II. Pathophysiology

  1. Focal arterial spasm of a major Coronary Artery
    1. Typically in the absence of high grade Coronary Artery stenosis
  2. Mechanisms
    1. Vascular smooth muscle hyperreactivity to Vasoconstrictors
    2. Increased vagal tone
    3. Increased hyperreactivity to sympathetic stimulation
    4. Endothelial dysfunction
  3. Comorbid arterial stenosis
    1. Most arterial lesions are eccentric (75%)
      1. Considerable vasoactivity in most vessels
    2. Vasoconstriction occurs at lesion with Exercise
      1. Paradoxical: vasodilatation would be expected
      2. Occurs due to passive vasodilation down stream
  4. May be associated with other vasospastic conditions
    1. Raynaud's Phenomenon
    2. Migraine Headache

III. Epidemiology

  1. Age
    1. More common under age 50 years old
  2. Race
    1. More common in Japanese patients than in Caucasian
  3. Incidence
    1. Coronary Artery Vasospasm found in 4% of Coronary Artery angiograms in U.S.
      1. United States however has among lowest Incidence of Coronary Artery Vasospasm

IV. Risk Factors

V. Symptoms

  1. Recurrent episodic Chest Pain consistent with Angina
    1. Anterior chest discomfort or pressure
    2. Radiates to neck, jaw, arms as with typical Angina
  2. Triggers
    1. Typically occurs at rest without provocation (e.g. without Exercise, not modified by position)
    2. May be triggered by Hyperventilation, Cocaine, hypersympathetic states (e.g. peak Exercise)
  3. Timing
    1. Early morning hours after midnight
  4. Duration
    1. Episodes last 5 to 15 minutes
  5. Characteristics
    1. Gradual onset and resolution
  6. Associated symptoms
    1. Typical Angina related symptoms (Nausea, diaphoresis, Dyspnea)

VI. Labs

  1. Obtain typical labs for chest Pain Evaluation
  2. Serial Troponin
  3. Magnesium

VII. Diagnostics

  1. Electrocardiogram
    1. Normal between episodes
    2. ST Segment elevation during vasospastic episode
  2. Holter Monitor
    1. May detect episodes of periodic ST Elevation
  3. Stress Testing
    1. Evaluate for severe fixed cardiovascular disease
  4. Coronary Angiography
    1. Consider in most patients with suspected Variant Angina

IX. Management: Prevention of vasospasm

  1. First-Line measures
    1. Tobacco Cessation
    2. Calcium Channel Blockers
      1. Diltiazem 240 to 360 mg orally daily
      2. Nifedipine
      3. Verapamil
    3. Other measures as indicated
      1. Statins (e.g. Atorvastatin, Simvastatin, Fluvastatin)
      2. Replace Magnesium in Magnesium deficiency
  2. Second-Line measures
    1. Long-Acting Nitroglycerin
      1. Isosorbide Mononitrate 30-60 mg once daily
      2. Indicated if refractory to Calcium Channel Blockers
      3. Risk of nitrate tolerance (Calcium Channel Blockers are preferred)
  3. Third-Line Measures
    1. Percutaneous Coronary Intervention (PCI)
      1. May be indicated in moderate fixed coronary obstruction
  4. Avoid provocative agents
    1. Limit Aspirin to low dose
      1. Higher dose may predispose to vasospasm
      2. Aspirin 81 mg should be prescribed in atherosclerotic cardiovascular disease
    2. Avoid non-selective Beta Blockers (e.g. Propranolol)
      1. Risk of vasospasm
    3. Avoid other agents associated with vasospasm
      1. Triptans (e.g. Sumatriptan)
      2. 5-Fluorouracil

X. Complications

  1. Myocardial Infarction
    1. Risk of thrombus forming in response to vasospasm
  2. Arrhythmia
    1. Ventricular Tachycardia (left Coronary Artery)
    2. Heart Block (right Coronary Artery)
    3. May present with Syncope to Cardiac Arrest
      1. Consider Implantable Cardioverter-Defibrillator (ICD)

XI. Resources

XII. References

  1. Pinto, Beltrame, Crea in Saperia (2015) Variant Angina, UpToDate, Wolters Kluwer (accessed 6/13/2015)

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Ontology: Angina Pectoris, Variant (C0002963)

