Hypertrophic cardiomyopathy epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease. Prevalence rates have been reported between 1:500 (0.2%) and 1:3,000 (0.03%) because of variations in study designs and cohort characteristics including different age groups and ethnicity. According to the CARDIA (Coronary Artery Risk Development in Young Adults) cohort study that used standard echocardiography in 4,111 unrelated people 23 to 35 years of age, HCM prevalence is reported as 1 in 500 persons (0.2%). Nevertheless, lower prevalence has been reported in some European countries such as Germany (0.07%). Patients of all age groups may develop hypertrophic cardiomyopathy. Prevalence increased with advancing age and showed a constant yearly rise but sudden death is more prevalent in young patients, particularly athletes. The case-fatality rate is 6 per 10,000 per year in young people without symptoms of hypertrophic cardiomyopathy but in syptomatic patients a case-fatality rate is 420 and 110 deaths per 10,000 per year in tertiary referral centers and general hospital clinics respectively. Hypertrophic cardiomyopathy affects men and women equally. However, despite more frequent outflow obstruction, women with HCM are underrecognized and referred to centers later than men, often with more advanced heart failure. Greater awareness of HCM in women should lead to earlier diagnosis and treatment, with implications for improved quality of life. HCM is less prevalent in African Americans, but they are more pron to early presentation, developing heart failure, and sudden death is more prevalent due to less awareness and screening in this population.

Epidemiology and Demographics

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease. Prevalence rates have been reported between 1:500 (0.2%) and 1:3,000 (0.03%) because of variations in study designs and cohort characteristics including different age groups and ethnicity. According to the CARDIA (Coronary Artery Risk Development in Young Adults) cohort study that used standard echocardiography in 4,111 unrelated people 23 to 35 years of age, HCM prevalence is reported as 1 in 500 persons (0.2%).[1] Nevertheless, lower prevalence has been reported in some European countries such as Germany (0.07%). [2]A recent analysis of U.S. claims data reported a prevalence of clinically diagnosed HCM in approximately 1:3,000 (0.03%)[3] According to Semsarian et al. "For the past 20 years, most data have supported the occurrence of HCM at about 1 in 500. However, the authors have interrogated a number of relevant advances in cardiovascular medicine, including widespread fee-for-service genetic testing, population genetic studies, and contemporary diagnostic imaging, as well as a greater index of suspicion and recognition for both the clinically expressed disease and the gene-positive– phenotype-negative subset (at risk for developing the disease). Accounting for the potential impact of these initiatives on disease occurrence, the authors have revisited the prevalence of HCM in the general population."[4]

Incidence

  • The incidence/prevalence of Hypertrophic cardiomyopathy is approximately [number range] per 100,000 individuals worldwide.
  • In [year], the incidence/prevalence of Hypertrophic cardiomyopathy was estimated to be [number range] cases per 100,000 individuals worldwide.


Prevalence

  • The prevalence of hypertrophic cardiomyopathy is approximately 200 per 100,000 individuals worldwide. Nevertheless recent studies suggested a higher prevalence. [4]

Case-fatality rate/Mortality rate

  • The case-fatality rate/mortality rate of is different in asymptomatic vs symptomatic patients.
  • The case-fatality rate is 6 per 10,000 per year in young people without symptoms of hypertrophic cardiomyopathy but in syptomatic patients a case-fatality rate is 420 and 110 deaths per 10,000 per year in tertiary referral centers and general hospital clinics respectively.
  • Although it is quite prevalent, hypertrophic cardiomyopathy rarely causes death; the case-fatality rate is about 6 per 10,000 per year in young people without symptoms of hypertrophic cardiomyopathy.[5]
  • Current risk estimates from the study of patients in tertiary referral centers or general hospital clinics are not applicable to asymptomatic people in the general population.[6]

Age

  • Patients of all age groups may develop hypertrophic cardiomyopathy.
  • Patients can be diagnosed at any age, from birth to age 80 and beyond.
  • Children and adolescents with the condition usually come to attention when a family screening is performed after an adult in the family is found to be affected or at the presence of a heart murmur that is evaluated more closely.
  • Approximately 50% of adults with the condition present with symptoms, the average age of diagnosis within the HCMA database is 39 years.
  • Prevalence increased with advancing age and showed a constant yearly rise.
  • But sudden death is more prevalent in young patients, particularly athletes.
  • Hypertrophic cardiomyopathy can cause heart-related sudden death in people of all ages, but the condition most often causes sudden cardiac death in people under the age of 30.

