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)
- Maron BJ, "Hypertrophic cardiomyopathy : an important global disease", Am J Med, 116, pp.63~65 (2004)
- Kaludercic N, "Genes, geography and geometry:the “critical mass” in hypertrophic cardiomyopathy", J Mol Diagn, 11, pp.12~16 (2009)
- Gersh BJ, "American College of Cardiology Foundation", American Heart Association Task Force on Practice Guidelines. 2011 ACCF, American Heart Association Task Force on Practice Guidelines. Developed in collaboration with the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons, pp.J Am Coll Cardiol, 2011[DOI]
- McKenna W, "Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features", Am J Cardiol, 47, pp.532~538 (1981)
- Maron BJ, "Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults", Circulation, 92, pp.785~789 (1995)
- Kaple RK, "Mitral valve abnormalities in hypertrophic cardiomyopathy: echocardiographic features and surgical outcomes", Ann Thorac Surg (2008)
- Nagueh SF, "Tissue Doppler imaging predicts the development of hypertrophic cardiomyopathy in subjects with subclinical disease", Circulation, 108, pp.395~398 (2003)
- He XW, "Evaluation of left ventricular function, rotation, twist and untwist in patients with hypertrophic cardiomyopathy", Exp Clin Cardiol, 18, pp.47~49 (2013)
- Kimura A, "Mutations in the cardiac troponin I gene associated with hypertrophic cardiomyopathy", Nat Genet, 16, pp.379~382 (1997)
- Bonne G, "Familial hypertrophic cardiomyopathy : from mutations to functional defects", Circ Res, 83, pp.580~593 (1998)
- Geisterfer-Lowrance AA, "A molecular basis for familial hypertrophic cardiomyopathy : a beta cardiac myosin heavy chain gene missense mutation", Cell, 62, pp.999~1006 (1990)
- McKenna WJ, "Sudden death (V). Identification and treatment of patients with hypertrophic cardiomyopathy at risk of sudden death", Rev Esp Cardiol, 53, pp.123~130 (2000)
- Wigle ED, "Hypertrophic cardiomyopathy. Clinical spectrum and treatment", Circulation, 92, pp.1680~1692 (1995)
- Maron BJ, "Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients", Am J Cardiol, 48, pp.418~428 (1981)
- Eriksson MJ, "Long-term outcome in patients with apical hypertrophic cardiomyopathy", J Am Coll Cardiol, 39, pp.638~645 (2002)
- Binder J, "Apical hypertrophic cardiomyopathy : prevalence and correlates of apical outpouching", J Am Soc Echocardiogr, 24, pp.775~781 (2011)
- Klarich KW, "Risk of death in long-term follow-up of patients with apical hypertrophic cardiomyopathy", Am J Cardiol, 111, pp.1784~1791 (2013)
- Woo A, "Mutations of the beta myosin heavy chain gene in hypertrophic cardiomyopathy : critical functional sites determine prognosis", Heart, 89, pp.1179~1185 (2003)
- Ommen SR, "Hypertrophic cardiomyopathy", Curr Probl Cardiol, 29, pp.239~291 (2004)
- Braunwald E, "Contemporary evaluation and management of hypertrophic cardiomyopathy", Circulation, 106, pp.1312~1316 (2002)
- Elliott PM, "Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy", Eur Heart J, 27, pp.1933~1941 (2006)
- Maron MS, "Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy", N Engl J Med, 348, pp.295~303 (2003)
- O’Mahony C, "A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy(HCM risk-SCD)", Eur Heart J, 35, pp.2010~2020 (2014)
- Elliott PM, "Sudden death in hypertrophic cardiomyopathy : identification of high risk patients", J Am Coll Cardiol, 36, pp.2212~2218 (2000)
- Arad M, "Gene mutations in apical hypertrophic cardiomyopathy", Circulation, 112, pp.2805~2811 (2005)
