A Comparison Between MUGA and Echocardiography in Patients with Muscular Dystrophy in the Early Detection of Cardiac Involvement

Oğuz, Deniz; Olguntürk, Rana; Gücüyener, Kıvılcım; Acikgoz, Guncel; Tunaoğlu, F. Sedef

doi:10.1007/s002469900264

Cited by 15 publications

(15 citation statements)

References 19 publications

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“…The echocardiographic criteria were similar to those used in our study. There is evidence in XLD patients and carriers that echocardiography is a non-invasive diagnostic tool with similar sensitivity to radionuclide angiography techniques in assessing left ventricular systolic function [15].…”

Section: Discussionmentioning

confidence: 99%

Cardiac assessment in childhood carriers of Duchenne and Becker muscular dystrophies

Nolan

Jones

Pedersen

et al. 2003

Neuromuscular Disorders

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Cardiac disease in adult female carriers of the X-linked dystrophinopathies, Duchenne and Becker muscular dystrophies, is a wellrecognised entity. A single study has reported a 15% incidence of cardiac abnormalities in female carriers under 16 years. Our study aims, clinically and with electrocardiograph and echocardiograph, to determine the incidence of cardiac abnormality in young girls who are proven carriers of X-linked dystrophinopathies. Twenty-three girls aged 6.2-15.9 years were assessed. All had normal cardiac examination. None had electrocardiograph abnormalities consistent with dystrophic cardiomyopathy. Left ventricular fractional shortening ranged from 33 to 55% (normal . 28%). Septal thickness, posterior wall thickness and wall thickness ratio were within normal limits. No cardiac abnormalities have been demonstrated in young girls who are proven carriers of X-linked dystrophinopathies in our study. This has important implications for planning timing of carrier determination and cardiac assessment. q

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Section: Discussionmentioning

confidence: 99%

Cardiac assessment in childhood carriers of Duchenne and Becker muscular dystrophies

Nolan

Jones

Pedersen

et al. 2003

Neuromuscular Disorders

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“…Multigated cardiac radionuclide ventriculography (MUGA) is not affected by these problems and has been found to be sensitive in detecting subclinical cardiomyopathy in DMD, BMD and female carriers [6, 31]. MUGA was able to detect a predominant systolic and minor diastolic left ventricular dysfunction even in the BMD patients and female carriers where there was no skeletal muscle involvement [31].…”

Section: Diagnosis Of Cardiomyopathy In Dmd Patientsmentioning

confidence: 99%

“…MUGA was able to detect a predominant systolic and minor diastolic left ventricular dysfunction even in the BMD patients and female carriers where there was no skeletal muscle involvement [31]. However, MUGA is associated with repeated radiation exposure, which becomes more relevant in DMD when annual screening of cardiac function is recommended to monitor the function of the heart and the progression of the cardiomyopathy in DMD patients.…”

Section: Diagnosis Of Cardiomyopathy In Dmd Patientsmentioning

confidence: 99%

Experimental Models of Duchenne Muscular Dystrophy: Relationship with Cardiovascular Disease

Ameen

Robson²

2010

TOCMJ

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Almost every boy that has Duchenne Muscular Dystrophy (DMD) will develop cardiac problems. Whereas, it used to be respiratory problems that was the main cause of death in these DMD boys; with the advent of better respiratory care it is now the cardiac involvement that is becoming the most common cause of their death. Once the heart is affected, there is progressive deterioration in the function of the heart over time. The main problem is the death of the cardiomyocytes. The cause of the cardiomyocyte death is due to the loss of dystrophin, this makes the sarcolemma more susceptible to damage, and leads to a cascade of calcium influx, calcium activated proteases and ultimately the death of the cardiomyocyte. The dead cardiomyocytes are replaced by fibrotic tissue, which results in a dilated cardiomyopathy (DCM) developing, which begins in the base of the left ventricle and progresses to involve the entire left ventricle. The treatments used for the DMD cardiomyopathy are based on ones designed for other forms of cardiac weakness and include ACE-inhibitors and β-blockers. New therapies based around the pathophysiology in DMD are now being introduced. This review will look at the pathophysiology of the cardiac problems in DMD and how the various animal models that are available can be used to design new treatment options for DMD boys.

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“…MUGA scanning has been used to accurately assess left ventricular ejection fraction in all types of patients. Although it is an effective and sensitive technique for assessing LV function in DMD, 7 it is much more time consuming. In total, this examination takes at least an hour as the patient has to be positioned in front of the camera and it is more laborious for the technician and accompanying parents or other helpers, as well as more burdensome for these severely disabled patients.…”

Section: Figure 2 Left Ventricular Ejection Fraction (%) Measured Bymentioning

confidence: 99%

“…Multigated cardiac radionuclide ventriculography (MUGA) is not affected by these problems and has been found to be sensitive in detecting subclinical DMD cardiomyopathy. 7 However, MUGA is not easy to perform in DMD patients and, moreover, it is associated with radiation exposure, which is even more relevant in case of repeated investigations.…”

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confidence: 99%

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

Bockel¹,

Lind²,

Zijlstra³

et al. 2009

NHJL

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Background. Duchenne muscular dystrophy (DMD) patients used to die mainly from pulmonary problems. However, as advances in respiratory care increase life expectancy, mortality due to cardiomyopathy rises. Echocardiography remains the standard diagnostic modality for cardiomyopathy in DMD patients, but is hampered by scoliosis and poor echocardiographic acoustic windows in adult DMD patients. Multigated cardiac radionuclide ventriculography (MUGA) does not suffer from these limitations. N-terminal proBNP (NTproBNP) has shown to be a diagnostic factor for heart failure. We present our initial experience with plasma NT-proBNP measurement in the routine screening and diagnosis of cardiomyopathy in adult mechanically ventilated DMD patients.Methods. Retrospective study, 13 patients. Echocardiography classified left ventricular (LV) function as preserved or depressed. NT-proBNP was determined using immunoassay. LV ejection fraction (LVEF) was determined using MUGA.Results. Median (range) NT-proBNP was 73 (25 to 463) ng/l. Six patients had an NT-proBNP >125 ng/l. Seven patients showed an LVEF <45% on MUGA. DMD patients with depressed LV function (n=4) as assessed by echocardiography had significantly higher median NT-proBNP than those (n=9) with preserved LV function: 346 (266 to 463) ng/l versus 69 (25 to 257) ng/l (p=0.003). NT-proBNP significantly correlated with depressed LV function on echocardiogram and with LVEF determined by MUGA.Conclusion. Although image quality of MUGA is superior to echocardiography, the combination of echocardiography and NT-proBNP achieves similar results in the evaluation of left ventricular function and is less time consuming and burdensome for our patients. We advise to add NT-proBNP to echocardiography in the routine cardiac assessment of DMD patients. (Neth Heart J 2009;17:232-7.).

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A Comparison Between MUGA and Echocardiography in Patients with Muscular Dystrophy in the Early Detection of Cardiac Involvement

Cited by 15 publications

References 19 publications

Cardiac assessment in childhood carriers of Duchenne and Becker muscular dystrophies

Cardiac assessment in childhood carriers of Duchenne and Becker muscular dystrophies

Experimental Models of Duchenne Muscular Dystrophy: Relationship with Cardiovascular Disease

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

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