Pulmonary hypertension causes

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ralph Matar; Mohamed Moubarak, M.D. [2]

Overview

Pulmonary hypertension can be classified into primary pulmonary hypertension(of unknown cause) which is currently known as idiopathic pulmonary arterial hypertension (IPAH), and secondary pulmonary hypertension (due to another medical condition). The most common cause of pulmonary hypertension is left heart failure, other common causes include HIV, systemic sclerosis, portal hypertension, sickle cell disease, and congenital heart disease.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated. Pulmonary hypertension in itself is not a life threatening condition, but it is progressively fatal if left untreated.

Common Causes

The most common cause of pulmonary hypertension is left heart failure leading to pulmonary venous hypertension. Other Common causes of pulmonary arterial hypertension (PAH) include:

• Cor Pulmonale( Right heart failure due to pulmonary disease)
• Congestive heart failure
• Congenital heart disease
• COPD
• Familial Pulmonary Hypertension
• HIV
• Interstitial lung disease
• Mitral stenosis
• Obstructive sleep apnea
• Portal hypertension
• Pickwickian syndrome
• Right sided valvular disease
• Systemic sclerosis
• Sickle cell disease

Idiopathic Pulmonary Arterial Hypertension

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

Causes 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 and Toxin Side Effect	Definite: Aminorex, fenfluramine, dexfenfluramine, toxic rapeseed oil, benfluorex, Likely: Amphetamines, L-tryptophan, methamphetamine Possible: Cocaine, phenylpropanolamine, St John's 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,

Causes in Alphabetical Order

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 High Altitude(chronically) Idiopathic pulmonary haemosiderosis Indian familial childhood cirrhosis Interstitial Lung Disease Idiopathic spinal scoliosis 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

Causes by Clinical Classification

Class 1: Pulmonary Arterial 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.