Abstract
Advances in medical care for patients with Duchenne muscular dystrophy (DMD) have resulted in improved survival and an increased prevalence of cardiomyopathy. Serial echocardiographic surveillance is recommended to detect early cardiac dysfunction and initiate medical therapy. Clinical anecdote suggests that echocardiographic quality diminishes over time, impeding accurate assessment of left ventricular systolic function. Furthermore, evidence-based guidelines for the use of cardiac imaging in DMD, including cardiac magnetic resonance imaging (CMR), are limited. The objective of our single-center, retrospective study was to quantify the deterioration in echocardiographic image quality with increasing patient age and identify an age at which CMR should be considered. We retrospectively reviewed and graded the image quality of serial echocardiograms obtained in young patients with DMD. The quality of 16 left ventricular segments in two echocardiographic views was visually graded using a binary scoring system. An endocardial border delineation percentage (EBDP) score was calculated by dividing the number of segments with adequate endocardial delineation in each imaging window by the total number of segments present in that window and multiplying by 100. Linear regression analysis was performed to model the relationship between the EBDP scores and patient age. Fifty-five echocardiograms from 13 patients (mean age 11.6 years, range 3.6-19.9) were systematically reviewed. By 13 years of age, 50% of the echocardiograms were classified as suboptimal with ≥30% of segments inadequately visualized, and by 15 years of age, 78% of studies were suboptimal. Linear regression analysis revealed a negative correlation between patient age and EBDP score (r = -2.49, 95% confidence intervals -4.73, -0.25; p = 0.032), with the score decreasing by 2.5% for each 1 year increase in age. Echocardiographic image quality declines with increasing age in DMD. Alternate imaging modalities may play a role in cases of poor echocardiographic image quality.
Highlights
Duchenne muscular dystrophy (DMD) is a devastating, progressive neuromuscular disease caused by mutations in the gene encoding the cytoskeletal protein dystrophin
We retrospectively reviewed and graded the image quality of serial echo cardiograms obtained in young patients with DMD
Subclinical cardiac dysfunction can begin as early as 6 years of age with clinical heart failure often manifesting after age 10 years and the incidence increasing with age [7]
Summary
Duchenne muscular dystrophy (DMD) is a devastating, progressive neuromuscular disease caused by mutations in the gene encoding the cytoskeletal protein dystrophin. This X-linked recessive disease is the most common inherited muscular dystrophy, affecting 1 in 3,500–6,000 live male births [1,2,3]. Subclinical cardiac dysfunction can begin as early as 6 years of age with clinical heart failure often manifesting after age 10 years and the incidence increasing with age [7]. Advances in medical care for patients with Duchenne muscular dystrophy (DMD) have resulted in improved survival and an increased prevalence of cardiomyo pathy. The objective of our singlecenter, retrospective study was to quantify the deterioration in echocar diographic image quality with increasing patient age and identify an age at which CMR should be considered
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