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*The most common cause of pulmonary hypertension is [[left heart failure]] leading to pulmonary venous hypertension.
*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]]
*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 possibly [[sleep apnea]].
*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]].


*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 an 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> However, 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 HHV-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 triatriatum]], [[Left heart failure]], [[Fallot tetralogy]], [[Persistent fetal circulation]], [[Mitral valve stenosis]],[[Mitral valve insufficiency]], [[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"
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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]], [[Methamphetamine]] Possible: [[Cocaine]], [[Phenylpropanolamine]], St.Johns wort, [[Chemotherapeutic agents]], [[SSRI]], [[Pergolide]].
|bgcolor="Beige"|  Definite: [[Aminorex]], [[Fenfluramine]], [[Dexfenfluramine]], Toxic [[Rapeseed]] oil, [[Benfluorex]], <br> Likely: [[Amphetamines]], L-[[Tryptophan]], [[Methamphetamine]] <br> Possible: [[Cocaine]], [[Phenylpropanolamine]], St.Johns wort, [[Chemotherapeutic agents]], [[SSRI]], [[Pergolide]].


|-
|-
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Endocrine'''
| '''Endocrine'''
|bgcolor="Beige"| [[Thyroid diseases]]
|bgcolor="Beige"| [[Thyroid diseases]].
|-  
|-  
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Environmental'''
| '''Environmental'''
|bgcolor="Beige"| [[ High altitude sickness|High Altitude(chronically)]]
|bgcolor="Beige"| [[ High altitude sickness|High Altitude(chronically)]].
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Gastroenterologic'''
| '''Gastroenterologic'''
|bgcolor="Beige"| [[Portal Hypertension]]
|bgcolor="Beige"| [[Portal Hypertension]].
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Hematologic'''
| '''Hematologic'''
|bgcolor="Beige"| [[Myeloproliferative disorders]], [[Paroxysmal nocturnal haemoglobinuria]], [[Polycythemia Vera]], [[Splenectomy]](due to 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"| [[Schistosoma japonicum]] and [[Schistosoma mansoni]], HHV8 also associated with [[Kaposi's sarcoma]]
|bgcolor="Beige"| [[Schistosoma japonicum]] and [[Schistosoma mansoni]], HHV-8.
|-
|-
|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
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|-bgcolor="LightSteelBlue"
|-bgcolor="LightSteelBlue"
| '''Overdose / Toxicity'''
| '''Overdose / Toxicity'''
|bgcolor="Beige"| [[Diethylpropion]], 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]], [[Langerhans 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"
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* [[Bronchopulmonary dysplasia]]
* [[Bronchopulmonary dysplasia]]
* [[Cholesterol ester storage disease]]
* [[Cholesterol ester storage disease]]
* [[Hemolytic anemia|Chronic hemolytic anemia]]
* Chronic [[hemolytic anemia]]
* [[Chronic obstructive pulmonary disease]]
* [[Chronic obstructive pulmonary disease]]
* [[renal failure|Chronic renal failure]] on [[dialysis]]
* Chronic [[renal failure]] on [[dialysis]]
* [[Churg-Strauss syndrome]]
* [[Churg-Strauss syndrome]]
* [[Coal workers' pneumoconiosis]]
* [[Coal workers' pneumoconiosis]]
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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]],[[Langerhans cell histiocytosis]], [[Neurofibromatosis]], [[Vasculitis]].
#'''Systemic disorders''': [[Sarcoidosis]], [[Langerhans cell histiocytosis]], [[Neurofibromatosis]], [[Vasculitis]].
#'''Metabolic disorders''': [[Glycogen storage diseases]], [[Gaucher disease]], [[Thyroid disease|thyroid disease]].
#'''Metabolic disorders''': [[Glycogen storage diseases]], [[Gaucher disease]], [[Thyroid disease|thyroid disease]].
#'''Miscellaneous''': Tumor obstruction, [[Fibrosing Mediastinitis|fibrosing mediastinitis]], chronic [[renal failure]] on [[dialysis]].
#'''Miscellaneous''': Tumor obstruction, [[Fibrosing Mediastinitis|fibrosing mediastinitis]], chronic [[renal failure]] on [[dialysis]].

Revision as of 16:36, 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 HHV-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, 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,

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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