Cardiac asynchrony in Duchenne muscular dystrophy

Abdallah Fayssoil1, Olivier Nardi, David Orlikowski

  • 1Réanimation médicale, hôpital Raymond Poincaré (AP-HP), Université de Versailles SQY, 104 boulevard Raymond Poincaré, 92380, Garches, France, fayssoil2000@yahoo.fr.

Insights

Duchenne muscular dystrophy (DMD) patients often have left ventricular dysfunction. Systolic ventricular asynchrony is frequent, especially in those with reduced ejection fraction (EF).

Area of Science:

  • Cardiology
  • Genetics
  • Neuromuscular Disorders

Background:

  • Duchenne muscular dystrophy (DMD) is an inherited disorder affecting muscle, frequently leading to heart failure.
  • The prevalence and characteristics of systolic dyssynchrony in DMD patients are not well-documented.
  • Left ventricular (LV) dysfunction is a known complication, but its association with asynchrony requires further investigation.

Purpose of the Study:

  • To evaluate the prevalence of left ventricular dysfunction in adult DMD patients.
  • To assess the incidence of systolic asynchrony, both inter-ventricular and intra-ventricular, in this population.
  • To explore the relationship between LV ejection fraction (EF) and the presence of systolic asynchrony.

Main Methods:

  • Electrocardiography and echocardiography were performed on 48 adult DMD patients.
  • Systolic asynchrony was assessed using echocardiography-Doppler and tissue Doppler imaging.
  • Patients' ages ranged from 20 to 37 years.

Main Results:

  • Left ventricular ejection fraction (EF) varied widely, with a median of 43%.
  • Inter-ventricular asynchrony was observed in 11.9% of patients with EF < 35% and 2.6% with EF > 35%.
  • Intra-ventricular asynchrony was present in 6% of patients with EF < 35%.

Conclusions:

  • DMD patients exhibit a high prevalence of left ventricular dysfunction.
  • Systolic ventricular asynchrony appears to be a common finding in DMD, particularly in those with significantly reduced LV function (EF < 35%).
  • These findings highlight the importance of cardiac monitoring in DMD management.

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