Classification of the cardiomyopathies: a position statement from the european society of cardiology working group on myocardial and pericardial diseases

Elliott, Perry; Andersson, Bert; Arbustini, Eloisa; Bilińska, Zofia T.; Cecchi, Fabiola; Charron, Philippe; Dubourg, Olivier; Kühl, Uwe; Maisch, Bernhard; McKenna, William J.; Monserrat, Lorenzo; Pankuweit, Sabine; Rapezzi, Claudio; Seferović, Petar; Tavazzi, Luigi; Keren, Andre

doi:10.1093/eurheartj/ehm342

Cited by 2,406 publications

(1,831 citation statements)

References 19 publications

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“…Records older than 1997 from Kungälv Hospital and 1999 from Lidköping Hospital were not identified. A diagnosis was defined as definite if the ESC diagnostic criteria from 20088 were fulfilled. Besides, in cases with present coronary artery disease and DCM with underlying causes such as tachycardia, alcohol and drug abuse, and myocarditis, or where coronary angiography revealed minimal signs of coronary artery disease not sufficient to cause a global reduction of systolic cardiac function and, finally, in cases with present hypertension but well treated and insufficient to cause global impact on the cardiac function, the diagnosis was defined as definite.…”

Section: Resultsmentioning

confidence: 99%

“…chemotherapy), and radiation therapy to the chest. In addition, type of cardiomyopathy and diagnostic criteria for the specific type of cardiomyopathy according to the diagnostic criteria and definition of the ESC from 20088 were recorded. Details from echocardiography, coronary angiography, and cardiac magnetic resonance imaging, when performed, were collected.…”

Section: Methodsmentioning

confidence: 99%

“…A diagnosis was defined as definite if the ESC diagnostic criteria from 20088 were fulfilled. If not all criteria were fulfilled, such as in patients with HCM and underlying untreated or poorly controlled hypertension or DCM and a prior myocardial infarction with secondary HF, the diagnoses were defined as miscoded.…”

Section: Methodsmentioning

confidence: 99%

“…Cardiomyopathy includes a heterogeneous group of diseases 8. More detailed and specific definitions have evolved over the years 8, 9, 10, 11.…”

Section: Introductionmentioning

confidence: 99%

“…With increasing knowledge of pathogenesis, aetiology, and genetic defects, cardiomyopathy was defined in 1995 as ‘heart muscle disease associated with cardiac dysfunction’ 14. A new classification by the European Society of Cardiology (ESC)8 was introduced in 2008: ‘A myocardial disease in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.’ This includes hereditary forms as well as those induced by viral infection, inflammatory disease, medication, alcohol and/or drug abuse, tachycardia, and dilated cardiomyopathy (DCM) in pregnancies 8. A recent update of the ESC guidelines from 2014 of the diagnosis of hypertrophic cardiomyopathy (HCM) is not taken into account in this study.…”

Section: Introductionmentioning

confidence: 99%

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High validity of cardiomyopathy diagnoses in western Sweden (1989–2009)