Definition (NCI) (syn. Prinzmetal's angina or coronary artery vasospasm). It usually occurs spontaneously, and unlike typical angina, it nearly always occurs when a person is at rest and does not require physical exertion. It frequently is associated with transient ST-segment elevation.(NIH Roadmap Cardiovascular Data Standards Working Group)
Definition (NCI_CDISC) A variant form of angina pectoris caused by coronary artery vasospasm, usually occurring spontaneously and frequently associated with ST segment elevation.
Definition (NCI) A variant form of angina pectoris caused by coronary artery vasospasm, usually occurring spontaneously and frequently associated with ST segment elevation.
Definition (MSH) A clinical syndrome characterized by the development of CHEST PAIN at rest with concomitant transient ST segment elevation in the ELECTROCARDIOGRAM, but with preserved exercise capacity.
Concepts Disease or Syndrome (T047)
MSH D000788
ICD9 413.1
ICD10 I20.1
SnomedCT 87343002
English Angina Pectoris, Variant, Prinzmetal's Angina, Prinzmetals Angina, Angina, Prinzmetal's, Prinzmetal Angina, ANGINA VARIANT <PRINZMETAL>, Variant Angina Pectoris, Variant Angina, variant angina pectoris, Prinzmental angina, Prinzmetal's angina (diagnosis), Vasospastic angina, ANGINA VARIANT PRINZMETAL, Angina Pectoris, Variant [Disease/Finding], vasospastic angina, prinzmetals angina, prinzmetal variant angina, angina prinzmetal, prinzmetal angina, prinzmetal's angina, angina variant, variant angina, Angina, Prinzmetal, VARIANT ANGINA, Prinzmetal angina, Prinzmetal's angina, Variant angina pectoris, Variant angina, Coronary artery spasm angina, Prinzmetal angina (disorder), Prinzmetal; angina, angina pectoris; variant, variant; angina
Italian Angina di Prinzmetal, Angina variante, Angina vasospastica, Angina pectoris variante
Dutch variante angina, vasospastische angina, Prinzmetal; angina, angina pectoris; variant, variant; angina, Angina pectoris, variant, Prinzmetal-angina, Variant angina pectoris
Portuguese Variante de angina, Angina vasospástica, Angina de prinzmetal, Angina de Prinzmetal, Angina Pectoris Variante
Spanish Angina variante, Angina vasoespástica, Angina de Pecho Variable, angina de Prinzmetal (trastorno), angina de Prinzmetal, angina de pecho variante, Angina de Prinzmetal, Angina Pectoris Variable
Japanese プリンツメタル狭心症, イケイキョウシンショウ, プリンツメタルキョウシンショウ, ケッカンレンシュクセイキョウシンショウ, 血管攣縮性狭心症, Prinzmetal型狭心症, 狭心症-異型, Prinzmetal狭心症, 冠動脈攣縮性狭心症, 冠攣縮性狭心症, 異型狭心症, プリンツメタル型狭心症, 冠れん縮性狭心症, 冠血管攣縮性狭心症, 狭心症-冠れん縮性, 狭心症-冠攣縮性, 狭心症-冠血管攣縮性
Swedish Variantangina
Czech Prinzmetalova angina pectoris, angina pectoris inverzní, Variantní angina, Prinzmetalova angina, Vazospastická angina, variantní angina pectoris, angina pectoris variabilis, vazospastická angina pectoris
Finnish Käänteisangina
French Angor spastique de Prinzmetal, Angor de Prinzmetal, Angor type Prinzmetal, Angor vasospastique
Russian VARIANTNAIA STENOKARDIIA, STENOKARDIIA VARIANTNAIA, PRINTSMETALA STENOKARDIIA, ВАРИАНТНАЯ СТЕНОКАРДИЯ, ПРИНЦМЕТАЛА СТЕНОКАРДИЯ, СТЕНОКАРДИЯ ВАРИАНТНАЯ
German vasospastische Angina, Prinzmetal - Angina, Angina pectoris, Variant-, Prinzmetal-Angina
Polish Dławica odmienna, Postać odmienna dusznicy bolesnej, Dławica Prinzmetala, Dusznica bolesna Prinzmetala
Hungarian Varians angina, Vasospastikus angina, Prinzmetal-angina
Norwegian Prinzmetals angina pectoris, Variantangina

Ontology: Coronary Artery Vasospasm (C0010073)