Race

  • For years, HCM was considered an uncommon condition among the African American population. Maron et al, in a multicenter analysis of clinically diagnosed HCM patients noted only 8% of the study population was black. In Movahed et al study, African Americans constitute only 9% of the population. However, in the light of several premature cardiovascular deaths among African American athletes, Maron et al, in an autopsy analysis of young athletes surprisingly noted HCM was 7 times more commonly identified in African Americans for the first time at autopsy compared to when clinically identified in African American population.[7]
  • HCM-related sudden death is more prevalent in black male athletes.[8]Many HCM cases go unrecognized in the African American community, underscoring the need for enhanced clinical recognition of HCM to create the opportunity for preventive measures to be employed in high-risk patients with this complex disease.
  • According to Sheikh et al who studied 425 patients in the UK, there are differences in presenting features including the prevalence of hypertension and an abnormal ECG in black and white patients with HCM. Apical and concentric hypertrophy is more common in black patients and may have implications for appropriate diagnosis, especially in those with concurrent hypertension. Overall freedom from adverse events is similar.[9]
  • Sorensen et al compared 76 blacks for clinical presentation, electrocardiogram, exercise capacity, left ventricular morphology, and hemodynamics by echocardiography to 365 whites. They concluded that "blacks have an HC phenotype characterized by a lower prevalence of the well-recognized echocardiographic features of HC such as the systolic anterior movement of the mitral valve and left ventricular outflow tract obstruction and display worse exercise capacity."[10]
  • The Sarcomeric Human Cardiomyopathy Registry (1989 - 2018)
  • Lauren A. Eberly, M.D., identified 2,467 patients with hypertrophic cardiomyopathy (8.3 percent black; 91.7 percent white). Black patients were younger at the time of diagnosis (mean age, 36.5 versus 41.9 years), had a higher prevalence of New York Heart Association (NYHA) class III or IV heart failure at presentation (22.6 versus 15.8 percent), had lower rates of genetic testing (54.1 versus 62.1 percent) and were less likely to have sarcomeric mutations identified by genetic testing (26.1 versus 40.5 percent) compared with white patients. There were no racial differences noted in implantation of implantable cardioverter-defibrillators, but the invasive septal reduction was less common among black patients (14.6 versus 23 percent). Black patients had less incident atrial fibrillation (35 [17.1 percent] versus 608 [26.9 percent]). There was an association between black race and increased development of NYHA class III or IV heart failure (hazard ratio, 1.45).[11]
  • The United States National Registry
  • Within this large forensic registry of competitive athletes, cardiovascular sudden deaths due to genetic and/or congenital heart diseases were uncommon in females and more common in African Americans/other minorities than in whites. Hypertrophic cardiomyopathy is an under-appreciated cause of sudden death in male minority athletes.[12]

Germany

  • Prevalence of clinically diagnosed HCM in Germany is lower than in systematic population studies based on the echocardiographic diagnosis.


Gender

  • Hypertrophic cardiomyopathy affects men and women equally.
  • Survival was not less favorable in women with HCM.
  • Contemporary treatments including surgical myectomy to reverse heart failure and defibrillators to prevent sudden death, were effective in both sexes contributing to low mortality.
  • However, despite more frequent outflow obstruction, women with HCM are underrecognized and referred to centers later than men, often with more advanced heart failure. Greater awareness of HCM in women should lead to earlier diagnosis and treatment, with implications for improved quality of life.[13][14]

Region

  • The majority of Hypertrophic cardiomyopathy cases are reported in [geographical region].
  • Hypertrophic cardiomyopathy is a common/rare disease that tends to affect [patient population 1] and [patient population 2].

