title: Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy.
abstract: 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.
authors.affiliation: Allegheny General Hospital, Pittsburgh, PA, USA.
authors.forename: Muddassir
authors.initials: M
authors.lastname: Mehmood
authors.affiliation: University of Cincinnati, Cincinnati, OH, USA.
authors.forename: Stephanie A
authors.initials: SA
authors.lastname: Ambach
authors.affiliation: Cincinnati Children's Hospital, Cincinnati, OH, USA.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Taylor
authors.affiliation: Cincinnati Children's Hospital, Cincinnati, OH, USA.
authors.forename: John L
authors.initials: JL
authors.lastname: Jefferies
authors.affiliation: Ohio State University, Columbus, OH, USA.
authors.forename: Subha V
authors.initials: SV
authors.lastname: Raman
authors.affiliation: The Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham, UK.
authors.forename: Robin J
authors.initials: RJ
authors.lastname: Taylor
authors.affiliation: Cincinnati Children's Hospital, Cincinnati, OH, USA.
authors.forename: Hemant
authors.initials: H
authors.lastname: Sawani
authors.affiliation: Cincinnati Children's Hospital, Cincinnati, OH, USA.
authors.forename: Jacob
authors.initials: J
authors.lastname: Mathew
authors.affiliation: Heart and Vascular Center, The Christ Hospital Health Network, Cincinnati, OH, USA. mazurw@ohioheart.org.
authors.forename: Wojciech
authors.initials: W
authors.lastname: Mazur
authors.affiliation: Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Kan N
authors.initials: KN
authors.lastname: Hor
authors.affiliation: Duke University School of Medicine, Durham, NC, USA.
authors.forename: Hussein R
authors.initials: HR
authors.lastname: Al-Khalidi
mesh_terms: D000293:Adolescent; D002648:Child; D006321:Heart; D006352:Heart Ventricles; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne; D012129:Respiratory Function Tests; D013318:Stroke Volume; D016278:Ventricular Function, Right; D055815:Young Adult
keywords: Cardiac magnetic resonance; Duchenne muscular dystrophy; Pulmonary function testing; Right ventricular function; Right ventricular volume