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{{Pulmonary hypertension}}
{{Pulmonary hypertension}}
{{CMG}}; '''Assistant Editor(s)-in-Chief:''' [[User:Lisa Prior|Lisa Prior]]; {{Jose}}


'''Editor(s)-in-Chief:''' [[User:C Michael Gibson |C. Michael Gibson, M.S., M.D.]] [mailto:mgibson@perfuse.org] Phone:617-632-7753; '''Assistant Editor(s)-in-Chief:''' [[User:Lisa Prior|Lisa Prior]]
==Overview==
==Introduction==
The hallmark of [[pulmonary hypertension]] is [[progressive dyspnea]]. The most common symptoms of [[pulmonary hypertension]] include [[dyspnea]], [[fatigue]], [[chest pain]] and [[syncope]] or [[presyncope]]. [[Ortner syndrome]] may also be seen (characterized by [[hoarseness]] due to compression of the left laryngeal nerve caused by enlargement of the pulmonary artery).
When approaching pulmonary hypertension, it is important to commence with a detailed clinical history.
The '''presenting symptoms''' are important but a comprehensive '''past medical history''', '''medication history''', '''family and social history''' and '''review of systems''' may reveal further clues as to the etiology of the condition. 
This will be explored in more detail in the sections below.


==Symptoms==
==History==
The average time of onset of initial symptoms in [[Pulmonary hypertension|pulmonary hypertension]] (PH) to diagnosis is 2 years.<ref name="pmid3605900">{{cite journal |author=Rich S, Dantzker DR, Ayres SM, ''et al.'' |title=Primary pulmonary hypertension. A national prospective study |journal=Ann. Intern. Med. |volume=107 |issue=2 |pages=216–23 |year=1987 |month=August |pmid=3605900 |doi= |url=}}</ref>  
The history should be focused on the areas including:<ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref>
This is perhaps primarily due to non-specificity of symptoms and considerable overlap with symptoms of other pulmonary and cardiovascular diseases.  
* The average time from the onset of the initial symptoms of PH to the diagnosis is approximately 2 years.<ref name="pmid3605900">{{cite journal |author=Rich S, Dantzker DR, Ayres SM, ''et al.'' |title=Primary pulmonary hypertension. A national prospective study |journal=Ann. Intern. Med. |volume=107 |issue=2 |pages=216–23 |year=1987 |month=August |pmid=3605900 |doi= |url=}}</ref> This can  be attributed primarily due to the non-specificity of symptoms and the considerable overlap with symptoms of other pulmonary and cardiovascular diseases. Therefore, a detailed clinical history must be obtained.  
It for this reason, a detailed clinical history must be obtained.  
* Pulmonary arterial hypertension (PAH) does not typically present with [[orthopnea]] or [[paroxysmal nocturnal dyspnea]], while pulmonary venous hypertension typically does.
Presenting symptoms suggestive of pulmonary hypertension on initial presentation are as follows in order of likelihood (National Registry):
* Also, a history of exposure to [[cocaine]], [[methamphetamine]], [[alcohol]] leading to [[cirrhosis]], and smoking leading to [[emphysema]] are considered significant.


*[[Dyspnea]] - 60%
* Many conditions are associated with PH and symptoms suggestive of [[Liver|hepatic disease]], [[Congenital heart disease|congenital heart disease]], [[Thyroid disease|thyroid diseases]], and diseases that cause [[Hypoxia|hypoxia]] must be considered in the clinical history. 
*[[Fatigue]] - 19%
* If the patient complains of [[Snoring|snoring]] and [[Somnolence|daytime sleepiness]], then [[Sleep apnea|obstructive sleep apnea]] (OSA) under [[Pulmonary hypertension|group 3 hypoxic PH]] may be a likely culprit.
*[[Raynaud's phenomenon]] - 10%
* A cluster of associated symptoms such as skin changes, [[Raynaud's phenomenon]] and [[Arthralgia|joint pain]] may point towards a [[Connective tissue disease|connective tissue disorder]] under [[Pulmonary hypertension|group 1 PAH]] as the underlying cause.
*[[Syncope]] - 8%
* A history of [[deep vein thrombosis]] or [[pulmonary embolism]] may lead one to consider [[Pulmonary hypertension|group 4 chronic thromboembolic PH]].<ref name="isbn0-07-121971-4">{{cite book |author=Carolyn H. Welsh; Michael E. Hanley |title=Current diagnosis & treatment in pulmonary medicine |publisher=Lange Medical Books / McGraw-Hill |location=New York |year=2003 |pages= |isbn=0-07-121971-4 |oclc= |doi= |accessdate=}}</ref>  
*[[Chest pain]] - 7%
*It is important to gather a comprehensive medication history including use of over-the-counter medications and herbal supplements as well as illicit drug use as many substances are associated with [[Pulmonary hypertension|group 1 PAH]].
*[[Syncope|Near Syncope]] - 5%
* It is also wise to discern if the patient is high risk for [[Human Immunodeficiency Virus|HIV]] exposure as it has been shown that PH disease course is accelerated in HIV-affected patients.
*[[Palpitation|Palpitations]] - 5%
* Finally, a family history should be examined to see if there is a hereditary component at play.
*[[Edema|Leg swelling]] - 3%
*[[Cough]]- rare
*[[Hemoptysis]] - rare
*[[Hoarseness]] - rare <ref name="pmid3605900">{{cite journal |author=Rich S, Dantzker DR, Ayres SM, ''et al.'' |title=Primary pulmonary hypertension. A national prospective study |journal=Ann. Intern. Med. |volume=107 |issue=2 |pages=216–23 |year=1987 |month=August |pmid=3605900 |doi= |url=}}</ref>  


