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Background: Dilated cardiomyopathy (DCM) has a prevalence of 36.5 cases per 100,000 persons in USA. It represents a major health burden and also remains the primary indication for heart transplantation. Almost half to the cases are idiopathic and 20 - 50% of these patients are found to have monogenic associations. Familial DCM is usually asymptomatic and the patients are frequently present at the late stage. In Thailand, no certain prevalence of Familial DCM has been studied. Hence, this work is expected to provide the further benefit of genetic study in this particular condition.
Methods: During January 2011 – December 2012, the data of all clinical cases presented with heart failure and underwent coronary angiography were recruited. Only the patients with normal coronary angiography were included. Family history, comorbidity diseases, current and previous medications, body mass index, laboratory data including kidney function, thyroid function test, and echocardiography report were collected. The etiologies of DCM were characterized into hereditary and known acquired causes. The diagnoses of idiopathic and familial DCM were performed relying on the clinical practice guideline.
Results: Total 178 patients presented with heart failure were recruited for the study and 72 (40.45%) met the criteria for either definite or suspected DCM (39 and 33 patients, repectively). Diabetes mellitus, arrhythmias and chronic kidney diseases were the most common etiologies of impaired systolic function without significant coronary artery disease whereas hyperthyroidism and morbid obesity occurred less frequently. Twelve from 72 patients were defined as unknown acquired etiologies (idiopathic DCM). Among these 12 patients, only 1 patient had history of familial dilated cardiomyopathy (8.33%). However, these patients from 12 patients (25%) were characterized as having positive family histories, but the clinical data in their affected family member did not meet the complete criteria for DCM.
Conclusions: Approximately 17% of our patients affected by DCM are classified as idiopathic. This finding suggests the possibility to have underlying monogenic mechanism. Hence, this is a pilot study for further gene and mutation study in idiopathic and familial DCM in Thai population.
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