et al. 2017

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AimHospital discharges with a diagnosis of cardiomyopathy have more than doubled in Sweden since 1987. We validated the cardiomyopathy diagnoses over this time period to investigate that the increase was real and not a result of improved recognition of the diagnosis and better diagnostic methods.Methods and resultsEvery fifth year from 1989 to 2009, records for all patients with a cardiomyopathy diagnosis were identified by searching the local registers in three hospitals in Västra Götaland, Sweden. The diagnoses were validated according to criteria defined by the European Society of Cardiology from 2008. The population comprised 611 cases with cardiomyopathy diagnoses [mean age 58.9 (SD 15.5) years, 68.2% male], divided into three major groups: dilated, hypertrophic, and other cardiomyopathies. Hypertrophic cardiomyopathy and hypertrophic obstructive cardiomyopathy were analysed as a group. Cardiomyopathies for which there were few cases, such as restrictive, arrhythmogenic right ventricular, left ventricular non‐compaction, takotsubo, and peripartum cardiomyopathies, were analysed together and defined as ‘other cardiomyopathies’. Relevant co‐morbidities were registered. The use of echocardiography was 99.7%, of which 94.6% was complete echocardiography reports. The accuracy rates of the diagnoses dilated cardiomyopathy, hypertrophic cardiomyopathy, and other cardiomyopathies were 85.5%, 87.5%, and 100%, respectively, with no differences between the three hospitals or years studied; nor did the prevalence of co‐morbidities differ.ConclusionsThe accuracy rate of the cardiomyopathy diagnoses from in‐hospital records from >600 patients in western Sweden during a 20 year period was 86.6%, with no significant trend over time, strengthening epidemiological findings that this is likely due to an actual increase in cardiomyopathy diagnoses rather than changes in coding practices. The use of echocardiography was high, and there was no significant difference in co‐morbidities during the study period. The accuracy rate of the cardiomyopathy diagnoses during the 20 year period was high. The use of diagnostic tools did not increase under the study period, and once cardiomyopathy diagnoses were suspected, echocardiography was performed in almost all cases. In this study, the occurrence of cardiomyopathy was increasing over time without significant increase of co‐morbidity, supporting that an actual increase of cardiomyopathy has occurred.

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Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

“…Cardiomyopathy includes a heterogeneous group of diseases 8. More detailed and specific definitions have evolved over the years 8, 9, 10, 11.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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High validity of cardiomyopathy diagnoses in western Sweden (1989–2009)

et al. 2017

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Association Between Hypertensive Disorders of Pregnancy and Later Risk of Cardiomyopathy

Behrens

Basit

Lykke

et al. 2016

JAMA

109

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IMPORTANCE Women with hypertensive disorders of pregnancy, preeclampsia in particular, have an increased risk of cardiomyopathy during the peripartum period. Whether hypertensive disorders of pregnancy are also associated with cardiomyopathy later in life is unknown.OBJECTIVE To determine whether hypertensive disorders of pregnancy are associated with cardiomyopathy beyond the peripartum period. DESIGN, SETTING, AND PARTICIPANTSNationwide register-based cohort study using Cox regression to compare rates of cardiomyopathy in women with and without a history of hypertensive disorders of pregnancy in a cohort of 1 075 763 women with at least 1 pregnancy ending in live birth or stillbirth in Denmark, 1978, with follow-up through December 31, 2012.EXPOSURES A hypertensive disorder of pregnancy (severe or moderate preeclampsia or gestational hypertension) registered in the National Patient Register.MAIN OUTCOMES AND MEASURES Cardiomyopathy more than 5 months after delivery (outside the peripartum period) up to 34 years 7 months.RESULT The women in the primary cohort had 2 067 633 eligible pregnancies during the study period, 76 108 of which were complicated by a hypertensive disorder of pregnancy. During follow-up, 1577 women (mean age, 48.5 years at cardiomyopathy diagnosis; 2.6% with multiple pregnancies) developed cardiomyopathy. Compared with women with normotensive pregnancies (18 211 603 person-years of follow-up; n = 1408 cardiomyopathy events, 7.7/100 000 person-years [95% CI, 7.3-8.2]), women with a history of hypertensive disorders of pregnancy had significantly increased rates of cardiomyopathy (in 173 062 person-years of follow-up among women with severe preeclampsia, n = 27 cardiomyopathy events; 15.6/100 000 person-years [95% CI, 10.7-22.7]; adjusted hazard ratio [HR], 2.20 [95% CI,; in 697 447 person-years of follow-up among women with moderate preeclampsia, n = 102 cardiomyopathy events; 14.6/100 000 person-years [95% CI, 12.0-17.8]; adjusted HR, 1.89 [95% CI, 1.55-2.23]; in 213 197 person-years of follow-up among women with gestational hypertension, n = 40 cardiomyopathy events; 17.3/100 000 person-years [95% CI, 12.7-23.6]; adjusted HR, 2.06 [95% CI, 1.50-2.82]). These increases persisted more than 5 years after the latest pregnancy. Mediation analyses suggested that only about 50% of the association was an indirect association through postpregnancy chronic hypertension. In this cohort, 11% of all cardiomyopathy events occurred in women with a history of hypertensive disorders of pregnancy. CONCLUSIONS AND RELEVANCEWomen with a history of hypertensive disorders of pregnancy, compared with women without such a history, had a small but statistically significant increased risk of cardiomyopathy more than 5 months after delivery. Further research is necessary to understand whether there is a causal mechanism behind this association.

