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{{Pulmonary hypertension}}
{{Pulmonary hypertension}}
{{CMG}}; '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]]
{{CMG}}; '''Assistant Editor(s)-in-Chief:''' [[User:Ralph Matar|Ralph Matar]]; {{Rim}}


==Overview==
==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]], [[inflammation#Chronic inflammation|chronic inflammatory disorders]], [[myeloproliferative syndrome]]s, and [[splenectomy]]) are at an increased risk for chronic [[thromboembolic]] pulmonary hypertension.
[[BMPR2]] [[mutation]], [[systemic sclerosis]], [[HIV infection]], [[portal hypertension]], [[fenfluramine]] use, [[congenital heart disease]] with [[shunt]], recent acute [[pulmonary embolism]], and [[sickle cell disease]] are established risk factors for development of [[pulmonary arterial hypertension]]. On the other hand, patients in a hypercoagulable state (such as the presence of [[lupus anticoagulant]], deficiency of [[protein C]], [[protein S]], or [[antithrombin III]], [[inflammation#Chronic inflammation|chronic inflammatory disorders]], [[myeloproliferative syndrome]]s, and [[splenectomy]]) are at an increased risk for chronic thromboembolic pulmonary hypertension.<ref name="McLaughlin-2009">{{Cite journal  | last1 = McLaughlin | first1 = VV. | last2 = Archer | first2 = SL. | last3 = Badesch | first3 = DB. | last4 = Barst | first4 = RJ. | last5 = Farber | first5 = HW. | last6 = Lindner | first6 = JR. | last7 = Mathier | first7 = MA. | last8 = McGoon | first8 = MD. | last9 = Park | first9 = MH. | title = 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. | journal = Circulation | volume = 119 | issue = 16 | pages = 2250-94 | month = Apr | year = 2009 | doi = 10.1161/CIRCULATIONAHA.109.192230 | PMID = 19332472 }}</ref>


==Risk Factors==
==Risk Factors==
Risk factors of pulmonary hypertension are divided as follows:<ref name="McLaughlin-2009">{{Cite journal  | last1 = McLaughlin | first1 = VV. | last2 = Archer | first2 = SL. | last3 = Badesch | first3 = DB. | last4 = Barst | first4 = RJ. | last5 = Farber | first5 = HW. | last6 = Lindner | first6 = JR. | last7 = Mathier | first7 = MA. | last8 = McGoon | first8 = MD. | last9 = Park | first9 = MH. | title = 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. | journal = Circulation | volume = 119 | issue = 16 | pages = 2250-94 | month = Apr | year = 2009 | doi = 10.1161/CIRCULATIONAHA.109.192230 | PMID = 19332472 }}</ref>


===Gender===
===Pulmonary Arterial Hypertension===
 
* [[BMPR2]] gene mutation
Females are at least two-and-a-half more likely to have idiopathic and familial pulmonary hypertension than males.
* [[ALK1]] gene mutation
 
* [[Connective tissue disease]]
===Family History===
* [[HIV]] infection
 
* [[Portal hypertension]]
A positive family history is defined by having two or more family members with a gene mutation causing pulmonary hypertension.
* [[Congenital heart disease]]
 
* [[Schistosomiasis]]
===Co-morbidities===
* Chronic [[hemolytic anemia]]
 
* [[Drug]]s and [[toxin]]s
Certain diseases or conditions, such as heart and [[lung diseases]], [[liver disease]], [[HIV]] infection, [[connective tissue disorders]] like [[scleroderma]] and [[lupus]] are associated with pulmonary hypertension.
 
===Obstructive Sleep Apnea===
 
Obstructive sleep apnea predisposes to pulmonary hypertension by causing a drop in oxygen level when the patient is asleep.
 
===Predisposition to Recurrent Pulmonary Embolism===
 
===Drugs and Toxins use===
 
The use of street drugs (such as [[cocaine]]) or certain appetite suppressors like methamphetamines and the diet drug “fen phen” predisposes to having pulmonary hypertension.
 
===Chronic Hypoxemia===


Living at high altitudes for a long period of time is a risk factor.
Shown below is a table summarizing the list of drugs associated with PAH:


===Pregnancy===
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 25%" align="center" |Definite risk || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 25%" align="center" |Possible risk || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 25%" align="center" |Likely risk || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 25%" align="center" |Unlikely risk
|-
| style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* [[Aminorex]]
* [[Fenfluramine]]
* [[Dexfenfluramine]]
* Toxic rapeseed oil
* [[Benfluorex]]
| style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* [[Cocaine]]
* [[Phenylpropanolamine]]
* [[St John's wort]]
* [[Chemotherapy]]
* [[Selective serotonin reuptake inhibitor]]s
* [[Pergolide]]
| style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* [[Amphetamine]]
* [[L-tryptophan]]
* [[Methamphetamine]]
| style="font-size: 100; padding: 0 5px; background: #F5F5F5" align="left" |
* [[Oral contraceptive]]
* [[Estrogen|Estrogen therapy]]
* [[Smoking]]
|}


*While [[pregnancy]] in itself is not considered a risk factor for pulmonary hypertension, a female who gets pregnant and has pulmonary hypertension carries a higher risk of mortality, for this reason, pregnancy is contraindicated in patients with PAH.
===Pulmonary hypertension due to Left Heart Disease===
* [[Systolic dysfunction]]
* [[Diastolic dysfunction]]
* Left [[valvular disease]]


*If needed, birth control pills containing the lowest amount of estrogen are recommended. However, nearly half of the specialists did not advocate using birth control pills for their patients, and some actively discouraged patients from doing so because of concern over the possible role of estrogen in worsening PAH.<ref>Consensus statement issued by the scientific leadership council,  birth control and hormonal therapy in pulmonary arterial hypertension.</ref>
===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


===Thalassemia===
===Chronic Thromboembolic Pulmonary Hypertension===
* [[Pulmonary embolism]]
* [[Hypercoagulability]] ([[lupus anticoagulant]], deficiency of [[protein C]], [[protein S]], or [[antithrombin III]], [[inflammation|chronic inflammatory disorder]]s, [[myeloproliferative syndrome]]s, and [[splenectomy]])


Pulmonary hypertension is present in 2.1% of patients with [[thalassemia]], for whom advanced age and splenectomy are risk factors.<ref name="pmid24081970">{{cite journal| author=Derchi G, Galanello R, Bina P, Cappellini MD, Piga A, Lai ME et al.| title=Prevalence and Risk Factors for Pulmonary Arterial Hypertension in a Large Group of β-Thalassemia Patients Using Right Heart Catheterization: A Webthal Study. | journal=Circulation | year= 2014 | volume= 129 | issue= 3 | pages= 338-45 | pmid=24081970 | doi=10.1161/CIRCULATIONAHA.113.002124 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24081970  }} </ref>
===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==
==References==
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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

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

Chronic Thromboembolic Pulmonary Hypertension

PH with Unclear and/or Multifactorial Mechanisms

References

  1. 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 |month= ignored (help)

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