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        Prevalence of Congenital Heart Disease and Pulmonary Hypertension in Down’s Syndrome: An Echocardiographic Study

        Nilda Espinola-Zavaleta,,María Elena Soto,Angel Romero-Gonzalez,Lidia del Carmen Gómez-Puente,Luis Muñoz-Castellanos,Aasha S. Gopal,Candace Keirns,Eulo Lupi-Herrera 한국심초음파학회 2015 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.23 No.2

        Background: Down’s syndrome (DS) is a genetic anomaly, which undergoes increased morbidity and mortality when associatedwith congenital heart disease (CHD). The aims of the study were to determine the prevalence of CHD and pulmonary hypertension(PH) in DS. Methods: One hundred twenty-seven patients with DS living in Mexico City were evaluated by physical exam, electrocardiogramand echocardiogram. Results: CHD was found in 40%. In 80% (n = 102) PH was present [systolic pulmonary artery pressure (SPAP) of 47 ± 19mm Hg and mean pulmonary artery pressure (MPAP) of 32 ± 11 mm Hg]. Patients with CHD and PH were classified ashaving 1) no shunt (n = 18) with SPAP of 37 ± 9 mm Hg and MPAP of 25 ± 6 mm Hg and 2) with shunt (n = 26) with PASPof 57 ± 29 mm Hg and MPAP of 38 ± 19 mm Hg (p ≤ 0.001). In those without CHD or with CHD without shunt (n = 76), SPAPwas 37 ± 19 mm Hg and the MPAP 25 ± 6 mm Hg. The prevalence of PH in DS was 5.9% at one year and 15% at 10 years. Theodds ratio of PH in DS with CHD was 7.3 vs. 3 without CHD. Conclusion: DS has a high prevalence of CHD and PH. PH prevalence increases when it is associated with CHD. Thepathophysiology of PH in DS without CHD should be studied in the near future. Echocardiography is an indispensible tool forevaluation of DS.

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

        Nilda Espinola-Zavaleta,Antonio Vega,Diego Martínez Basto,Ana Cecilia Alcantar-Fernández,Veronica Guarner Lans,María Elena Soto 한국심초음파학회 2015 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.23 No.1

        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.

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