Echocardiographic Features of Cardiac Involvement in Myotonic Dystrophy 1: Prevalence and Prognostic Value.
Vincenzo RussoAntonio CapolongoRoberta BottinoAndreina CarboneAlberto PalladinoBiagio LiccardoGerardo NigroMichał MarchelPaolo GolinoAntonello D'AndreaPublished in: Journal of clinical medicine (2023)
Myotonic dystrophy type 1 (DM1) is the most common muscular dystrophy in adults. Cardiac involvement is reported in 80% of cases and includes conduction disturbances, arrhythmias, subclinical diastolic and systolic dysfunction in the early stage of the disease; in contrast, severe ventricular systolic dysfunction occurs in the late stage of the disease. Echocardiography is recommended at the time of diagnosis with periodic revaluation in DM1 patients, regardless of the presence or absence of symptoms. Data regarding the echocardiographic findings in DM1 patients are few and conflicting. This narrative review aimed to describe the echocardiographic features of DM1 patients and their prognostic role as predictors of cardiac arrhythmias and sudden death.
Keyphrases
- left ventricular
- ejection fraction
- end stage renal disease
- newly diagnosed
- early stage
- heart failure
- chronic kidney disease
- muscular dystrophy
- pulmonary hypertension
- mitral valve
- peritoneal dialysis
- left atrial
- magnetic resonance
- early onset
- type diabetes
- risk factors
- squamous cell carcinoma
- patient reported outcomes
- lymph node
- congenital heart disease
- patient reported
- glycemic control
- data analysis