==Symptoms==
Symptoms of pulmonary hypertension include:<ref name="pmid12716138" />
*Progressive [dyspnea]] (~85%)
*[[Fatigue]] (~26%)
*[[Chest pain]] (~22%)
*[[Lower extremity edema]] (~20%)
**As systemic venous hypertension develops secondary to a failing right ventricle, [[Edema|leg swelling]] may be a feature of the condition in addition to upper right abdominal discomfort (from hepatic congestion) and abdominal swelling ([[Ascites|ascites]]).<ref name="isbn0-07-121971-4">{{cite book |author=Carolyn H. Welsh; Michael E. Hanley |title=Current diagnosis & treatment in pulmonary medicine |publisher=Lange Medical Books / McGraw-Hill |location=New York |year=2003 |pages= |isbn=0-07-121971-4 |oclc= |doi= |accessdate=}}</ref>
*[[Syncope]] or [[presyncope]](~17%)
*[[Raynaud's phenomenon]] (~10%)
**Anginal [[Chest pain|chest pain]] is thought to be due to increased myocardial oxygen demand in a strained right heart that is either hypertrophied or dilated.
**However, there have also been reports of angina due to decreased [[Myocardium|myocardial]] oxygen supply from compression of the left main [[Coronary circulation|coronary artery]] by a dilated pulmonary artery.<ref name="pmid10190427">{{cite journal |author=Kawut SM, Silvestry FE, Ferrari VA, ''et al.'' |title=Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension |journal=Am. J. Cardiol. |volume=83 |issue=6 |pages=984–6, A10 |year=1999 |month=March |pmid=10190427 |doi= |url=}}</ref>
*[[Syncope|Near Syncope]] (~5%)
**[[Syncope]] can occur through either reduced [[Cardiac output|cardiac output]], [[Cardiac arrhythmia|arrhythmias]] or ventricular [[Ischemia|ischemia]] and indicates pulmonary hypertension is severe.
*[[Palpitation|Palpitations]] (~5%)
*[[Cough]] (rare)
*[[Hemoptysis]] (rare)
*[[Hoarseness]] (rare)<ref name="pmid3605900">{{cite journal |author=Rich S, Dantzker DR, Ayres SM, ''et al.'' |title=Primary pulmonary hypertension. A national prospective study |journal=Ann. Intern. Med. |volume=107 |issue=2 |pages=216–23 |year=1987 |month=August |pmid=3605900 |doi= |url=}}</ref>
**Hoarseness ([[Ortner's syndrome]]) is thought to be due to compression of the left [[Recurrent laryngeal nerve|recurrent laryngeal nerve]] between a dilated pulmonary artery and the [[Aorta|aorta]].<ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref>


