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. 2021 Jul 28;18(4):211–224. doi: 10.1007/s11897-021-00521-2

Table 1.

Overview of muscular dystrophies and the associated cardiac abnormalities

General Cardiologic manifestations
Clinical CMR
Disease Pathology Inheritance Age of onset of primary manifestation Cardiac involvement (%) Potential phenotype Common/typical conduction disturbances/arrhythmias LGE Fat/Water
Duchenne muscular dystrophy Dystrophinopathy X-linked recessive 3–5 years Up to 90 [2] DCM Sinus tachycardia, ventricular tachycardias + +
Becker muscular dystrophy Dystrophinopathy X-linked recessive 6–20 (variable) 60–70 [3] DCM AV nodal and bundle branch blocks + No data
Emery-Dreifuss muscular dystrophy Nuclear envelopathies, laminopathies X-linked recessive, autosomal dominant or recessive 10–20 years 50–90 (highly variable) [4] DCM, HCM, LVNC, biatrial dilation Bradycardias (AV blocks), tachycardias (SVTs) Rare, if present associated with tachycardias Rare
Limb girdle muscular dystrophy Calpainopathies, sarcoglycanopathies, etc. Autosomal dominant or recessive 5-20 25–90 [5] DCM, HCM SVTs, VTs + +
Myofibrillar myopathy Desminopathy, etc. Autosomal dominant (other forms exist) 2–50 (highly variable) 15–30 [6] DCM, HCM, LVNC Complete AV block No data No data
Facioscapulohumeral muscular dystrophy DUX/SMCHD1 gene Autosomal dominant (other forms exist) 0–20 5–15 [7] DCM (rare) RBBB, SVTs + +
Myotonic dystrophy type 1 DMPK gene Autosomal dominant 0–50 (highly variable) 60–80 [8] DCM, HCM AV Blocks, QTc/QRS prolongation, VES, Afib, Aflatter, RBBB/LBBB, VTs + No data
Myotonic dystrophy type 2 ZNF-9 gene Autosomal dominant 30–50 Up to 25 [9] DCM, HCM Afib + +