Restrictive cardiomyopathy. Presentation of seven cases
ResumenRestrictive cardiomyopathy is a disease characterized by diastolic ventricular failure with increase in telediastolic pressure and systolic function preserved.
Materials and methods: a retrospective study of patients with diagnosis of restrictive cardiomyopathy. We conducted an analysis of demographic data, clinical manifestation, and studies of patients diagnosed in the last 15 years at Instituto Nacional de Pediatría.
Results: all the patients included had clinical signs of heart failure manifested mainly by medium dyspnea on effort in school-age patients and dyspnea on eating in infants, as well as polypnea and diaphoresis. The most prominent signs were hepatomegaly, ascites, and gallop rhythm.
Cardiomegaly due to right auricular dilation was the most common radiological finding. The most common electrocardiographic findings were dilation of both auricles, ST segment depression and negative T waves. The echocardiogram showed biauricular dilation and restrictive pattern in all cases.
Conclusions: our patients had findings similar to those described in the specialized literature. Echocardiogram remains the best study for diagnosis, and the use of functional measurements like Doppler tissue imaging may help detect early diastolic failure. In Mexico, heart transplant remains unfeasible, with 100% mortality.
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