[[Dyspnea]] emerges as by far the most common symptom followed by fatigue.
==WHO Functional Classification==
These symptoms represent the inability of the [[Heart|heart]] to increase its output on exertion.
Clinically, a patient may be categorized based on the severity of symptoms into a particular class using the WHO modified functional classification system for [[Pulmonary hypertension|pulmonary hypertension]] (modified from [[Congestive heart failure classification|NYHA functional classification system]] for heart failure).  The baseline WHO functional classification is used for the assessment of the severity of PH in order to tailor the choice of therapy.  Shown below is a table summarizing the different functional classes.<ref>Rich S, Rubin LJ, Abenhail L, et al. Executive summary from the World Symposium on Primary Pulmonary Hypertension 1998, Evian, France, September 6-10, 1998. Geneva: The World Health Organization.</ref>
Anginal [[Chest pain|chest pain]] is purported to be due to increased myocardial oxygen demand in a strained right heart that is either hypertrophied or dilated.<ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref>
However, there have also been reports of angina due to decreased [[Myocardium|myocardial]] oxygen supply from compression of the left main [[Coronary circulation|coronary artery]] by a dilated pulmonary artery.<ref name="pmid10190427">{{cite journal |author=Kawut SM, Silvestry FE, Ferrari VA, ''et al.'' |title=Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension |journal=Am. J. Cardiol. |volume=83 |issue=6 |pages=984–6, A10 |year=1999 |month=March |pmid=10190427 |doi= |url=}}</ref>
[[Syncope]] can occur through either reduced [[Cardiac output|cardiac output]], [[Cardiac arrhythmia|arrhythmias]] or ventricular [[Ischemia|ischemia]] and indicates pulmonary hypertension is severe. <ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref>
As systemic venous hypertension develops secondary to a failing right ventricle, [[Edema|leg swelling]] may be a feature of the condition as well as upper right abdominal discomfort (from hepatic congestion) and abdominal swelling ([[Ascites|ascites]]). <ref name="isbn0-07-121971-4">{{cite book |author=Carolyn H. Welsh; Michael E. Hanley |title=Current diagnosis & treatment in pulmonary medicine |publisher=Lange Medical Books / McGraw-Hill |location=New York |year=2003 |pages= |isbn=0-07-121971-4 |oclc= |doi= |accessdate=}}</ref>
[[Cough]], [[Hemoptysis|hemoptysis]] and [[Hoarseness|hoarseness]] have been described but are relatively rare.  
Hoarseness ([[Ortner's syndrome]]) is thought to be due to compression of the left [[Recurrent laryngeal nerve|recurrent laryngeal nerve]] between a dilated pulmonary artery and the [[Aorta|aorta]]. <ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref>  


==WHO Functional Classification==
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
Clinically, a patient may be categorised into a particular class using the WHO modified functional classification system for [[Pulmonary hypertension|pulmonary hypertension]] (modified from [[Congestive heart failure classification|NYHA functional classification system]] for heart failure).
|-
The benefit of this model is that it provides a baseline for monitoring; it is a prognostic predictor and it guides therapy selection.
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Class''' || style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF" align="left" |'''Description'''
See table below for WHO functional classification: <ref name="isbn1-58829-661-X">{{cite book |author=Harrison W. Farber; Hill, Nicholas Morison |title=Pulmonary Hypertension (Contemporary Cardiology) |publisher=Humana Press |location=Totowa, NJ |year=2008 |pages= |isbn=1-58829-661-X |oclc= |doi= |accessdate=}}</ref>
 
{|border="1" cellpadding="5"
|- align = "center" bgcolour="LightSkyBlue"
|'''Class'''
|'''WHO Functional Classification'''
|-
|-
|I
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" | '''I''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
|Patients with pulmonary hypertension but without resulting limitations of physical activity. Ordinary physical activity does not cause undue fatigue or dyspnea, chest pain, or heart syncope.
* No limitation of usual physical activity
* No increased [[dyspnea]], [[fatigue]], [[chest pain]], or presyncope upon ordinary physical activity
|-
|-
|II  
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" |'''II''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
|Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity results in undue fatigue or dyspnea, chest pain, or heart syncope.
* Mild limitation of physical activity
* No discomfort at rest  
* Increased [[dyspnea]], [[fatigue]], [[chest pain]], or presyncope upon normal physical activity
|-
|-
|III  
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" |'''III''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
|Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary physical activity causes undue fatigue or dyspnea, chest pain, or heart syncope.
* Marked limitation of physical activity
* No discomfort at rest
* Increased [[dyspnea]], [[fatigue]], [[chest pain]], or presyncope upon less than ordinary activity
|-
|-
|IV  
| style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 30%" align="left" |'''IV''' || style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align="left" |
|Patients with pulmonary hypertension resulting in inability to carry on any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may be present even at rest. Discomfort is increased by physical activity.
* Inability to perform any physical activity at rest with/without signs of right ventricular failure
* Dyspnea and/or fatigue may be present at rest
* Increased [[dyspnea]], [[fatigue]], [[chest pain]], or presyncope by almost any physical activity
|}
|}


