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

Muddassir Mehmood, Stephanie A. Ambach, Michael D. Taylor, John Jefferies, Subha V. Raman, Robin J. Taylor, Hemant Sawani, Jacob Mathew, Wojciech Mazur, Kan N. Hor, Hussein R. Al-Khalidi

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)878-883
Number of pages6
JournalPediatric Cardiology
Volume37
Issue number5
DOIs
StatePublished - Jun 1 2016
Externally publishedYes

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Right Ventricular Function
Duchenne Muscular Dystrophy
Vital Capacity
Heart Ventricles
Magnetic Resonance Spectroscopy
Spirometry
Lung

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

Mehmood, M., Ambach, S. A., Taylor, M. D., Jefferies, J., Raman, S. V., Taylor, R. J., ... Al-Khalidi, H. R. (2016). Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy. Pediatric Cardiology, 37(5), 878-883. https://doi.org/10.1007/s00246-016-1362-2

Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy. / Mehmood, Muddassir; Ambach, Stephanie A.; Taylor, Michael D.; Jefferies, John; Raman, Subha V.; Taylor, Robin J.; Sawani, Hemant; Mathew, Jacob; Mazur, Wojciech; Hor, Kan N.; Al-Khalidi, Hussein R.

In: Pediatric Cardiology, Vol. 37, No. 5, 01.06.2016, p. 878-883.

Research output: Contribution to journalArticle

Mehmood, M, Ambach, SA, Taylor, MD, Jefferies, J, Raman, SV, Taylor, RJ, Sawani, H, Mathew, J, Mazur, W, Hor, KN & Al-Khalidi, HR 2016, 'Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy', Pediatric Cardiology, vol. 37, no. 5, pp. 878-883. https://doi.org/10.1007/s00246-016-1362-2
Mehmood, Muddassir ; Ambach, Stephanie A. ; Taylor, Michael D. ; Jefferies, John ; Raman, Subha V. ; Taylor, Robin J. ; Sawani, Hemant ; Mathew, Jacob ; Mazur, Wojciech ; Hor, Kan N. ; Al-Khalidi, Hussein R. / Relationship of Right Ventricular Size and Function with Respiratory Status in Duchenne Muscular Dystrophy. In: Pediatric Cardiology. 2016 ; Vol. 37, No. 5. pp. 878-883.
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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.",
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AU - Sawani, Hemant

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AU - Al-Khalidi, Hussein R.

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