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DES Gene Risk Stratification (Myofibrillar Myopathy) Calculator

DES Gene Risk Stratification (proposed, literature-informed): Explanation and Clinical Context This calculator implements a pragmatic, composite risk tool for individuals with DES gene variants (desmin-related myofibrillar myopathy) that combines genotype (pathogenic/likely pathogenic vs VUS/negative), presence of clinical cardiomyopathy, left ventricular ejection fraction (LVEF), arrhythmic burden (non-sustained VT,

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Dystrophin-Associated Cardiomyopathy Risk Index

Explanation — (imaging-based) This tool implements an imaging-based composite risk index developed to stratify patients with dystrophin-associated cardiomyopathy (principally Duchenne muscular dystrophy, DMD) by combining the timing and severity of left ventricular dysfunction with the timing and severity of myocardial fibrosis detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR). The ind