Your browser doesn't support javascript.
loading
Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy.
Mehmood, Muddassir; Ambach, Stephanie A; Taylor, Michael D; Jefferies, John L; Raman, Subha V; Taylor, Robin J; Sawani, Hemant; Mathew, Jacob; Mazur, Wojciech; Hor, Kan N; Al-Khalidi, Hussein R.
Afiliação
  • Mehmood M; Allegheny General Hospital, Pittsburgh, PA, USA.
  • Ambach SA; University of Cincinnati, Cincinnati, OH, USA.
  • Taylor MD; Cincinnati Children's Hospital, Cincinnati, OH, USA.
  • Jefferies JL; Cincinnati Children's Hospital, Cincinnati, OH, USA.
  • Raman SV; Ohio State University, Columbus, OH, USA.
  • Taylor RJ; The Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham, UK.
  • Sawani H; Cincinnati Children's Hospital, Cincinnati, OH, USA.
  • Mathew J; Cincinnati Children's Hospital, Cincinnati, OH, USA.
  • Mazur W; Heart and Vascular Center, The Christ Hospital Health Network, Cincinnati, OH, USA. mazurw@ohioheart.org.
  • Hor KN; Nationwide Children's Hospital, Columbus, OH, USA.
  • Al-Khalidi HR; Duke University School of Medicine, Durham, NC, USA.
Pediatr Cardiol ; 37(5): 878-83, 2016 Jun.
Article em En | MEDLINE | ID: mdl-26936620
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.
Assuntos
Palavras-chave

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Distrofia Muscular de Duchenne Limite: Adolescent / Adult / Child / Humans / Male Idioma: En Ano de publicação: 2016 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Distrofia Muscular de Duchenne Limite: Adolescent / Adult / Child / Humans / Male Idioma: En Ano de publicação: 2016 Tipo de documento: Article