Definition (MSH) Spasm of the large- or medium-sized coronary arteries.
Concepts Disease or Syndrome (T047)
MSH D003329
SnomedCT 23687008
English Artery Vasospasm, Coronary, Artery Vasospasms, Coronary, Coronary Artery Vasospasms, Coronary Vasospasm, Coronary Vasospasms, Vasospasm, Coronary, Vasospasm, Coronary Artery, Vasospasms, Coronary, Vasospasms, Coronary Artery, CORONARY ARTERY SPASM, CORONARY SPASM, Coronary vasospasm, coronary artery spasm, coronary artery spasm (diagnosis), Arteriospasm coronary, Spasm coronary artery, Coronary vascular spasm, Coronary Vasospasm [Disease/Finding], coronary vasospasms, Spasm;artery;coronary, artery coronary spasms, coronary vasospasm, artery coronary spasm, coronary spasms, spasm coronary artery, vasospasm coronary, coronary artery vasospasm, coronary spasm, arteriospasm coronary, coronaries spasm, Coronary artery spasm, Coronary spasm, Coronary artery spasm (disorder), coronary; spasm, a.coronaria; spasm, spasm; coronary artery, spasm; coronary, Coronary Artery Vasospasm
French SPASME CORONAIRE, Spasme de l'artère coronaire, Spasme artériel coronaire, Angiospasme coronarien, Artériospasme coronaire, Spasme coronaire, Angiospasme coronaire, Spasme d'une artère coronaire, Vasospasme coronaire, Spasme coronarien, Vasospasme coronarien
Italian Arteriospasmo coronarico, Spasmo di arteria coronarica, Spasmo coronarico, Spasmo arterioso coronarico, Spasmo vascolare coronarico, Vasospasmo coronarico
Dutch kransslagaderkramp, coronair vasospasme, a.coronaria; spasme, coronair; spasme, spasme; arteria coronaria, spasme; coronair, kransslagaderspasme, Coronair vasospasme, Coronariaspasme, Spasme, coronaria-, Vasospasme, coronair
German Herzkranzgefaessverengung, Spasmus Koronararterie, KORONARARTERIENSPASMUS, KORONARSPASMUS, Koronararterienspasmus, Koronarspasmus, Koronargefäßspasmus
Portuguese Espasmo vascular coronário, Vasospasmo coronário, Espasmo das artérias coronárias, Espamo coronário, ESPASMO CORONARIO, ESPASMO DAS ARTERIAS CORONARIAS, Arteriospasmo coronário, Vasoespasmo Coronário, Vasoespasmo da Artéria Coronária
Spanish Espasmo de la arteria coronaria, Espasmo vascular coronario, Vasoespasmo coronario, Espasmo coronario, CORONARIO ESPASMO, ESPASMO CORONARIO, arterioespasmo coronario, espasmo arterial coronario, espasmo de arteria coronaria (trastorno), espasmo de arteria coronaria, Arteriospasmo coronario, Vasoespasmo Coronario, Vasoespasmo de la Arteria Coronaria
Japanese 冠血管攣縮, カンドウミャクレンシュク, カンケッカンレンシュク, 冠れん縮, 冠血管けいれん, 冠攣縮, 冠状動脈痙攣, 冠状血管痙攣, 冠スパスム, 冠スパズム, 冠動脈Spasm, 冠動脈スパスム, 冠動脈スパズム, 冠動脈攣縮, 冠状動脈けいれん, 冠状動脈スパズム, 冠状血管けいれん
Swedish Kranskärlskramp
Czech koronární vasospasmus, Koronární spasmus, Koronární cévní spasmus, Spasmus koronární arterie, Koronární arteriospasmus, Koronární vazospasmus
Finnish Sepelvaltimospasmi
Russian KORONARNYKH ARTERII SPAZM, KORONARNYKH SOSUDOV SPAZM, КОРОНАРНЫХ АРТЕРИЙ СПАЗМ, КОРОНАРНЫХ СОСУДОВ СПАЗМ
Polish Skurcz naczyń wieńcowych
Hungarian Coronaria spasmus, Coronaria vascularis görcs, Arteria coronaria görcse, Coronariaspasmus, Coronaria vasospasmus, Arteria coronaria görcs
Norwegian Koronarspasme, Koronar vasospasme