Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy

Arshjot Khokhar, Athira Nair, Vishal Midya, Ashutosh Kumar, Ankita Sinharoy, Tariq Ali Ahmad, Mutasim Abu-Hasan, Pritish Mondal

Research output: Contribution to journalArticle

Abstract

Introduction: Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. Methods: We conducted a retrospective chart review of 27 patients with DMD. Our primary objective was to compare the rates of decline between pulmonary function test (PFT) measures (forced expiratory volume in the first second, forced vital capacity, peak expiratory flow rate, maximal inspiratory/expiratory pressure) and echocardiographic estimates of LV end-diastolic volume and LV ejection fraction. Results: The rates of decline/year of PFTs and LV estimates were not significantly different. Pulmonary function test measures of ventilatory efficiency and strength had strong intercorrelations. Pulmonary function tests and LV estimates had weak but statistically significant correlations. Discussion: A comparable rate of decline in PFTs and LV indices in DMD provides evidence for concurrently progressive deterioration in respiratory and LV functions. Muscle Nerve, 2019.

Original languageEnglish (US)
Pages (from-to)286-291
Number of pages6
JournalMuscle and Nerve
Volume60
Issue number3
DOIs
StatePublished - Jan 1 2019

Fingerprint

Ventricular Function
Duchenne Muscular Dystrophy
Respiratory Function Tests
Lung
Stroke Volume
Peak Expiratory Flow Rate
Dystrophin
Vital Capacity
Forced Expiratory Volume
Left Ventricular Function
Cardiac Myocytes
Skeletal Muscle
Muscles
Proteins
Maximal Respiratory Pressures

All Science Journal Classification (ASJC) codes

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

Cite this

Khokhar, Arshjot ; Nair, Athira ; Midya, Vishal ; Kumar, Ashutosh ; Sinharoy, Ankita ; Ahmad, Tariq Ali ; Abu-Hasan, Mutasim ; Mondal, Pritish. / Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy. In: Muscle and Nerve. 2019 ; Vol. 60, No. 3. pp. 286-291.
@article{55b74cf205a646eeb349106998457428,
title = "Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy",
abstract = "Introduction: Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. Methods: We conducted a retrospective chart review of 27 patients with DMD. Our primary objective was to compare the rates of decline between pulmonary function test (PFT) measures (forced expiratory volume in the first second, forced vital capacity, peak expiratory flow rate, maximal inspiratory/expiratory pressure) and echocardiographic estimates of LV end-diastolic volume and LV ejection fraction. Results: The rates of decline/year of PFTs and LV estimates were not significantly different. Pulmonary function test measures of ventilatory efficiency and strength had strong intercorrelations. Pulmonary function tests and LV estimates had weak but statistically significant correlations. Discussion: A comparable rate of decline in PFTs and LV indices in DMD provides evidence for concurrently progressive deterioration in respiratory and LV functions. Muscle Nerve, 2019.",
author = "Arshjot Khokhar and Athira Nair and Vishal Midya and Ashutosh Kumar and Ankita Sinharoy and Ahmad, {Tariq Ali} and Mutasim Abu-Hasan and Pritish Mondal",
year = "2019",
month = "1",
day = "1",
doi = "10.1002/mus.26623",
language = "English (US)",
volume = "60",
pages = "286--291",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "3",

}

Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy. / Khokhar, Arshjot; Nair, Athira; Midya, Vishal; Kumar, Ashutosh; Sinharoy, Ankita; Ahmad, Tariq Ali; Abu-Hasan, Mutasim; Mondal, Pritish.

In: Muscle and Nerve, Vol. 60, No. 3, 01.01.2019, p. 286-291.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Association between pulmonary function and left ventricular volume and function in duchenne muscular dystrophy

AU - Khokhar, Arshjot

AU - Nair, Athira

AU - Midya, Vishal

AU - Kumar, Ashutosh

AU - Sinharoy, Ankita

AU - Ahmad, Tariq Ali

AU - Abu-Hasan, Mutasim

AU - Mondal, Pritish

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Introduction: Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. Methods: We conducted a retrospective chart review of 27 patients with DMD. Our primary objective was to compare the rates of decline between pulmonary function test (PFT) measures (forced expiratory volume in the first second, forced vital capacity, peak expiratory flow rate, maximal inspiratory/expiratory pressure) and echocardiographic estimates of LV end-diastolic volume and LV ejection fraction. Results: The rates of decline/year of PFTs and LV estimates were not significantly different. Pulmonary function test measures of ventilatory efficiency and strength had strong intercorrelations. Pulmonary function tests and LV estimates had weak but statistically significant correlations. Discussion: A comparable rate of decline in PFTs and LV indices in DMD provides evidence for concurrently progressive deterioration in respiratory and LV functions. Muscle Nerve, 2019.

AB - Introduction: Duchenne muscular dystrophy (DMD) is characterized by absence of the subsarcolemmal protein dystrophin, present in skeletal muscles and cardiomyocytes. We hypothesized that progressive respiratory and left ventricular (LV) insufficiencies in DMD could be parallel and interrelated phenomena. Methods: We conducted a retrospective chart review of 27 patients with DMD. Our primary objective was to compare the rates of decline between pulmonary function test (PFT) measures (forced expiratory volume in the first second, forced vital capacity, peak expiratory flow rate, maximal inspiratory/expiratory pressure) and echocardiographic estimates of LV end-diastolic volume and LV ejection fraction. Results: The rates of decline/year of PFTs and LV estimates were not significantly different. Pulmonary function test measures of ventilatory efficiency and strength had strong intercorrelations. Pulmonary function tests and LV estimates had weak but statistically significant correlations. Discussion: A comparable rate of decline in PFTs and LV indices in DMD provides evidence for concurrently progressive deterioration in respiratory and LV functions. Muscle Nerve, 2019.

UR - http://www.scopus.com/inward/record.url?scp=85068789926&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85068789926&partnerID=8YFLogxK

U2 - 10.1002/mus.26623

DO - 10.1002/mus.26623

M3 - Article

C2 - 31250930

AN - SCOPUS:85068789926

VL - 60

SP - 286

EP - 291

JO - Muscle and Nerve

JF - Muscle and Nerve

SN - 0148-639X

IS - 3

ER -