==Other aspects of clinical history==
Many conditions are associated with PH and symptoms suggestive of [[Liver|hepatic disease]], [[Congenital heart disease|congenital heart disease]], [[Thyroid disease|thyroid diseases]] and diseases that cause [[Hypoxia|hypoxia]] or increased left atrial pressures etc. must be considered in the clinical history. <ref name="pmid12716138">{{cite journal |author=Budev MM, Arroliga AC, Jennings CA |title=Diagnosis and evaluation of pulmonary hypertension |journal=Cleve Clin J Med |volume=70 Suppl 1 |issue= |pages=S9–17 |year=2003 |month=April |pmid=12716138 |doi= |url=}}</ref> 
For example, if the patient complains of [[Snoring|snoring]] and [[Somnolence|daytime sleepiness]], then [[Sleep apnea|Obstructive Sleep Apnea]] (OSA) under [[Pulmonary hypertension|Group 3 Hypoxic PH]] may be a likely culprit.
A cluster of associated symptoms such as [[Rash|rash]], [[Raynaud's phenomenon]] and [[Arthralgia|joint pain]] may point towards a [[Connective tissue disease|connective tissue disorder]] under [[Pulmonary hypertension|Group 1 Pulmonary Arterial Hypertension]] as the underlying cause.
A history of [[Deep vein thrombosis|DVT]]/[[Pulmonary embolism|PE]] (deep venous thrombosis/pulmonary embolism) may lead one to consider [[Pulmonary hypertension|Group 4 Chronic Thromboembolic PH]]. <ref name="isbn0-07-121971-4">{{cite book |author=Carolyn H. Welsh; Michael E. Hanley |title=Current diagnosis & treatment in pulmonary medicine |publisher=Lange Medical Books / McGraw-Hill |location=New York |year=2003 |pages= |isbn=0-07-121971-4 |oclc= |doi= |accessdate=}}</ref>
It is important to gather a comprehensive medication history including use of over-the-counter medications and herbal supplements as well as illicit drug use as many substances are associated with [[Pulmonary hypertension|Group 1 Pulmonary Arterial Hypertension]].
It is also wise to discern if the patient is high risk for [[Human Immunodeficiency Virus|HIV]] exposure as it has been shown that PH disease course is accelerated in HIV-affected patients.
Finally, a family history should be sought to see if there is a hereditary component at play.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
 
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Latest revision as of 14:17, 9 June 2021

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor(s)-in-Chief: Lisa Prior; José Eduardo Riceto Loyola Junior, M.D.[2]

Overview

The hallmark of pulmonary hypertension is progressive dyspnea. The most common symptoms of pulmonary hypertension include dyspnea, fatigue, chest pain and syncope or presyncope. Ortner syndrome may also be seen (characterized by hoarseness due to compression of the left laryngeal nerve caused by enlargement of the pulmonary artery).

History

The history should be focused on the areas including:[1]

  • The average time from the onset of the initial symptoms of PH to the diagnosis is approximately 2 years.[2] This can be attributed primarily due to the non-specificity of symptoms and the considerable overlap with symptoms of other pulmonary and cardiovascular diseases. Therefore, a detailed clinical history must be obtained.
  • Pulmonary arterial hypertension (PAH) does not typically present with orthopnea or paroxysmal nocturnal dyspnea, while pulmonary venous hypertension typically does.
  • Also, a history of exposure to cocaine, methamphetamine, alcohol leading to cirrhosis, and smoking leading to emphysema are considered significant.

Symptoms

Symptoms of pulmonary hypertension include:[1]

WHO Functional Classification

Clinically, a patient may be categorized based on the severity of symptoms into a particular class using the WHO modified functional classification system for pulmonary hypertension (modified from NYHA functional classification system for heart failure). The baseline WHO functional classification is used for the assessment of the severity of PH in order to tailor the choice of therapy. Shown below is a table summarizing the different functional classes.[5]

Class Description
I
  • No limitation of usual physical activity
  • No increased dyspnea, fatigue, chest pain, or presyncope upon ordinary physical activity
II
  • Mild limitation of physical activity
  • No discomfort at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope upon normal physical activity
III
  • Marked limitation of physical activity
  • No discomfort at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope upon less than ordinary activity
IV
  • Inability to perform any physical activity at rest with/without signs of right ventricular failure
  • Dyspnea and/or fatigue may be present at rest
  • Increased dyspnea, fatigue, chest pain, or presyncope by almost any physical activity

References

  1. ↑ 1.0 1.1 1.2 Budev MM, Arroliga AC, Jennings CA (2003). "Diagnosis and evaluation of pulmonary hypertension". Cleve Clin J Med. 70 Suppl 1: S9–17. PMID 12716138. Unknown parameter |month= ignored (help)
  2. ↑ 2.0 2.1 Rich S, Dantzker DR, Ayres SM; et al. (1987). "Primary pulmonary hypertension. A national prospective study". Ann. Intern. Med. 107 (2): 216–23. PMID 3605900. Unknown parameter |month= ignored (help)
  3. ↑ 3.0 3.1 Carolyn H. Welsh; Michael E. Hanley (2003). Current diagnosis & treatment in pulmonary medicine. New York: Lange Medical Books / McGraw-Hill. ISBN 0-07-121971-4.
  4. ↑ Kawut SM, Silvestry FE, Ferrari VA; et al. (1999). "Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension". Am. J. Cardiol. 83 (6): 984–6, A10. PMID 10190427. Unknown parameter |month= ignored (help)
  5. ↑ Rich S, Rubin LJ, Abenhail L, et al. Executive summary from the World Symposium on Primary Pulmonary Hypertension 1998, Evian, France, September 6-10, 1998. Geneva: The World Health Organization.

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