Pulmonary hypertension risk factors
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor(s)-in-Chief: Ralph Matar; Rim Halaby, M.D. [2]
Overview
Pulmonary hypertension (PH) is a multifactorial disease involving genetic and environmental risk factors. Risk factors for pulmonary arterial hypertension include BMPR2 mutation, connective tissue disease, HIV infection, portal hypertension, fenfluramine use, and congenital heart disease with a shunt. Left heart and lung diseases are risk factors for PH. Patients with a hypercoagulable state (such as the presence of lupus anticoagulant, deficiency of protein C, protein S, or antithrombin III, chronic inflammatory disorders, myeloproliferative syndromes, and splenectomy) are at an increased risk for chronic thromboembolic pulmonary hypertension.
Risk Factors
Risk factors of pulmonary hypertension are divided as follows:[1]
Pulmonary Arterial Hypertension
- BMPR2 gene mutation
- ALK1 gene mutation
- Connective tissue disease
- HIV infection
- Portal hypertension
- Congenital heart disease
- Schistosomiasis
- Chronic hemolytic anemia
- Drugs and toxins
Shown below is a table summarizing the list of drugs associated with PAH:
| Definite risk | Possible risk | Likely risk | Unlikely risk |
|
Pulmonary hypertension due to Left Heart Disease
Pulmonary Hypertension due to Lung Diseases and/or Hypoxia
- Alveolar hypoventilation disorders
- Chronic exposure to high altitude
- Chronic obstructive pulmonary disease
- Developmental abnormalities
- Interstitial lung disease
- Pulmonary diseases with mixed restrictive and obstructive pattern
- Sleep related breathing problems
Chronic Thromboembolic Pulmonary Hypertension
- Pulmonary embolism
- Hypercoagulability (lupus anticoagulant, deficiency of protein C, protein S, or antithrombin III, chronic inflammatory disorders, myeloproliferative syndromes, and splenectomy)
PH with Unclear and/or Multifactorial Mechanisms
- Myeloproliferative disease
- Splenectomy
- Sarcoidosis
- Histiocytosis
- Lymphangioleiomyomatosis
- Neurofibromatosis
- Vasculitis
- Glycogen storage disease
- Gaucher disease
- Thyroid disorder
- Obstruction by tumor
- Fibrosing mediastinitis
- Chronic renal failure on dialysis
References
- ↑ McLaughlin, VV.; Archer, SL.; Badesch, DB.; Barst, RJ.; Farber, HW.; Lindner, JR.; Mathier, MA.; McGoon, MD.; Park, MH. (2009). “ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association”. Circulation. 119 (16): 2250–94. doi:10.1161/CIRCULATIONAHA.109.192230. PMID 19332472. Unknown parameter
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