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PREVALENCE OF DIFFERENT ETIOLOGIES IN DILATED CARDIOMYOPATHY
A35.E342 JACC March 9, 2010 Volume 55, issue 10A
CARDIAC FUNCTION AND HEART FAILURE PREVALENCE OF DIFFERENT ETIOLOGIES IN DILATED CARDIOMYOPATHY ACC Poster Contributions Georgia World Congress Center, Hall B5 Tuesday, March 16, 2010, 9:30 a.m.-10:30 a.m.
Session Title: Myocarditis and dilated cardiomyopathy Abstract Category: Cardiomyopathies/Myocarditis/Pericardial Disease Presentation Number: 1231-45 Authors: Sabine Pankuweit, Anette Richter, Volker Ruppert, Götz Gelbrich, Bernhard Maisch, Competence Net work heart failure, Dept. of Cardiology, Philipps-University Marburg, Marburg, Germany Indroduction: The phenotype DCM is assumed to be the endstage of a multifactorial etiopathogenetic pathophysiology, which includes a yet not defined part of patients with inflammatory/familial cardiomyopathy. Precipitating factors include enhanced autoimmunity, predisposition for viral infections and environmental factors. Methods: Within the German Heart Failure Network we started our project Ikarius with the inclusion of patients with dilated cardiomyopathy (ejection fraction 56mm) and in addition the inclusion of all relevant data regarding a possible familial, infectious or autoimmune etiology of the disease. Further aims of the project were the search for a genetic link or predispisition to autoimmune diseases in patients with familial / inflammatory DCM. Results: After screening of more than 1600 patients we have included 274 index patients (211 male / 63 female) in total with an age of 51±13 years, an ejection fraction (EF) of 29%±8% and a left ventricular enddiastolic volume (LVEDD)of 68mm±8mm. Blood samples and endomyocardial biopsies from all patients, a pedigree including data for a familial history for heart diseases or autoimmune diseases are available in all cases. In total, we identified 57 families (21%) with a minimum of two affected family members. We were able to identify in 23,5% of the index patients an inflammatory DCM and in 24.5% of index patients a viral heart disease. In more than 50% of all patients’ families a history of autoimmune diseases was detected. Genetic analysis of the patients resulted so far in identification of a lamin A/C mutation in two patients, a new phopholamban mutation and a N-cadherin mutation in one patient each. The CD 45 polymophismus 77C>G was detected in 5 patients. An association of HLA-DR polymorphisms with a better prognosis was shown in a subgroup of patients with DCMi. After 1-year-follow-up of all patients EF (39%±13%) and a LVEDD (65mm±9mm) improved in all patients. Conclusion: Epidemiological data with a special focus regarding an familial, infectious and inflammatory etiology of the disease are now available for this large patient cohort. Follow-up data will be available to identify new prognostic biomarker in patients with DCM.
CARDIAC FUNCTION AND HEART FAILURE PREVALENCE OF DIFFERENT ETIOLOGIES IN DILATED CARDIOMYOPATHY ACC Poster Contributions Georgia World Congress Center, Hall B5 Tuesday, March 16, 2010, 9:30 a.m.-10:30 a.m.
Session Title: Myocarditis and dilated cardiomyopathy Abstract Category: Cardiomyopathies/Myocarditis/Pericardial Disease Presentation Number: 1231-45 Authors: Sabine Pankuweit, Anette Richter, Volker Ruppert, Götz Gelbrich, Bernhard Maisch, Competence Net work heart failure, Dept. of Cardiology, Philipps-University Marburg, Marburg, Germany Indroduction: The phenotype DCM is assumed to be the endstage of a multifactorial etiopathogenetic pathophysiology, which includes a yet not defined part of patients with inflammatory/familial cardiomyopathy. Precipitating factors include enhanced autoimmunity, predisposition for viral infections and environmental factors. Methods: Within the German Heart Failure Network we started our project Ikarius with the inclusion of patients with dilated cardiomyopathy (ejection fraction 56mm) and in addition the inclusion of all relevant data regarding a possible familial, infectious or autoimmune etiology of the disease. Further aims of the project were the search for a genetic link or predispisition to autoimmune diseases in patients with familial / inflammatory DCM. Results: After screening of more than 1600 patients we have included 274 index patients (211 male / 63 female) in total with an age of 51±13 years, an ejection fraction (EF) of 29%±8% and a left ventricular enddiastolic volume (LVEDD)of 68mm±8mm. Blood samples and endomyocardial biopsies from all patients, a pedigree including data for a familial history for heart diseases or autoimmune diseases are available in all cases. In total, we identified 57 families (21%) with a minimum of two affected family members. We were able to identify in 23,5% of the index patients an inflammatory DCM and in 24.5% of index patients a viral heart disease. In more than 50% of all patients’ families a history of autoimmune diseases was detected. Genetic analysis of the patients resulted so far in identification of a lamin A/C mutation in two patients, a new phopholamban mutation and a N-cadherin mutation in one patient each. The CD 45 polymophismus 77C>G was detected in 5 patients. An association of HLA-DR polymorphisms with a better prognosis was shown in a subgroup of patients with DCMi. After 1-year-follow-up of all patients EF (39%±13%) and a LVEDD (65mm±9mm) improved in all patients. Conclusion: Epidemiological data with a special focus regarding an familial, infectious and inflammatory etiology of the disease are now available for this large patient cohort. Follow-up data will be available to identify new prognostic biomarker in patients with DCM.