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]; José Eduardo Riceto Loyola Junior, M.D.[3]
Overview
Pulmonary hypertension may be caused by either left heart failure (the most common cause) or other somewhat common causes such as HIV, systemic sclerosis, portal hypertension, congenital heart disease, and sickle cell disease. The World Health Organization (WHO) has classified PH based on etiology into five distinct groups: Group 1 (pulmonary arterial hypertension), Group 2 (PH due to left heart failure), Group 3 (PH due to chronic lung disease and/or hypoxemia), Group 4 (PH due to chronic thromboembolic disease), and Group 5 (PH due to multifactorial mechanisms).
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 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:[1][2][3][4]
- Cor pulmonale (right heart failure due to pulmonary disease)
- Congestive heart failure
- Congenital heart disease
- Chronic pulmonary thromboembolism
- COPD
- Familial Pulmonary Hypertension
- HIV
- Interstitial lung disease
- Mitral stenosis
- Obstructive sleep apnea
- Portal hypertension
- Pickwickian syndrome
- Right-sided valvular disease
- Systemic sclerosis
- Systemic lupus erythematosus
- Sickle cell disease
- Stimulant drugs such as amphetamines
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
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
- Pergolide
- 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
- Idiopathic pulmonary arterial hypertension
- Heritable (BMPR2, ALK-1, ENG, SMAD9, CAV1, KCNK3)
- Drug and toxin induced
Class 2: Pulmonary hypertension due to left heart disease
- Left ventricular systolic dysfunction
- Left ventricular diastolic dysfunction
- Valvular disease
- Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
Class 3: Pulmonary hypertension due to lung diseases and/or hypoxia
- Chronic obstructive pulmonary disease
- Interstitial lung disease
- Other pulmonary diseases with mixed restrictive and obstructive pattern
- Sleep-disordered breathing
- Alveolar hypoventilation disorders
- Chronic exposure to high altitude
- Developmental lung diseases
Class 4: Chronic thromboembolic pulmonary hypertension
Class 5: Pulmonary hypertension with unclear multifactorial mechanisms
- Hematologic disorders: Chronic hemolytic anemia, Myeloproliferative disorders, splenectomy,
- Systemic disorders: Sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
- Metabolic disorders: Glycogen storage disease, Gaucher disease, thyroid disorders
- Miscellaneous: Tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH
References
- ↑ Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K; et al. (2004). "Pulmonary hypertension as a risk factor for death in patients with sickle cell disease". N Engl J Med. 350 (9): 886–95. doi:10.1056/NEJMoa035477. PMID 14985486.
- ↑ Nayak NC, Chitale AR (2013). "Indian childhood cirrhosis (ICC) & ICC-like diseases: the changing scenario of facts versus notions". Indian J Med Res. 137 (6): 1029–42. PMC 3734708. PMID 23852284.
- ↑ Schultze AE, Roth RA (1998). "Chronic pulmonary hypertension--the monocrotaline model and involvement of the hemostatic system". J Toxicol Environ Health B Crit Rev. 1 (4): 271–346. doi:10.1080/10937409809524557. PMID 9776954.
- ↑ Kashyap S, Mohapatra PR (2013). "Pulmonary alveolar microlithiasis". Lung India. 30 (2): 143–7. doi:10.4103/0970-2113.110424. PMC 3669555. PMID 23741096.