Developed Countries

Developing Countries

References

  1. Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE (1995). "Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults". Circulation. 92 (4): 785–9. doi:10.1161/01.cir.92.4.785. PMID 7641357.
  2. Husser D, Ueberham L, Jacob J, Heuer D, Riedel-Heller S, Walker J; et al. (2018). "Prevalence of clinically apparent hypertrophic cardiomyopathy in Germany-An analysis of over 5 million patients". PLoS One. 13 (5): e0196612. doi:10.1371/journal.pone.0196612. PMC 5933727. PMID 29723226.
  3. Maron MS, Hellawell JL, Lucove JC, Farzaneh-Far R, Olivotto I (2016). "Occurrence of Clinically Diagnosed Hypertrophic Cardiomyopathy in the United States". Am J Cardiol. 117 (10): 1651–1654. doi:10.1016/j.amjcard.2016.02.044. PMID 27006153.
  4. 4.0 4.1 Semsarian C, Ingles J, Maron MS, Maron BJ (2015). "New perspectives on the prevalence of hypertrophic cardiomyopathy". J Am Coll Cardiol. 65 (12): 1249–1254. doi:10.1016/j.jacc.2015.01.019. PMID 25814232.
  5. Wald DS, Law M, Morris JK (2004). "Mortality from hypertrophic cardiomyopathy in England and Wales: clinical and screening implications". Int J Cardiol. 97 (3): 479–84. doi:10.1016/j.ijcard.2003.11.014. PMID 15561336.
  6. Maron BJ, Rowin EJ, Casey SA, Maron MS (2016). "How Hypertrophic Cardiomyopathy Became a Contemporary Treatable Genetic Disease With Low Mortality: Shaped by 50 Years of Clinical Research and Practice". JAMA Cardiol. 1 (1): 98–105. doi:10.1001/jamacardio.2015.0354. PMID 27437663.
  7. Movahed MR, Strootman D, Bates S, Sattur S (2010). "Prevalence of suspected hypertrophic cardiomyopathy or left ventricular hypertrophy based on race and gender in teenagers using screening echocardiography". Cardiovasc Ultrasound. 8: 54. doi:10.1186/1476-7120-8-54. PMC 3019148. PMID 21143986.
  8. Maron BJ, Carney KP, Lever HM, Lewis JF, Barac I, Casey SA; et al. (2003). "Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy". J Am Coll Cardiol. 41 (6): 974–80. doi:10.1016/s0735-1097(02)02976-5. PMID 12651044.
  9. Sheikh N, Papadakis M, Panoulas VF, Prakash K, Millar L, Adami P; et al. (2016). "Comparison of hypertrophic cardiomyopathy in Afro-Caribbean versus white patients in the UK". Heart. 102 (22): 1797–1804. doi:10.1136/heartjnl-2016-309843. PMID 27679836.
  10. Sorensen LL, Pinheiro A, Dimaano VL, Pozios I, Nowbar A, Liu H; et al. (2016). "Comparison of Clinical Features in Blacks Versus Whites With Hypertrophic Cardiomyopathy". Am J Cardiol. 117 (11): 1815–20. doi:10.1016/j.amjcard.2016.03.017. PMID 27084053.
  11. Eberly LA, Day SM, Ashley EA, Jacoby DL, Jefferies JL, Colan SD; et al. (2019). "Association of Race With Disease Expression and Clinical Outcomes Among Patients With Hypertrophic Cardiomyopathy". JAMA Cardiol. doi:10.1001/jamacardio.2019.4638. PMID 31799990.
  12. Maron BJ, Haas TS, Ahluwalia A, Murphy CJ, Garberich RF (2016). "Demographics and Epidemiology of Sudden Deaths in Young Competitive Athletes: From the United States National Registry". Am J Med. 129 (11): 1170–1177. doi:10.1016/j.amjmed.2016.02.031. PMID 27039955.
  13. Rowin EJ, Maron MS, Wells S, Patel PP, Koethe BC, Maron BJ (2019). "Impact of Sex on Clinical Course and Survival in the Contemporary Treatment Era for Hypertrophic Cardiomyopathy". J Am Heart Assoc. 8 (21): e012041. doi:10.1161/JAHA.119.012041. PMC 6898820 Check |pmc= value (help). PMID 31663408.
  14. Maron BJ (2018). "Clinical Course and Management of Hypertrophic Cardiomyopathy". N Engl J Med. 379 (7): 655–668. doi:10.1056/NEJMra1710575. PMID 30110588.


References


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