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Association of Titin Variations With Late-Onset Dilated Cardiomyopathy

et al. 2022

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ilated cardiomyopathy (DCM) is a nonischemic, nontoxic, noninfectious, or nonvalvular heart muscle disease characterized by left ventricular or biventricular systolic dysfunction. 1,2 Typical onset of the disease is in the fourth or fifth decade of life and it affects predominantly male individuals with a male-to-female ratio of 3:1. 2 Although a specific cause might be unknown in a non-negligible number of patients, to date, more than one-third of familial forms and approximately 25% of sporadic cases have positive genetic testing for pathogenic (P) or likely pathogenic (LP) variants. [3][4][5][6] Recent evidence highlights the importance of specific genotypes in determining the prognosis of patients with DCM, either alone or in combination with environmental factors. [7][8][9][10][11] Contemporary analyses highlight that DCM is increasingly diagnosed in older patients 12 and a late-onset (ie, age >60 years) form of DCM could differ from more typical-onset DCM presentations. 13 However, the genetic background and the prognostic impact of genetic characterization among patients with DCM presenting who are older than 60 years are largely unknown.The aim of the present study was to investigate the genetic background and the natural history of a large international cohort of individuals with DCM presenting in individuals older than 60 years to assess the importance of genetic testing in those patients. Methods Study PopulationFor the present study, we included all consecutive patients with DCM older than 60 years at the time of diagnosis (ie, lateonset DCM), with available genetic testing analysis, from 7 international tertiary centers worldwide: Cardiovascular IMPORTANCE Dilated cardiomyopathy (DCM) is frequently caused by genetic factors. Studies identifying deleterious rare variants have predominantly focused on early-onset cases, and little is known about the genetic underpinnings of the growing numbers of patients with DCM who are diagnosed when they are older than 60 years (ie, late-onset DCM).OBJECTIVE To investigate the prevalence, type, and prognostic impact of disease-associated rare variants in patients with late-onset DCM. DESIGN, SETTING, AND PARTICIPANTSA population of patients with late-onset DCM who had undergone genetic testing in 7 international tertiary referral centers worldwide were enrolled from March 1990 to August 2020. A positive genotype was defined as the presence of pathogenic or likely pathogenic (P/LP) variants. MAIN OUTCOMES AND MEASURESThe study outcome was all-cause mortality.RESULTS A total of 184 patients older than 60 years (103 female [56%]; mean [SD] age, 67 [6] years; mean [SD] left ventricular ejection fraction, 32% [10%]) were studied. Sixty-six patients (36%) were carriers of a P/LP variant. Titin-truncating variants were the most prevalent (present in 46 [25%] of the total population and accounting for 46 [69%] of all genotype-positive patients). During a median (interquartile range) follow-up of 42 (10-115) months, 23 patients (13%) died; 17 (25%) of these were carriers o...

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Classification of the cardiomyopathies: a position statement from the european society of cardiology working group on myocardial and pericardial diseases

Cited by 2,406 publications

References 19 publications

High validity of cardiomyopathy diagnoses in western Sweden (1989–2009)

High validity of cardiomyopathy diagnoses in western Sweden (1989–2009)

Association Between Hypertensive Disorders of Pregnancy and Later Risk of Cardiomyopathy

Association of Titin Variations With Late-Onset Dilated Cardiomyopathy

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