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==Overview==
==Overview==
'''(Cardiac) syndrome X''' is [[Angina pectoris|angina]] (chest pain) associated with objective evidence of myocardial ischemia in the setting of normal epicardial  [[coronary artery|coronary arteries]]. It is thought to involve the coronary microvasculature rather than the large epicardial arteries.
'''(Cardiac) syndrome X''' is [[Angina pectoris|angina]] (chest pain) associated with objective evidence of myocardial ischemia in the setting of normal epicardial  [[coronary artery|coronary arteries]]. The disorder has been hypothesized to involve the coronary microvasculature rather than the large caliber epicardial arteries.


==Pathophysiology==
==Pathophysiology==
In a large percentage of patients, there is a finding of systemic microvascular abnormalities, causing reduced blood flow in the microvasculature of the cardiac muscles.  While numerous physiological mechanisms have been proposed, none have been proven.
In a large percentage of patients, there is microvascular dysfunction.


==Epidemiology and Demographics==
==Epidemiology and Demographics==
Syndrome X occurs more often in young women. Some studies have found increased risk of other vasospastic disorders in syndrome X patients, such as [[migraine]] and [[Raynaud's phenomenon]].
Syndrome X occurs more often in young women. Some studies have found an increased risk of other vasospastic disorders in syndrome X patients, such as [[migraine]] and [[Raynaud's phenomenon]].


==Natural history, complications, and prognosis==
==Natural history, complications, and prognosis==
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==Other Conditions to Distinguish Syndrome X From==
==Other Conditions to Distinguish Syndrome X From==
Syndrome X is a distinct diagnosis from [[Prinzmetal's angina]] which involves spasm of the main epicardial coronary arteries. Syndrome X involves spasm of the downstream microvasculature.  Syndrome X must also be distinguished from esophageal spasm.
Syndrome X should be distinguished from [[Prinzmetal's angina]], a disorder which involves spasm of the main epicardial coronary arteries. Syndrome X involves dysfunction of the downstream microvasculature.  Syndrome X must also be distinguished from [[esophageal spasm]].


==Diagnosis==
==Diagnosis==
Syndrome X is a diagnosis of exclusion. Typically this will necessitate both a clinical diagnosis, appropriate stress testing, and a coronary angiogram that meet the criteria below:
Syndrome X is a diagnosis of exclusion. The diagnostic criteria are as follows:


*To establish the diagnosis, patients must have evidence of [[myocardial ischemia]] by exercise [[ECG]], [[stress scintigraphy]], or [[stress echocardiography]] in conjunction with anginal chest discomfort.
*There must be evidence of [[myocardial ischemia]]:  Diagnostic studies include an exercise [[ECG]], [[stress scintigraphy]], or [[stress echocardiography]] in conjunction with anginal chest discomfort.
:*Some of these patients have documented reductions in coronary vasodilator reserve presumably due to abnormalities in the [[coronary microcirculation]] and can be shown to have true [[ischemia]] because their [[myocardium]] produces lactate rather than removing it during stress.  While there is no formal definition for Syndrome X, the general consensus is that it entails all of the following:
* [[Angina pectoris|Angina]]: Angina pectoris must be present. The angina pectoris associated with Syndrome X may last longer that the anginal discomfort associated with the fixed epicardial stenoses of atherosclerotic heart disease.
* [[Angina pectoris|Angina]]: This usually does not cause dysfunction on [[echocardiogram]] and can last longer than that of heart disease.
* Abnormal [[Cardiac stress test]]: ST changes are typically similar to those of [[coronary artery disease]] and opposite of those with [[Prinzmetal's angina]]. Myocardial perfusion imaging can be abnormal in 30% of patients.
* Abnormal [[Cardiac stress test]]: ST changes are typically similar to those of [[coronary artery disease]] and opposite of those with [[Prinzmetal's angina]]. Myocardial perfusion imaging can be abnormal in 30% of patients.
* [[Coronary angiogram]]: Normal
* [[Coronary angiogram]]: There is no narrowing of the epicardial arteries.  However, Syndrome X may be associated with a reduction in coronary [[vasodilator reserve]] presumably due to abnormalities in the [[coronary microcirculation]].  During stress, sampling of the [[coronary sinus]] demonstrates the production of [[lactate]] by the [[myocardium]].


==Treatment==
==Treatment==

Revision as of 11:04, 24 July 2011

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

The terms Syndrome X or Metabolic syndrome X may also be referring to metabolic syndrome.

Synonyms and key words: Microvascular angina

Overview

(Cardiac) syndrome X is angina (chest pain) associated with objective evidence of myocardial ischemia in the setting of normal epicardial coronary arteries. The disorder has been hypothesized to involve the coronary microvasculature rather than the large caliber epicardial arteries.

Pathophysiology

In a large percentage of patients, there is microvascular dysfunction.

Epidemiology and Demographics

Syndrome X occurs more often in young women. Some studies have found an increased risk of other vasospastic disorders in syndrome X patients, such as migraine and Raynaud's phenomenon.

Natural history, complications, and prognosis

Syndrome X does not appear to be associated with an excess of major coronary events.

Risk Factors

Female gender and hypertrophy of the myocardium are associated with an excess risk of Syndrome X.

Other Conditions to Distinguish Syndrome X From

Syndrome X should be distinguished from Prinzmetal's angina, a disorder which involves spasm of the main epicardial coronary arteries. Syndrome X involves dysfunction of the downstream microvasculature. Syndrome X must also be distinguished from esophageal spasm.

Diagnosis

Syndrome X is a diagnosis of exclusion. The diagnostic criteria are as follows:

Treatment

The mainstay of treatment in patients with Syndrome X are calcium channel blockers, such as nifedipine and diltiazem. Other therapies include:

ESC Guidelines for investigation in patients with Syndrome X (DO NOT EDIT)[1]

Class I

1. Resting echocardiogram in patients with angina and normal or non-obstructed coronary arteries to assess for presence of ventricular hypertrophy and/or diastolic dysfunction. (Level of Evidence: C)

Class IIb

1. Intracoronary acetylcholine during coronary arteriography, if the arteriogram is visually normal, to assess endothelium-dependent coronary flow reserve, and exclude vasospasm. (Level of Evidence: C)

2. Intracoronary ultrasound, coronary flow reserve, or FFR measurement to exclude missed obstructive lesions, if angiographic appearances are suggestive of a nonobstructive lesion rather than completely normal, and stress imaging techniques identify an extensive area of ischaemia. (Level of Evidence: C)

ESC Guidelines for pharmacological therapy to improve symptoms in patients with Syndrome X (DO NOT EDIT)[1]

Class I

1. Therapy with nitrates, beta blockers, and calcium channel blockers alone or in combination. (Level of Evidence: B)

2. Statin therapy in patients with hyperlipidaemia. (Level of Evidence: B)

3. ACE inhibitors in patients with hypertension. (Level of Evidence: C)

Class IIa

1. Trial of therapy with other anti-anginals including nicorandil and metabolic agents. (Level of Evidence: C)

Class IIb

1. Aminophylline for continued pain, despite Class I measures. (Level of Evidence: C)

2. Imipramine for continued pain, despite Class I measures. (Level of Evidence: C)

References

  1. 1.0 1.1 Fox K, Garcia MA, Ardissino D, Buszman P, Camici PG, Crea F; et al. (2006). "Guidelines on the management of stable angina pectoris: executive summary: The Task Force on the Management of Stable Angina Pectoris of the European Society of Cardiology". Eur Heart J. 27 (11): 1341–81. doi:10.1093/eurheartj/ehl001. PMID 16735367.


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