Abstract
Background Myotonic dystrophy type II (formerly denoted as proximal myotonic myopathy (PROMM)) is an autosomal dominantly inherited disease [1]. Sufferers are afflicted with skeletal muscle (SM) symptoms. Histopathologic changes of the SM include mild fibrosis and fatty degeneration[2]. The multisystemic disorder is also characterized by endocrine and metabolism disorder such as hypercholesterolemia and diabetes mellitus type II as well as cardiac arrhythmias [1]. The aim of this study is to evaluate myocardial structural abnormalities in preserved ejection fraction (EF).
Highlights
Myotonic dystrophy type II (formerly denoted as proximal myotonic myopathy (PROMM)) is an autosomal dominantly inherited disease [1]
All applied sequences were evaluable besides 3 fat/water images due to artifacts
Small myocardial fat deposits were identified in the apical portion of the interventricular septum in 2 patients (Figure 2)
Summary
Myotonic dystrophy type II (formerly denoted as proximal myotonic myopathy (PROMM)) is an autosomal dominantly inherited disease [1]. The aim of this study is to evaluate myocardial structural abnormalities in preserved ejection fraction (EF)
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