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연세대학교 의과대학 Yonsei Medical Journal Yonsei Medical Journal 제60권 제9호
발행연도
2019.1
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816 - 823 (8page)

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Purpose: The present study aimed to investigate the prevalence, characteristics, and clinical significance of concomitant specificcardiomyopathies in subjects with bicuspid aortic valves (BAVs). Materials and Methods: A total of 1186 adults with BAV (850 males, mean age 56±14 years) at a single tertiary center were comprehensivelyreviewed. Left ventricular non-compaction, hypertrophic cardiomyopathy, and idiopathic dilated cardiomyopathywere confirmed when patients fulfilled current clinical and echocardiographic criteria. Clinical and echocardiographic characteristics,including comorbidities, heart failure presentation, BAV morphology, function, and aorta phenotypes, in BAV subjectswith or without specific cardiomyopathies were compared. Results: Overall, 67 subjects (5.6%) had concomitant cardiomyopathies: 40 (3.4%) patients with left ventricular non-compaction,17 (1.4%) with hypertrophic cardiomyopathy, and 10 (0.8%) with dilated cardiomyopathy. BAV subjects with hypertrophic cardiomyopathyhad higher prevalences of diabetes mellitus and heart failure with preserved ejection fraction, and tended to havetype 0 phenotype, while BAV subjects with dilated cardiomyopathy showed higher prevalences of chronic kidney disease andheart failure with reduced ejection fraction. BAV subjects with left ventricular non-compaction were significantly younger andpredominantly male, and had greater BAV dysfunction and a higher prevalence of normal aorta shape. In multiple regressionanalysis, cardiomyopathy was independently associated with heart failure (odds ratio 2.795, 95% confidential interval 1.603–4.873, p<0.001) after controlling for confounding factors. Conclusion: Concomitant cardiomyopathies were observed in 5.6% of subjects with BAV. A few different clinical and echocardiographiccharacteristics were found. The presence of cardiomyopathy was independently associated with heart failure.

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