Boys With Duchenne Muscular Dystrophy Have Diastolic Dysfunction Based on CMR.

IF 6.5 1区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS
Circulation: Cardiovascular Imaging Pub Date : 2024-12-01 Epub Date: 2024-12-04 DOI:10.1161/CIRCIMAGING.124.017287
Joseph R Starnes, Jeffrey G Weiner, Kristen George-Durrett, Kimberly Crum, Christopher C Henderson, M Jay Campbell, Katheryn Gambetta, Kan N Hor, Nazia Husain, Jennifer S Li, Frank J Raucci, Brian D Soriano, Christopher F Spurney, Larry W Markham, Jonathan H Soslow
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引用次数: 0

Abstract

Background: Cardiomyopathy is the leading cause of death in boys with Duchenne muscular dystrophy (DMD). While cardiac magnetic resonance (CMR) is routinely used to assess fibrosis and left ventricular (LV) ejection fraction, CMR measures of LV filling and ejection in DMD have not been reported.

Methods: Patients with DMD (n=179) and healthy controls (n=96) were prospectively enrolled and underwent CMR. The DMD cohort was followed clinically at multiple institutions, and clinical data were recorded. Standard volumes and functions were calculated, and LV filling and ejection curves were measured from baseline CMR. Multivariable linear regressions were used to compare ventricular filling and ejection measures between groups, adjusting for baseline differences. Cox regressions were used to evaluate the relationship between diastolic function measures and mortality in the DMD cohort.

Results: Patients with DMD had significantly smaller stature and ventricular volumes than healthy control patients (P<0.001). They had lower baseline LV ejection fraction (P<0.001), though most had normal systolic function. When adjusted for age, sex, heart rate, body surface area, and LV end-diastolic volume, patients with DMD had slower peak filling rates (P<0.001) and peak ejection rates (P<0.001), as well as slower time to peak ventricular ejection rate (P=0.011). When adjusted for heart rate, a lower peak ventricular ejection rate (P=0.007) and peak filling rate (P=0.033), normalized to LV end-diastolic volume, were associated with mortality in patients with DMD.

Conclusions: Patients with DMD have significantly different baseline CMR filling and ejection indices compared with controls. Some filling indices are associated with mortality and may be useful prognostic measures. Further research is needed in larger cohorts to determine the prognostic value of these differences.

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来源期刊
CiteScore
6.30
自引率
2.70%
发文量
225
审稿时长
6-12 weeks
期刊介绍: Circulation: Cardiovascular Imaging, an American Heart Association journal, publishes high-quality, patient-centric articles focusing on observational studies, clinical trials, and advances in applied (translational) research. The journal features innovative, multimodality approaches to the diagnosis and risk stratification of cardiovascular disease. Modalities covered include echocardiography, cardiac computed tomography, cardiac magnetic resonance imaging and spectroscopy, magnetic resonance angiography, cardiac positron emission tomography, noninvasive assessment of vascular and endothelial function, radionuclide imaging, molecular imaging, and others. Article types considered by Circulation: Cardiovascular Imaging include Original Research, Research Letters, Advances in Cardiovascular Imaging, Clinical Implications of Molecular Imaging Research, How to Use Imaging, Translating Novel Imaging Technologies into Clinical Applications, and Cardiovascular Images.
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