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Survival and Clinical Behavior of Hypertrophic Cardiomyopathy in a Latin American Cohort in Contrast to Cohorts from the Developed World

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Background: Hypertrophic cardiomyopathy (HCM) is the most common hereditary heart disease with diverse phenotipyc,genetic expession and clinical presentations. The evolution of patients with HCM in Latin America has not been properly describedbeing the frequency, the long-term prognosis as well as the predominant phenotypic expression still unknown. The aim of thisstudy was to determine the survival rate of HCM patients having different phenotypes in a Mexican cohort of patients. Methods: Clinical and echocardiographic data obtained from 77 Mexican patients with recently diagnosed HCM wereanalyzed. The follow-up was of 12.5 years. Results: 96.1% of patients were in functional class I/II according to the New York Heart Association, 2.6% in class III and 1.3%in class IV. Only 3.9% of them went to surgery for myectomy. During the follow-up, 17 patients (22%) died: 4/9 (44%) had apicalHCM, 5/20 (25%) had obstructive septal asymmetric HCM, 6/35 (17%) had nonobstructive septal asymmetric HCM and 2/3(15%) had concentric HCM. The survival rate was worse for patients with apical HCM, followed by those with obstructive andnonobstructive septal asymmetric HCM and patients showing concentric HCM had the best survival rates. There is significantdifference in survival rates which declined in 65% in a 9 years-period. Log rank test showed significant differences (p < 0.002). Conclusion: The survival rate of patients with HCM was worse in those with apical variety. The majority of patients receivedmedical treatment. The indication for myectomy was below that observed in other international centers.

Abstract

Background: Hypertrophic cardiomyopathy (HCM) is the most common hereditary heart disease with diverse phenotipyc,genetic expession and clinical presentations. The evolution of patients with HCM in Latin America has not been properly describedbeing the frequency, the long-term prognosis as well as the predominant phenotypic expression still unknown. The aim of thisstudy was to determine the survival rate of HCM patients having different phenotypes in a Mexican cohort of patients. Methods: Clinical and echocardiographic data obtained from 77 Mexican patients with recently diagnosed HCM wereanalyzed. The follow-up was of 12.5 years. Results: 96.1% of patients were in functional class I/II according to the New York Heart Association, 2.6% in class III and 1.3%in class IV. Only 3.9% of them went to surgery for myectomy. During the follow-up, 17 patients (22%) died: 4/9 (44%) had apicalHCM, 5/20 (25%) had obstructive septal asymmetric HCM, 6/35 (17%) had nonobstructive septal asymmetric HCM and 2/3(15%) had concentric HCM. The survival rate was worse for patients with apical HCM, followed by those with obstructive andnonobstructive septal asymmetric HCM and patients showing concentric HCM had the best survival rates. There is significantdifference in survival rates which declined in 65% in a 9 years-period. Log rank test showed significant differences (p < 0.002). Conclusion: The survival rate of patients with HCM was worse in those with apical variety. The majority of patients receivedmedical treatment. The indication for myectomy was below that observed in other international centers.

참고문헌 (25)

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