Pulmonary hypertension causes

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Differential Diagnosis of the causes of Pulmonary Hypertension

(By Organ System)

Cardiovascular	Atrial Septal Defects, Cor triatum, Left heart Dysfunction or Failure, Teralogy of Fallot, Persistent fetal circulation, Mitral Valve stenosis and incompetence, Ventricular Septal Defect
Chemical / poisoning	Coal worker pneumoconiosis
Dermatologic	Neurofibromatosis, Systemic lupus erythematosus
Drug amd Toxin Side Effect	Definite: Aminorex, Fenfluramine, Dexfenfluramine, Toxic rapeseed oil, Benfluorex, Likely: Amphetamines, L-Tryptophan, Methamphetamines Possible: Cocaine, Phenylpropanolamine, St.Johns wort, Chemotherapeutic agents, SSRI, Pergolide.
Ear Nose Throat	Pickwickian syndrome.
Endocrine	Thyroid disorders
Environmental	Altitude sickness,chronic
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 discorders, Paroxysmal nocturnal haemoglobinuria, Polycythemia Vera, Splenectomy(Thrombophilia).
Iatrogenic	No underlying cause
Infectious Disease	Shistosoma japonicum and 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	Diethypropion, 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, Langerhan 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,

(In Alphabetical Order)

• Alveolar capillary dysplasia with misalignment of pulmonary veins
• Atrial Septal Defects
• Altitude sickness, chronic
• Bronchiectasis
• Bronchopulmonary Dysplasia
• Cholesterol ester storage disease
• Chronic obstructive pulmonary disease
• Cor triatriatum
• Cystic fibrosis
• Churg-Strauss syndrome
• Coal worker pneumoconiosis
• Chronic renal failure on dialysis
• Chronic hemolytic anemia
• Diastolic left heart dysfunction
• Diethylpropion
• Fallot tetralogy
• Fetal circulation, persistent
• Fibrosing mediastinitis
• Gaucher disease
• Glycogen storage diseases
• Idiopathic pulmonary haemosiderosis
• Idiopathic spinal scoliosis
• Indian familial childhood cirrhosis
• Interstitial Lung Disease
• Langerhan cell Histiocytosis
• Mitral valve incompetence
• Mitral valve stenosis
• Monocrotaline poisoning
• Myeloproliferative disorders
• Neurofibromatosis
• Obstructive sleep apnoea
• Pulmonary alveolar microlithiasis
• Pickwickian syndrome
• Paroxysmal nocturnal haemoglobinuria
• Portal hypertension
• Polycythemia vera
• Pulmonary fibrosis
• Pulmonary embolism
• Pulmonary veno-occlusive disease
• Pulmonary capillary hemangiomatosis
• Phentermine poisoning
• Primary pulmonary hypertension
• Sarcoidosis
• Schistosoma japonicum
• Schistosoma mansoni
• Splenectomy
• Systemic lupus erythematosus
• Systolic Left heart dysfunction
• Tropical pulmonary eosinophilia
• Vasculitis
• Ventricular septal defect

(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,Langerhan cell Histiocytosis, Neurofibromatosis, Vasculitis.
3. Metabolic disorders: Glycogen storage diseases, Gaucher disease, Thyroid disorders.
4. Miscellaneous: Tumor obstruction, Fibrosing mediastinitis, chronic renal failure on dialysis.