Four-dimensional echocardiography and left ventricular systolic strain measured via two-dimensional speckle-tracking for Danon disease: a case series.

ABSTRACT:

Background

Danon disease is an X-linked multisystemic disorder characterized by skeletal myopathy, cardiomyopathy, and intellectual disability.

Case summary

Herein, we describe two patients affected by Danon disease from the same family, a father (Patient 1) and his daughter (Patient 2). In Patient 1, a short PR interval with pre-excitation was evident. In Patient 2, over a 24-h period 2369 atrial premature beats and rare isolated ventricular ectopics were detected. Both patients exhibited left ventricular hypertrophy with non-compaction myocardium, and the left ventricular ejection fraction was impaired in Patient 1 and normal in Patient 2. In Patient 2, the total left ventricular strain value was reduced, and layer-specific strain revealed that subepicardial strain impaire

SUBMITTER: Changsheng M 

PROVIDER: S-EPMC8684807 | biostudies-literature | 2021 Dec

REPOSITORIES: biostudies-literature