Cardiac manifestations and clinical management of X-linked Emery-Dreifuss muscular dystrophy: a case series.

ABSTRACT:

Background

Heart disease is an under-recognized cause of morbidity and mortality in patients with Emery-Dreifuss muscular dystrophy (EDMD). Arrhythmias and conduction delays are highly prevalent and given the rarity of this disease the patient care process remains poorly defined.

Case summary

This study closely followed four adult patients from the Neuromuscular Multidisciplinary Clinic (Alberta, Canada) that presented with X-linked recessive EDMD. Patients were assessed and managed on a case-by-case basis. Clinical status and cardiac function were assessed through clinical history, physical examination, and investigations (12-lead electrocardiogram, 24 hour Holter monitor, transthoracic echocardiogram, and plasma biomarkers). Conduction disease, requiring permanent pacemake

SUBMITTER: Kashyap N 

PROVIDER: S-EPMC9879840 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature