal worker pneumoconiosis]]
* [[Coal workers' pneumoconiosis]]
* [[Cor triatriatum]]
* [[Cor triatriatum]]
* [[Cystic fibrosis]]
* [[Cystic fibrosis]]
* [[Diastolic left heart dysfunction]]
* [[ Diastolic heart failure|Diastolic dysfunction]].
* [[Diethylpropion]]
* [[Diethylpropion]]
* [[Fallot tetralogy]]
* [[Fallot tetralogy]]
* [[Fetal circulation, persistent]]
* [[Fetal circulation, persistent]]
* [[Fibrosing mediastinitis]]
* [[Fibrosing Mediastinitis|Fibrosing mediastinitis]]
* [[Gaucher disease]]
* [[Gaucher disease]]
* [[Glycogen storage diseases]]
* [[Glycogen storage diseases]]
* [[Idiopathic pulmonary haemosiderosis]]
* [[Idiopathic pulmonary haemosiderosis]]
* [[Idiopathic spinal scoliosis]]
* [[Scoliosis|Idiopathic spinal scoliosis]]
* [[Indian familial childhood cirrhosis]]
* Indian familial childhood cirrhosis
* [[Interstitial Lung Disease]]
* [[Interstitial Lung Disease]]
* [[Langerhan cell Histiocytosis]]
* [[Langerhans cell histiocytosis]]
* [[Mitral valve incompetence]]
* [[Mitral valve insufficiency]]
* [[Mitral valve stenosis]]
* [[Mitral valve stenosis]]
* [[Monocrotaline poisoning]]
* Monocrotaline poisoning
* [[Myeloproliferative disorders]]
* [[Myeloproliferative disorders]]
* [[Neurofibromatosis]]
* [[Neurofibromatosis]]
* [[Obstructive sleep apnoea]]
* [[Obstructive sleep apnea]]
* [[Paroxysmal nocturnal haemoglobinuria]]
* [[Paroxysmal nocturnal haemoglobinuria]]
* [[Phentermine poisoning]]
* [[Phentermine]] poisoning
* [[Pickwickian syndrome]]
* [[Pickwickian syndrome]]
* [[Portal hypertension]]
* [[Portal hypertension]]
* [[Polycythemia vera]]
* [[Polycythemia vera]]
* [[Primary pulmonary hypertension]]
* Pulmonary alveolar microlithiasis
* [[Pulmonary alveolar microlithiasis]]
* Pulmonary capillary hemangiomatosis
* [[Pulmonary capillary hemangiomatosis]]
* [[Pulmonary embolism]]
* [[Pulmonary embolism]]
* [[Pulmonary fibrosis]]
* [[Pulmonary fibrosis]]
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* [[Sickle cell disease]]
* [[Sickle cell disease]]
* [[Splenectomy]]
* [[Splenectomy]]
* [[Systemic Lupus Erythematosus]]
* [[Systemic lupus erythematosus]]
* [[Systolic Left heart dysfunction]]
* [[Systolic dysfunction]]
* [[Tropical pulmonary eosinophilia]]
* [[Tropical pulmonary eosinophilia]]
* [[Vasculitis]]
* [[Vasculitis]]
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#Heritable( BMPR2,ALK-1,Endogin...)
#Heritable( BMPR2,ALK-1,Endogin...)
#Drug and toxin induced.
#Drug and toxin induced.
#Connective tissue diseases
#[[Connective tissue diseases]]
#HIV
#[[HIV]]
#Portal Hypertension
#[[Portal Hypertension]]
#[[Congenital heart disease|Congenital heart diseases]]
#[[Congenital heart disease|Congenital heart diseases]]
#Schistosomiasis
#[[Schistosomiasis]]
#Chronic hemolytic anemia.
#Chronic [[hemolytic anemia]].
#Persistent Pulmonary Hypertension of the newborn
#Persistent Pulmonary Hypertension of the newborn


Class 2: Pulmonary Venous Hypertension or pulmonary hypertension owing to left heart disease
Class 2: Pulmonary Venous Hypertension or pulmonary hypertension owing to left heart disease
#Systolic Dysfunction.
#[[Systolic dysfunction]].
#Diastolic dysfunction.
#[[ Diastolic heart failure|Diastolic dysfunction]].
#Valvular disease.
#[[Valvular disease]].


Class 2: Pulmonary Hypertension associated with disorders of the respiratory system and/or Hypoxemia
Class 2: Pulmonary Hypertension associated with disorders of the respiratory system and/or Hypoxemia
#Chronic Obstructive Pulmonary Disease.
#[[Chronic Obstructive Pulmonary Disease]].
#Interstitial Lung Disease
#[[Interstitial Lung Disease]]
#Pulmonary diseases with mixed restrictive and obstructive patterns.
#Pulmonary diseases with mixed [[Restrictive lung disease|restrictive]] and [[Obstructive Lung Disease|obstructive]] patterns.
#Obstructive sleep apnea.
#[[Obstructive sleep apnea]].
#High Altitude(chronically).
#[[ High altitude sickness|High Altitude(chronically)]].
#Developmental abnormalities.
#Developmental abnormalities.


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Class 5: Pulmonary Hypertension due to disorders directly affecting the pulmonary vasculature
Class 5: Pulmonary Hypertension due to disorders directly affecting the pulmonary vasculature
#Hematologic disorders: Myeloproliferative disorders,splenectomy,polycythemia vera.
#'''Hematologic disorders''': [[Myeloproliferative disorders]],[[splenectomy]],[[polycythemia vera]].
#Systemic disorders: Sarcoidosis,Langerhan cell Histiocytosis, Neurofibromatosis, Vasculitis.
#'''Systemic disorders''': [[Sarcoidosis]],[[Langerhans cell histiocytosis]], [[Neurofibromatosis]], [[Vasculitis]].
#Metabolic disorders: Glycogen storage diseases, Gaucher disease, Thyroid disorders.
#'''Metabolic disorders''': [[Glycogen storage diseases]], [[Gaucher disease]], [[Thyroid disease|thyroid disease]].
#Miscellaneous: Tumor obstruction, Fibrosing mediastinitis, chronic renal failure on dialysis.
#'''Miscellaneous''': Tumor obstruction, [[Fibrosing Mediastinitis|fibrosing mediastinitis]], chronic [[renal failure]] on [[dialysis]].

Revision as of 15:49, 9 September 2011

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

Overview

  • The most common cause of pulmonary hypertension is left heart failure leading to pulmonary venous hypertension.

thyroid disease.[4]However thyroid disease is only an association and is not regarded as causative.

  • The use of weight loss pills has also led to the development of PAH in the past [5]
  • Human herpesvirus 8, also associated with Kaposi's sarcoma, has been demonstrated in patients with PAH, suggesting that this virus may play a role in its development[6] However, recent studies have been unable to find an association between human herpesvirus 8 and idiopathic pulmonary arterial hypertension.
  • There also seems to be an association of idiopathic PAH (not only PAH caused by heart malformations) and Trisomy 21.
  • When none of these causes can be found, the disease is termed idiopathic pulmonary arterial hypertension (IPAH).


Complete Differential Diagnosis of the Causes of Pulmonary Hypertension

(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, HHV8 also associated with Kaposi's sarcoma
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,

Complete Differential Diagnosis of the Causes of Pulmonary Hypertension

(In Alphabetical Order)


Complete Differential Diagnosis of the Causes of Pulmonary Hypertension

(By Classes, Dana Point 2008)

Class 1: Pulmonary Aterial Hypertension

  1. Idiopathic PAH
  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 2: 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.
  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
  2. ↑ Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000;67:737-44.
  3. ↑ Blanpain C, Le Poul E, Parma J, Knoop C, Detheux M, Parmentier M, Vassart G, Abramowicz MJ. Serotonin 5-HT(2B) receptor loss of function mutation in a patient with fenfluramine-associated primary pulmonary hypertension. Cardiovasc Res 2003;60(3):518-28.
  4. ↑ Curnock AL, Dweik RA, Higgins BH, Saadi HF, Arroliga AC. High prevalence of hypothyroidism in patients with primary pulmonary hypertension. Am J Med Sci 1999;318:289-292
  5. ↑ Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, Higenbottam T, Oakley C, Wouters E, Aubier M, Simonneau G, Begaud B. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996;335:609-16
  6. ↑ Cool CD, Rai PR, Yeager ME, Hernandez-Saavedra D, Serls AE, Bull TM, Geraci MW, Brown KK, Routes JM, Tuder RM, Voelkel NF. Expression of Human Herpesvirus 8 in Primary Pulmonary Hypertension.N Engl J Med 2003;349:1113-22.
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