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==Overview== | ==Overview== | ||
When approaching pulmonary hypertension (PH), it is important to | 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> | ||
==History== | ==History== | ||
* The average time | * 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. | ||
This | * Pulmonary ''arterial'' hypertension ('''PAH''') does not typically present with [[orthopnea]] or [[paroxysmal nocturnal dyspnea]], while pulmonary ''venous'' hypertension typically does. | ||
*Pulmonary ''arterial'' hypertension ('''PAH''') | * 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> | ||
* | * 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 Pulmonary Arterial Hypertension]] 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 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]]. | ||
*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. | * 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 sought to see if there is a hereditary component at play. | ||
==Symptoms== | ==Symptoms== | ||
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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.[1]
History
- The average time from the onset of the initial symptoms of PH to the diagnosis is approximately 2 years.[1] 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 or increased left atrial pressures etc. must be considered in the clinical history. [2]
- 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 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 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) - ↑ 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.