Survival and clinical behavior of hypertrophic cardiomyopathy in a latin american cohort in contrast to cohorts from the developed world.

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 described being the frequency, the long-term prognosis as well as the predominant phenotypic expression still unknown. The aim of this study 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 were analyzed. 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 apical HCM, 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 and nonobstructive septal asymmetric HCM and patients showing concentric HCM had the best survival rates. There is significant difference 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 received medical treatment. The indication for myectomy was below that observed in other international centers.

[Uhl's anomaly. A case report with anatomoechocardiographic correlation]. / Anomalía de Uhl. Informe de un caso con correlación anátomo-ecocardiográfica.

UNLABELLED: Uhl's anomaly is an uncommon malformation of the right ventricle, which is characterized by the absence of the myocardium in the right ventricular free wall, while the tricuspid and pulmonary valves are morphologically normal. The aim of this work was to present a clinical case of a patient with Uhl's anomaly, to describe the echocardiographic findings and to perform an anatomoechocardiographic correlation with a corresponding specimen. This is a case report of a 33 year old man with suspicion of atrial septal defect, who was in functional class II of the NYHA and presented signs of right ventricular failure. The echocardiogram showed dilation of the right cavities, the free wall of the right ventricle was thin and smooth, and the tricuspid and pulmonary valves were normal, which prompted the diagnosis of Uhl's anomaly. Also, an anatomoechocardiographic correlation was done with an equivalent specimen of the Embryology Department from the Instituto Nacional de Cardiologia Ignacio Chavez's collection. CONCLUSIONS: Uhl's anomaly is an extremely rare entity. The clinical findings and echocardiography are the main tools for its diagnosis. The anatomoechocardiographic correlation was precise and allowed a better understanding of this anomaly."