Rare Cardiology News

Disease Profile

Prinzmetal’s variant angina

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

no.svg

Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

no.svg

X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

no.svg

X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

no.svg

Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

no.svg

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

no.svg

Not applicable

no.svg

Other names (AKA)

Prinzmetal angina; Variant angina; Angina inversa;

Summary

Prinzmetal's variant angina (PVA) is characterized by recurrent episodes of chest pain (angina) that usually occur when a person is at rest, between midnight and early morning. "Typical" angina, by contrast, is often triggered by physical exertion or emotional stress. Episodes of PVA can be very painful, and may last from several minutes to thirty minutes.[1][2][3] In some cases the pain may spread from the chest to the head, shoulder, or arm.[3] The pain associated with PVA is caused by a spasm in the arteries that supply blood to the heart muscle (coronary arteries). This results in an obstruction of blood flow.[1][2] In some people, persistent spasms increase the risk for serious complications such as a life-threatening arrhythmia or heart attack.[2]

PVA most commonly occurs in people who smoke and people who have high cholesterol or high blood pressure.[3] In many cases it occurs for unknown reasons in otherwise healthy people. In some cases it may be triggered by alcohol withdrawal, stress, exposure to cold, certain medications, or use of stimulants such as cocaine.[1][3]

The diagnosis of PVA involves findings on an electrocardiogram, evidence of the spasms on angiogram, and relief of sudden symptoms with medicines called nitrates.[2]

Treatment during an episode to relieve pain and shorten its duration involves sublingual nitroglycerin (a nitrate).[2][3] Treatment to reduce the frequency of episodes and possibly reduce the risk of serious complications involves calcium channel blockers or long-acting nitrates.[2] For people with PVA who smoke, quitting smoking can lead to a significant decrease in the frequency of episodes.[2] While most people with PVA do not experience serious complications, it is a chronic condition that needs to be monitored over time.[1]

Symptoms

The main symptom of Prinzmetal's variant angina is recurring episodes of chest pain (angina) that usually occur when a person is at rest, during bedtime hours (around midnight to 8am). Some people report feeling "discomfort" rather than "pain." In some people, pain may spread to the neck, jaw, shoulder, or arm. Other sensations during episodes may include tightness or pressure in the chest, "heart burn," nausea, sweating, dizziness, and palpitations. Episodes tend to last around 5 to 15 minutes (longer in some cases), and tend to be similar to each other.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • The American Heart Association has an information page on Prinzmetal's variant angina. The American Heart Association is the nation’s oldest and largest voluntary organization dedicated to fighting heart disease and stroke.
    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
    • The National Heart, Lung, and Blood Institute (NHLBI) has information on this topic. NHLBI is part of the National Institutes of Health and supports research, training, and education for the prevention and treatment of heart, lung, and blood diseases.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Prinzmetal's variant angina. Click on the link to view a sample search on this topic.

        References

        1. Prinzmetal's or Prinzmetal Angina, Variant Angina and Angina Inversa. American Heart Association. July, 2015; https://www.heart.org/HEARTORG/Conditions/HeartAttack/DiagnosingaHeartAttack/Prinzmetals-or-Prinzmetal-Angina-Variant-Angina-and-Angina-Inversa_UCM_435674_Article.jsp#.Wk-54lWnFD8.
        2. Pinto DS, Beltrame JF, Crea F. Vasospastic angina. UpToDate. Waltham, MA: UpToDate; February 6, 2017; https://www.uptodate.com/contents/vasospastic-angina.
        3. Chen MA. Coronary artery spasm. MedlinePlus. February 24, 2016; https://www.nlm.nih.gov/medlineplus/ency/article/000159.htm.

        Rare Cardiology News