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==Overview==
==Overview==
When approaching a patient with suspected or confirmed pulmonary hypertension (PH), it is important to elicit a detailed clinical history. The presenting symptoms are important but a comprehensive past medical history, medication history, family history, social history, and review of systems may reveal further clues as to the etiology of the condition.  The symptoms of PH include [[dyspnea]], [[fatigue]], and [[syncope]].<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>
When approaching a patient with suspected or confirmed pulmonary hypertension (PH), it is important to elicit a detailed clinical history. The presenting symptoms are important but a comprehensive past medical history, medication history, family history, social history, and review of systems may reveal further clues as to the etiology of the condition.  The symptoms of PH include [[dyspnea]], [[fatigue]], and [[syncope]].


==History==
==History==
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>
* 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.  
* 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.  
* Pulmonary arterial hypertension (PAH) does not typically present with [[orthopnea]] or [[paroxysmal nocturnal dyspnea]], while pulmonary venous hypertension typically does.
* 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.
* Also, a history of exposure to [[cocaine]], [[methamphetamine]], [[alcohol]] leading to [[cirrhosis]], and smoking leading to [[emphysema]] are considered significant.


* 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.<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>  
* 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.   
* 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.
* 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 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.  
* 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.  
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==Symptoms==
==Symptoms==
Symptoms of pulmonary hypertension include:<ref name="pmid12716138" />
*[[Dyspnea]] (~60%)
*[[Dyspnea]] (~60%)
*[[Fatigue]] (~19%)
*[[Fatigue]] (~19%)
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*[[Syncope]] (~8%)
*[[Syncope]] (~8%)
*[[Chest pain]] (~7%)
*[[Chest pain]] (~7%)
**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.<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>
**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>  
**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|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. <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>
**[[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%)
*[[Palpitation|Palpitations]] (~5%)
*[[Edema|Leg swelling]] (~3%)
*[[Edema|Leg swelling]] (~3%)
**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>
**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>
*[[Cough]] (rare)
*[[Cough]] (rare)
*[[Hemoptysis]] (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]] (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>  
**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==
==WHO Functional Classification==
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{| style="cellpadding=0; cellspacing= 0; width: 600px;"
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
|-
|-
| 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'''
| 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'''
|-
|-
| 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 |No limitation of usual physical activity <br>No increased dyspnea, fatigue, chest pain, or presyncope upon ordinary physical activity
| 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" |
* No limitation of usual physical activity
* No increased [[dyspnea]], [[fatigue]], [[chest pain]], or presyncope upon ordinary physical activity
|-
|-
| 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|Mild limitation of physical activity <br> No discomfort at rest <br> Increased dyspnea, fatigue, chest pain, or presyncope upon normal physical activity
| 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" |
* Mild limitation of physical activity
* No discomfort at rest  
* Increased [[dyspnea]], [[fatigue]], [[chest pain]], or presyncope upon normal physical activity
|-
|-
| 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|Marked limitation of physical activity <br> No discomfort at rest <br> Increased dyspnea, fatigue, chest pain, or presyncope upon less than ordinary activity
| 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" |
* Marked limitation of physical activity
* No discomfort at rest
* Increased [[dyspnea]], [[fatigue]], [[chest pain]], or presyncope upon less than ordinary activity
|-
|-
| 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 |Inability to perform any physical activity at rest with/without signs of right ventricular failure <br> Dyspnea and/or fatigue may be present at rest <br>  Increased dyspnea, fatigue, chest pain, or presyncope by almost any physical activity
| 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" |
* 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
|}
|}


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Revision as of 14:54, 27 March 2018

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

Overview

When approaching a patient with suspected or confirmed pulmonary hypertension (PH), it is important to elicit a detailed clinical history. The presenting symptoms are important but a comprehensive past medical history, medication history, family history, social history, and review of systems may reveal further clues as to the etiology of the condition. The symptoms of PH include dyspnea, fatigue, and syncope.

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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