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Persistent myocardial atrophy despite LV reverse remodeling in Duchenne cardiomyopathy treated by LVAD

Nicoletta Cantarutti1,2, Rachele Adorisio2,3, Anwar Baban1,2

  • 1Pediatric Cardiology and Cardiac Arrhythmias/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Pediatric Transplantation
|October 26, 2020
PubMed

Insights

Left Ventricular Assist Devices (LVADs) improve heart function in Duchenne cardiomyopathy patients. However, cardiac atrophy persists despite ventricular unloading and medical therapy.

Area of Science:

  • Cardiology
  • Biomedical Engineering
  • Genetics

Background:

  • Duchenne cardiomyopathy (DCM) is a primary cause of mortality in Duchenne muscular dystrophy patients.
  • Advanced heart failure (HF) in these patients may benefit from durable therapeutic options like Left Ventricular Assist Devices (LVADs).

Purpose of the Study:

  • To assess left ventricular (LV) remodeling in Duchenne patients following LVAD implantation and chronic medical management.
  • Evaluate the impact of LVADs on cardiac structure and function in end-stage DCM.

Main Methods:

  • Retrospective analysis of demographic and echocardiographic data from 8 Duchenne patients with LVADs.
  • Measurements collected pre-implantation, at 1 month, and 1 year post-implantation.
  • Patients received maximal medical therapy.

Main Results:

  • Heart rate significantly decreased from 110 ± 19 bpm to 82 ± 2 bpm one year post-LVAD (P = .002).
  • Significant reductions in LV volumes (LVEDD, LVESD, EDV, ESV) and a notable increase in ejection fraction (EF) were observed (P < .05).
  • Right Ventricular Taper (RWT) remained unchanged, indicating a persistent eccentric remodeling pattern.

Conclusions:

  • LVADs improve LV function and reduce volumes in Duchenne cardiomyopathy, likely due to ventricular unloading.
  • Despite functional improvements, cardiac atrophy persists in Duchenne cardiomyopathy patients on chronic therapy and LVAD support.

Keywords:
LVADcardiomyopathyduchennemyocardial atrophy

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