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CLINICAL RESEARCH: CARDIAC ULTRASOUND

Ultrasound tissue characterization detectspreclinical myocardial structural changes inchildren affected by Duchenne muscular dystrophy

Vincenzo Giglio, MD^*,*, Vincenzo Pasceri, MD, PhD, Loredana Messano, MD^*, Fortunato Mangiola, MD^*, Luciano Pasquini, MD^||, Antonio Dello Russo, MD^*, Antonello Damiani, MD^*, Massimiliano Mirabella, MD, PhD^*¶, Giuliana Galluzzi, MD, PhD^*¶, Pietro Tonali, MD^¶# and Enzo Ricci, MD^*¶

^* Center for Neuromuscular Diseases, Uildm, Rome, Italy
Division of Cardiology and ICU, St. Paolo Hospital Civitavecchia, Rome, Italy
Division of Cardiology, San Filippo Neri Hospital, Rome, Italy
Cardiovascular Diseases Department, Institute of Cardiology, Catholic University, Rome, Italy
^|| Division of Pediatric Cardiology, Bambino Gesù Hospital, Rome, Italy
^¶ Muscular Dystrophy Research Unit, Institute of Neurology, Catholic University, Rome, Italy
^# Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy

Manuscript received November 8, 2002; revised manuscript received December 18, 2002, accepted January 24, 2003.

^* Reprint requests and correspondence: Dr. Vincenzo Giglio, Center for Neuromuscular Diseases, Uildm, Prospero Santacroce Street, 5, 00167 Rome, Italy.
giglio.echo{at}libero.it

OBJECTIVES: Our goal was to identify early changes in myocardial physical properties in children with Duchenne muscular dystrophy (DMDch).

BACKGROUND: Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin, which triggers complex molecular and biological events in skeletal and cardiac muscle tissues. Although about 30% of patients display overt signs of cardiomyopathy in the late stage of the disease, it is unknown whether changes in myocardial physical properties can be detected in the early (preclinical) stages of the disease.

METHODS: We performed an ultrasonic tissue characterization (UTC) analysis of myocardium in DMDch with normal systolic myocardial function and no signs of cardiomyopathy. Both the cyclic variation of integrated backscatter (cvIBS) and the calibrated integrated backscatter (cIBS) were assessed in 8 myocardial regions of 20 DMDch, age 7 ± 2 years (range 4 to 10 years), and in 20 age-matched healthy controls.

RESULTS: We found large differences in the UTC data between DMDch and controls; the mean value of cvIBS was 4.4 ± 1.5 dB versus 8.8 ± 0.8 dB, whereas the mean value of cIBS was 36.4 ± 7.1 dB versus 26.9 ± 2.0 dB (p < 10^–6 for both). In DMDch, all eight sampled segments showed cIBS mean values to be significantly higher and cvIBS mean values to be significantly lower than those in the controls. Finally, interindividual differences were greater in DMDch than in controls for both parameters.

CONCLUSIONS: The myocardium in DMDch displays UTC features different from those in healthy controls. These results show that lack of dystrophin is commonly associated with changes in myocardial features well before the onset of changes of systolic function and overt cardiomyopathy.

Abbreviations and Acronyms   cIBS = calibrated integrated backscatter   cvIBS = cyclic variation of integrated backscatter   DMD = Duchenne muscular dystrophy   DMDch = Duchenne muscular dystrophy children   ECG = electrocardiogram   EF = ejection fraction   IBS = integrated backscatter   ROI = region of interest   UTC = ultrasonic tissue characterization   Vcfc = rate-corrected velocity of circumferential fiber shortening

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