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Correspondence |

Clinical Implications of MRI To Assess Cardiac and Pulmonary Function in Patients With Duchenne Muscular Dystrophy FREE TO VIEW

Josef Finsterer, MD; Claudia Stöllberger, MD
Author and Funding Information

From the Neurological Department (Dr Finsterer) and the Medical Department (Dr Stöllberger), Krankenanstalt Rudolfstiftung.

Correspondence to: Josef Finsterer, MD, Postfach 20, 1180 Vienna, Austria; e-mail: fifigs1@yahoo.de


Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2010 American College of Chest Physicians


Chest. 2010;138(3):756-757. doi:10.1378/chest.10-1282
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To the Editor:

We read with interest the article in CHEST (January 2005) by Mavrogeni et al1 about 17 patients with Duchenne muscular dystrophy (DMD), aged 7 to 25 years and without cardiac or pulmonary disease, in whom T2-relaxation time of the sternocleidomastoidei muscles and the myocardium indicated abnormal tissue composition, and why this technique was proposed for monitoring cardiac and pulmonary function in these patients. The presented findings raise the following concerns.

In the light of previous reports2,3 it is unusual that none of the 17 patients had clinical or subclinical cardiac disease. This is particularly curious because 12 patients were between 13 and 25 years old and because the prevalence of cardiac abnormalities in patients with DMD increases with age.3,4 Nearly all patients with DMD develop sinus tachycardia and in a series of 328 patients with DMD 25% had subclinical cardiac involvement before age 6 years and 59% between ages 7 and 10 years.3 Clinical cardiac disease develops after loss of ambulation and is present in all patients after 18 years of age.3 It is also uncommon that all patients with DMD had reduced T2-relaxation times, but none had any other abnormality on any other instrumental cardiac investigation. Were the cardiac investigations not carried out with proper care or attention? Which abnormalities of the skeletal muscle and myocardium can be determined by the T2-relaxation time, and which cannot be detected by any other method?

In a recent study, dystrophic changes within the myocardium before onset of overt cardiomyopathy or systolic dysfunction were detected on tissue Doppler imaging.5 Was tissue Doppler imaging carried out and compared with the MRI findings?

To effectively monitor cardiac and respiratory function in patients with DMD, more than a single MRI measurement is warranted. How can therapy be indicated for dilative cardiomyopathy in patients with DMD if only T2-relaxation time is measured? How can the right moment for starting noninvasive positive pressure ventilation be known if no capnographic examinations have been carried out? It is essential and recommended6,7 to apply a variety of clinical and instrumental cardiac and pulmonary investigations before eventually starting an appropriate therapy. We regard it as particularly irresponsible to propose a method without comparing it with reliable, well-established techniques beforehand.

Dystrophic alterations in the skeletal muscle of patients with DMD usually follow an inhomogeneous and patchy distribution. If a defined site within the muscle is investigated using MRI, it cannot be determined whether there is an extensive or scarce presence of dystrophy within this area. To correlate the MRI findings with the severity of the neurologic abnormalities, it would be useful to carry out a clinical neurologic investigation of each patient at the time when the MRI is carried out.

Which ECG and echocardiographic abnormalities did the authors particularly look for? If certain ECG and echocardiographic abnormalities are not expected to occur, they may be easily overlooked. This is particularly the case with echocardiographic abnormalities like left ventricular hypertrabeculation,8 which has been recently described for the first time, to our knowledge, in a patient with DMD.9

From 10% to 20% of the patients who are carriers of DMD are cardiologically and neurologically affected.10 Did the authors also investigate the mother of any patient? Did they find cardiac disease in any of these women?

Overall, monitoring of cardiac and pulmonary function in patients with DMD should not only rely on a single MRI parameter, but should also include various clinical and instrumental techniques. Before proposing a new technique as a means to monitor cardiac or pulmonary disease, the sensitivity and specificity of such a technique in comparison with an established technique should be assessed. Furthermore, the additional value of such a technique should be demonstrated.

Mavrogeni S, Tzelepis GE, Athanasopoulos G, et al. Cardiac and sternocleidomastoid muscle involvement in Duchenne muscular dystrophy: an MRI study. Chest. 2005;1271:143-148. [CrossRef] [PubMed]
 
Corrado G, Lissoni A, Beretta S, et al. Prognostic value of electrocardiograms, ventricular late potentials, ventricular arrhythmias, and left ventricular systolic dysfunction in patients with Duchenne muscular dystrophy. Am J Cardiol. 2002;897:838-841. [CrossRef] [PubMed]
 
Nigro G, Comi LI, Politano L, Bain RJ. The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy. Int J Cardiol. 1990;263:271-277. [CrossRef] [PubMed]
 
Bosser G, Lucron H, Lethor JP, et al. Evidence of early impairments in both right and left ventricular inotropic reserves in children with Duchenne’s muscular dystrophy. Am J Cardiol. 2004;936:724-727. [CrossRef] [PubMed]
 
Giglio V, Pasceri V, Messano L, et al. Ultrasound tissue characterization detects preclinical myocardial structural changes in children affected by Duchenne muscular dystrophy. J Am Coll Cardiol. 2003;422:309-316. [CrossRef] [PubMed]
 
Finder JD, Birnkrant D, Carl J, et al; American Thoracic Society American Thoracic Society Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med. 2004;1704:456-465. [CrossRef] [PubMed]
 
Shneerson JM, Simonds AK. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J. 2002;202:480-487. [CrossRef] [PubMed]
 
Stöllberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 2002;908:899-902. [CrossRef] [PubMed]
 
Finsterer J, Stöllberger C, Gaismayer K, et al. Acquired noncompaction in Duchenne muscular dystrophy. Int J Cardiol. 2006;1103:288-300. [CrossRef] [PubMed]
 
Nolan MA, Jones OD, Pedersen RL, Johnston HM. Cardiac assessment in childhood carriers of Duchenne and Becker muscular dystrophies. Neuromuscul Disord. 2003;132:129-132. [CrossRef] [PubMed]
 

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References

Mavrogeni S, Tzelepis GE, Athanasopoulos G, et al. Cardiac and sternocleidomastoid muscle involvement in Duchenne muscular dystrophy: an MRI study. Chest. 2005;1271:143-148. [CrossRef] [PubMed]
 
Corrado G, Lissoni A, Beretta S, et al. Prognostic value of electrocardiograms, ventricular late potentials, ventricular arrhythmias, and left ventricular systolic dysfunction in patients with Duchenne muscular dystrophy. Am J Cardiol. 2002;897:838-841. [CrossRef] [PubMed]
 
Nigro G, Comi LI, Politano L, Bain RJ. The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy. Int J Cardiol. 1990;263:271-277. [CrossRef] [PubMed]
 
Bosser G, Lucron H, Lethor JP, et al. Evidence of early impairments in both right and left ventricular inotropic reserves in children with Duchenne’s muscular dystrophy. Am J Cardiol. 2004;936:724-727. [CrossRef] [PubMed]
 
Giglio V, Pasceri V, Messano L, et al. Ultrasound tissue characterization detects preclinical myocardial structural changes in children affected by Duchenne muscular dystrophy. J Am Coll Cardiol. 2003;422:309-316. [CrossRef] [PubMed]
 
Finder JD, Birnkrant D, Carl J, et al; American Thoracic Society American Thoracic Society Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med. 2004;1704:456-465. [CrossRef] [PubMed]
 
Shneerson JM, Simonds AK. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J. 2002;202:480-487. [CrossRef] [PubMed]
 
Stöllberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 2002;908:899-902. [CrossRef] [PubMed]
 
Finsterer J, Stöllberger C, Gaismayer K, et al. Acquired noncompaction in Duchenne muscular dystrophy. Int J Cardiol. 2006;1103:288-300. [CrossRef] [PubMed]
 
Nolan MA, Jones OD, Pedersen RL, Johnston HM. Cardiac assessment in childhood carriers of Duchenne and Becker muscular dystrophies. Neuromuscul Disord. 2003;132:129-132. [CrossRef] [PubMed]
 
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