The prevalence of myocardial fibrosis on MRI attributable to SSc is 45%, is more frequent and severe in dcSSc patients, is associated with lower LVEF and affects mainly basal LV walls. Microvascular damage in SSc is common and is associated with elevated ultrasensitive CRP levels. Cardiac damage due to SSc is not associated with coronary artery disease.

Echocardiography makes it possible to identify and evaluate complications associated with elevated morbidity and mortality in patients with congenital heart disease and IE.

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We report a 36-year-old woman with hypertrophic cardiomyopathy with asymmetric septal hypertrophy without outflow tract obstruction associated with an ostium secundum-type atrial septal defect with significant hemodynamic repercussion. Diagnosis was established with transesophageal echocardiography. This is the second case of this rare association reported in the literature and the first evaluated by transesophageal echocardiography.