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, congenital heart disease and sickle cell 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. Pulmonary embolism and acute left heart failure are two causes of pulmonary hypertension that can be quickly fatal.

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 defect, 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, benfluorex, dexfenfluramine, fenfluramine, toxic rapeseed oil, benfluorex, Likely: Amphetamines, L-tryptophan, methamphetamine Possible: Cocaine, Lorcaserin, phentermine, phenylpropanolamine, pergolide, St John's wort, chemotherapeutic agents, SSRI, Aprotinin
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, sickle cell disease
Iatrogenic	No underlying cause
Infectious Disease	HHV-8, schistosoma japonicum and schistosoma 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	Diethylpropion, monocrotaline, phentermine, radiation exposure (fibrosing mediastinitis and pulmonary fibrosis).
Psychiatric	No underlying causes
Pulmonary	Idiopathic pulmonary hemosiderosis, 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, ENG, SMAD9, CAV1, KCNK3)
3. Drug and toxin induced

• Aminorex
• Fenfluramine
• Dexfenfluramine
• Toxic rapeseed oil
• Benfluorex
• SSRIs

Class 2: Pulmonary hypertension due to left heart disease

1. Left ventricular systolic dysfunction
2. Left ventricular diastolic dysfunction
3. Valvular disease
4. Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies

Class 3: Pulmonary hypertension due to lung diseases and/or hypoxia

1. Chronic obstructive pulmonary disease
2. Interstitial lung disease
3. Other pulmonary diseases with mixed restrictive and obstructive pattern
4. Sleep-disordered breathing
5. Alveolar hypoventilation disorders
6. Chronic exposure to high altitude
7. Developmental lung diseases

Class 4: Chronic thromboembolic pulmonary hypertension

Class 5: Pulmonary hypertension with unclear multifactorial mechanisms

1. Hematologic disorders: chronic hemolytic anemia, Myeloproliferative disorders, splenectomy,
2. Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
4. Miscellaneous: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH