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__NOTOC__ | __NOTOC__ | ||
{{Pulmonary hypertension}} | {{Pulmonary hypertension}} | ||
{{CMG}}; '''Assistant Editor(s)-in-Chief:''' [[User:Lisa Prior|Lisa Prior]] | |||
==Overview== | ==Overview== | ||
When approaching pulmonary hypertension, it is important to start with a detailed clinical history. The | When approaching pulmonary hypertension (PH), it is important to start 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. 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> | ||
==History== | ==History== | ||
* | * The average time of onset of initial symptoms of 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> | ||
* | This is perhaps primarily due to the non-specificity of symptoms and considerable overlap with symptoms of other pulmonary and cardiovascular diseases. It is for this reason that a detailed clinical history must be obtained. | ||
*Pulmonary ''arterial'' hypertension ('''PAH''') typically does not 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]] 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> | *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. | *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 | *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 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> | *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 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]]. | ||
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==Symptoms== | ==Symptoms== | ||
*[[Dyspnea]] (~60%) | |||
*[[Fatigue]] (~19%) | |||
*[[Raynaud's phenomenon]] (~10%) | |||
*[[Syncope]] (~8%) | |||
*[[Chest pain]] (~7%) | |||
*[[Dyspnea]] | |||
*[[Fatigue]] | |||
*[[Raynaud's phenomenon]] | |||
*[[Syncope]] | |||
*[[Chest pain]] | |||
**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> | **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> | **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]] | *[[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. <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> | ||
*[[Palpitation|Palpitations]] | *[[Palpitation|Palpitations]] (~5%) | ||
*[[Edema|Leg swelling]] | *[[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 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> | **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]] | *[[Cough]] (rare) | ||
*[[Hemoptysis]] | *[[Hemoptysis]] (rare) | ||
*[[Hoarseness]] | *[[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> | ||
Revision as of 13:40, 29 August 2014
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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 pulmonary hypertension (PH), it is important to start 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. The symptoms of PH include dyspnea, fatigue, and syncope.[1]
History
- The average time of onset of initial symptoms of PH to diagnosis is 2 years.[1]
This is perhaps primarily due to the non-specificity of symptoms and considerable overlap with symptoms of other pulmonary and cardiovascular diseases. It is for this reason that a detailed clinical history must be obtained.
- Pulmonary arterial hypertension (PAH) typically does not 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 or increased left atrial pressures etc. must be considered in the clinical history. [2]
- For example, 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 Pulmonary Arterial Hypertension as the underlying cause.
- A history of DVT/PE (deep venous thrombosis/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 Pulmonary Arterial Hypertension.
- 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 sought to see if there is a hereditary component at play.
Symptoms
- Dyspnea (~60%)
- Fatigue (~19%)
- Raynaud's phenomenon (~10%)
- Syncope (~8%)
- Chest pain (~7%)
- Anginal chest pain is purported to be due to increased myocardial oxygen demand in a strained right heart that is either hypertrophied or dilated.[2]
- 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. [2]
- Palpitations (~5%)
- Leg swelling (~3%)
- As systemic venous hypertension develops secondary to a failing right ventricle, leg swelling may be a feature of the condition as well as upper right abdominal discomfort (from hepatic congestion) and abdominal swelling (ascites). [3]
- Cough (rare)
- Hemoptysis (rare)
- Hoarseness (rare)[1]
- 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. [2]
WHO Functional Classification:
Clinically, a patient may be categorised into a particular class using the WHO modified functional classification system for pulmonary hypertension (modified from 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. See table below for WHO functional classification: [5]
| Class | WHO Functional Classification |
| I | 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. |
| II | 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. |
| III | 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. |
| IV | 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. |
References
- ↑ 1.0 1.1 1.2 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) - ↑ 2.0 2.1 2.2 2.3 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) - ↑ 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) - ↑ Harrison W. Farber; Hill, Nicholas Morison (2008). Pulmonary Hypertension (Contemporary Cardiology). Totowa, NJ: Humana Press. ISBN 1-58829-661-X.