Pulmonary hypertension causes

Assistant Editor(s)-in-Chief: Ralph Matar,

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, 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.

Idiopathic Pulmonary Arterial Hypertension
When none of the causes on this page can be found, the disease is termed idiopathic pulmonary arterial hypertension (IPAH).

Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(By Organ System)

Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(In Alphabetical Order)
 * High Altitude(chronically)
 * Alveolar capillary dysplasia with misalignment of pulmonary veins
 * Atrial Septal Defects
 * Bronchiectasis
 * Bronchopulmonary dysplasia
 * Cholesterol ester storage disease
 * Chronic hemolytic anemia
 * Chronic obstructive pulmonary disease
 * Chronic renal failure on dialysis
 * Churg-Strauss syndrome
 * Coal workers' pneumoconiosis
 * Cor triatriatum
 * Cystic fibrosis
 * Diastolic 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
 * Langerhans cell histiocytosis
 * Mitral valve insufficiency
 * Mitral valve stenosis
 * Monocrotaline poisoning
 * Myeloproliferative disorders
 * Neurofibromatosis
 * Obstructive sleep apnea
 * Paroxysmal nocturnal haemoglobinuria
 * Phentermine poisoning
 * Pickwickian syndrome
 * Portal hypertension
 * Polycythemia vera
 * Pulmonary alveolar microlithiasis
 * Pulmonary capillary hemangiomatosis
 * Pulmonary embolism
 * Pulmonary fibrosis
 * Pulmonary veno-occlusive disease
 * Sarcoidosis
 * Schistosoma japonicum
 * Schistosoma mansoni
 * Sickle cell disease
 * Splenectomy
 * Systemic lupus erythematosus
 * Systolic dysfunction
 * Tropical pulmonary eosinophilia
 * Vasculitis
 * Ventricular septal defect

Complete Differential Diagnosis of the Causes of Pulmonary Hypertension
(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.