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

(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
• Eisenmenger syndrome
• 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
• 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.