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Scientific Articles

Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy

key information

source: Pediatric cardiology

year: 2016

authors: Mehmood M, Ambach SA, Taylor MD, Jefferies JL, Raman SV, Taylor RJ, Sawani H, Mathew J, Mazur W, Hor KN, Al-Khalidi HR


The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing within 1 month at a single center (2013-2015) were enrolled. Comparisons of RV ejection fraction (RVEF) and end-diastolic volume index (RVEDVI) were made across categories of percent forced vital capacity (FVC%), and relationships were assessed. Mean age was 15.5 ± 3.5 years. Spirometry and CMR were performed within 3.9 ± 4.1 days. Median FVC% was 92.0 % (67.5-116.5 %). Twenty-three (40 %) patients had abnormal FVC% (<80 %) of which 13 (57 %) had mild (FVC% 60-79 %), 6 (26 %) had moderate (FVC% 40-59 %), and 4 (17 %) had severe (FVC <40 %) reductions. Mean RVEF was 58.3 ± 3.7 %. Patients with abnormal FVC% were older and had lower RVEF and RVEDVI. Both RVEF and RVEDVI were significantly associated with FVC% (r = 0.31, p = 0.02 and r = 0.39, p = 0.003, respectively). In a large DMD cohort, RVEF and RVEDVI were related to FVC%. Worsening respiratory status may guide monitoring of cardiac function in these patients.

organization: Allegheny General Hospital, USA; University of Cincinnati, USA; Cincinnati Children's Hospital, USA; Ohio State University, USA; The Queen Elizabeth Hospital Birmingham, UK; The Christ Hospital Health Network, Cincinnati, USA; Nationwide Children's Hospital, Columbus, USA; Duke University School of Medicine, USA

DOI: 10.1007/s00246-016-1362-2

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