Assessing Arrhythmic Risk in Adult Patients with Duchenne Muscular Dystrophy
Abstract
Background: Cardiac involvement is common in Duchenne Muscular Dystrophy (DMD). By the end of the second decade, cardiomyopathy is observed in >30% of men and can lead to heart failure, heart rhythm abnormalities and sudden cardiac death. Many men with DMD die suddenly, however, it remains unclear whether they die of arrhythmias. Objective and Study Design: We propose to study the natural history of the rhythm abnormalities in DMD by implanting miniaturized cardiac monitors (ILRs) in 75 DMD patients at risk for developing potentially life-threatening heart rhythm disorders (Specific Aim 1). Additionally, we will use computer simulations in personalized 3D heart models generated from cardiac MRI images obtained during clinical visits, to examine whether scar tissue found in DMD hearts will allow for a better identification of patients at highest risk for arrhythmias who may benefit from implantation of a cardioverter-defibrillator (Specific Aim 2). Major Findings: We have completed IRB/HRPO approval at this time and are actively enrolling patients. An alternative protocol which includes prolonged ambulatory rhythm monitoring with Ziopatches and Event monitors in addition to ILRs is proposed to help to meet enrollment goals during the COVID-19 pandemic.
Document Details
- Document Type
- Technical Report
- Publication Date
- Oct 01, 2020
- Accession Number
- AD1118347
Entities
People
- Andreas S. Barth
Organizations
- Johns Hopkins University