Global InformationHypertrophic cardiomyopathy information
| Hypertrophic cardiomyopathy | |
|---|---|
| Other names | Asymmetric septal hypertrophy; idiopathic hypertrophic subaortic stenosis;[1] hypertrophic obstructive cardiomyopathy (HOCM) |
 | |
| Specialty | Cardiology |
| Symptoms | Feeling tired, leg swelling, shortness of breath, chest pain, fainting[2] |
| Complications | Heart failure, irregular heartbeat, sudden cardiac death[3][4] |
| Causes | Genetics, Fabry disease, Friedreich's ataxia, certain medications[5][6] |
| Diagnostic method | Electrocardiogram, echocardiogram, stress testing, genetic testing[7] |
| Differential diagnosis | Hypertensive heart disease, aortic stenosis, athlete's heart[5] |
| Treatment | Medications, implantable cardiac defibrillator, surgery[7] |
| Medication | Beta blockers, verapamil, disopyramide[8] |
| Prognosis | Less than 1% per year risk of death (with treatment)[9] |
| Frequency | Up to 1 in 200 people[8] |
Hypertrophic cardiomyopathy (HCM, or HOCM when obstructive) is a condition in which muscle tissues of the heart become thickened without an obvious cause.[8] The parts of the heart most commonly affected are the interventricular septum and the ventricles.[10] This results in the heart being less able to pump blood effectively and also may cause electrical conduction problems.[3] Specifically, within the bundle branches that conduct impulses through the interventricular septum and into the Purkinje fibers, as these are responsible for the depolarization of contractile cells of both ventricles.[11]
People who have HCM may have a range of symptoms. People may be asymptomatic, or may have fatigue, leg swelling, and shortness of breath.[2] It may also result in chest pain or fainting.[2] Symptoms may be worse when the person is dehydrated.[10] Complications may include heart failure, an irregular heartbeat, and sudden cardiac death.[3][4]
HCM is most commonly inherited[6] in an autosomal dominant pattern.[10] It is often due to mutations in certain genes involved with making heart muscle proteins.[6] Other inherited causes of left ventricular hypertrophy may include Fabry disease, Friedreich's ataxia, and certain medications such as tacrolimus.[5] Other considerations for causes of enlarged heart are athlete's heart and hypertension (high blood pressure).[10] Making the diagnosis of HCM often involves a family history or pedigree, an electrocardiogram, echocardiogram, and stress testing.[7] Genetic testing may also be done.[7] HCM can be distinguished from other inherited causes of cardiomyopathy by its autosomal dominant pattern, whereas Fabry disease is X-linked, and Friedreich's ataxia is inherited in an autosomal recessive pattern.[10]
Treatment may depend on symptoms and other risk factors. Medications may include the use of beta blockers, verapamil or disopyramide.[8] An implantable cardiac defibrillator may be recommended in those with certain types of irregular heartbeat.[7] Surgery, in the form of a septal myectomy or heart transplant, may be done in those who do not improve with other measures.[7] With treatment, the risk of death from the disease is less than one percent per year.[9]
HCM affects up to one in 200 people.[8] People of all ages may be affected.[12] The first modern description of the disease was by Donald Teare in 1958.[13][14]
- ^ "Other Names for Cardiomyopathy". NHLBI. June 22, 2016. Archived from the original on 28 July 2016. Retrieved 31 August 2016.
- ^ a b c "What Are the Signs and Symptoms of Cardiomyopathy?". NHLBI. 22 June 2016. Archived from the original on 28 July 2016. Retrieved 10 November 2017.
- ^ a b c "What Is Cardiomyopathy?". NHLBI. 22 June 2016. Archived from the original on 10 November 2017. Retrieved 10 November 2017.
- ^ a b Barsheshet A, Brenyo A, Moss AJ, Goldenberg I (October 2011). "Genetics of sudden cardiac death". Current Cardiology Reports. 13 (5): 364–376. doi:10.1007/s11886-011-0209-y. PMID 21789574. S2CID 25887172.
- ^ a b c Ferri FF (2017). Ferri's Clinical Advisor 2018 E-Book: 5 Books in 1. Elsevier Health Sciences. p. 246. ISBN 9780323529570. Archived from the original on 2017-11-10. Retrieved 2017-11-10.
- ^ a b c "What Causes Cardiomyopathy?". NHLBI. 22 June 2016. Archived from the original on 5 October 2017. Retrieved 10 November 2017.
- ^ a b c d e f Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, et al. (December 2011). "2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines". The Journal of Thoracic and Cardiovascular Surgery. 142 (6): 1303–1338. doi:10.1016/j.jtcvs.2011.10.019. PMID 22093712.
- ^ a b c d e Cui H, Schaff HV (2020). "80. Hypertrophic cardiomyopathy". In Raja SG (ed.). Cardiac Surgery: A Complete Guide. Switzerland: Springer. pp. 735–748. ISBN 978-3-030-24176-6. Archived from the original on 2023-01-10. Retrieved 2022-10-20.
- ^ a b Maron BJ, Ommen SR, Semsarian C, Spirito P, Olivotto I, Maron MS (July 2014). "Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine". Journal of the American College of Cardiology. 64 (1): 83–99. doi:10.1016/j.jacc.2014.05.003. PMID 24998133.
- ^ a b c d e Basit H, Brito D, Sharma S (2020). "Hypertrophic Cardiomyopathy". StatPearls. Treasure Island (FL): StatPearls Publishing. PMID 28613539. Archived from the original on 2021-04-06. Retrieved 2020-09-20.
- ^ Bernardini A, Crotti L, Olivotto I, Cecchi F (May 2023). "Diagnostic and prognostic electrocardiographic features in patients with hypertrophic cardiomyopathy". European Heart Journal Supplements. 25 (Suppl C): C173–C178. doi:10.1093/eurheartjsupp/suad074. PMC 10132576. PMID 37125268.
- ^ "Types of Cardiomyopathy". NHLBI. 22 June 2016. Archived from the original on 4 October 2017. Retrieved 10 November 2017.
- ^ Teare D (January 1958). "Asymmetrical hypertrophy of the heart in young adults". British Heart Journal. 20 (1): 1–8. doi:10.1136/hrt.20.1.1. PMC 492780. PMID 13499764.
- ^ McKenna WJ, Sen-Chowdhry S (December 2008). "From Teare to the present day: a fifty year odyssey in hypertrophic cardiomyopathy, a paradigm for the logic of the discovery process". Revista Espanola de Cardiologia. 61 (12): 1239–1244. doi:10.1016/S1885-5857(09)60050-5. PMID 19080961. Archived from the original on 2017-11-11. Retrieved 2017-02-06.