Pulmonary hypertension history and symptoms: Difference between revisions

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Latest revision as of 14:17, 9 June 2021

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.

Many conditions are associated with PH and symptoms suggestive of hepatic disease, congenital heart disease, thyroid diseases, and diseases that cause hypoxia must be considered in the clinical history.
If the patient complains of snoring and daytime sleepiness, then obstructive sleep apnea (OSA) under group 3 hypoxic PH may be a likely culprit.
A cluster of associated symptoms such as skin changes, Raynaud's phenomenon and joint pain may point towards a connective tissue disorder under group 1 PAH as the underlying cause.
A history of deep vein thrombosis or pulmonary embolism may lead one to consider group 4 chronic thromboembolic PH.^[3]
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 group 1 PAH.
It is also wise to discern if the patient is high risk for HIV exposure as it has been shown that PH disease course is accelerated in HIV-affected patients.
Finally, a family history should be examined to see if there is a hereditary component at play.

Symptoms

Symptoms of pulmonary hypertension include:^[1]

Progressive [dyspnea]] (~85%)
Fatigue (~26%)
Chest pain (~22%)
Lower extremity edema (~20%)
- As systemic venous hypertension develops secondary to a failing right ventricle, leg swelling may be a feature of the condition in addition to upper right abdominal discomfort (from hepatic congestion) and abdominal swelling (ascites).^[3]
Syncope or presyncope(~17%)
Raynaud's phenomenon (~10%)
- Anginal 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 myocardial oxygen supply from compression of the left main coronary artery by a dilated pulmonary artery.^[4]
Near Syncope (~5%)
- Syncope can occur through either reduced cardiac output, arrhythmias or ventricular ischemia and indicates pulmonary hypertension is severe.
Palpitations (~5%)
Cough (rare)
Hemoptysis (rare)
Hoarseness (rare)^[2]
- Hoarseness (Ortner's syndrome) is thought to be due to compression of the left recurrent laryngeal nerve between a dilated pulmonary artery and the aorta.^[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

↑ ^1.0 ^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)
↑ ^2.0 ^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)
↑ ^3.0 ^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.
↑ 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)
↑ 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.

Template:WikiDoc Sources

[pmid12716138-1] 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)

[pmid3605900-2] 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)

[isbn0-07-121971-4-3] 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.

[pmid10190427-4] 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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[5]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Pulmonary hypertension}}
-{{CMG}}; '''Assistant Editor(s)-in-Chief:''' [[User:Lisa Prior|Lisa Prior]]
+{{CMG}}; '''Assistant Editor(s)-in-Chief:''' [[User:Lisa Prior|Lisa Prior]]; {{Jose}}
 ==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>
+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:<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.
 * 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.
-* 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.
 * 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.
@@ Line 17: / Line 18: @@
 *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]].
 * 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.
+* Finally, a family history should be examined to see if there is a hereditary component at play.
 ==Symptoms==
-*[[Dyspnea]] (~60%)
+Symptoms of pulmonary hypertension include:<ref name="pmid12716138" />
-*[[Fatigue]] (~19%)
+*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%)
-*[[Syncope]] (~8%)
+**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.
-*[[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>
 **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. <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%)
-*[[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>
 *[[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>
+**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==
 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>
 {| 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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