Year : 2016  |  Volume : 9  |  Issue : 2  |  Page : 190--191

Unique pattern of late gadolinium enhancement on cardiac magnetic resonance imaging in Duchenne muscular dystrophy


Madhusudan Ganigara1, Bharti Sharma1, Ravi Babu Komalla1, Suman Y Vyas1, Gopichand Mannam2, Nitin Krishna Rao1,  
1 Department of Pediatric Cardiology, Star Hospitals, Hyderabad, Telangana, India
2 Department of Cardiothoracic Surgery, Star Hospitals, Hyderabad, Telangana, India

Correspondence Address:
Madhusudan Ganigara
Department of Pediatric Cardiology, Star Hospitals, Banjara Hills, Hyderabad - 500 034, Telangana
India

Abstract

Cardiomyopathy is an important cause of morbidity and mortality in patients with Duchenne muscular dystrophy (DMD). Early recognition of myocardial involvement and initiation of therapy are important for improved outcomes. Cardiac magnetic resonance imaging (CMR) is a sensitive tool in early detection of myocardial fibrosis in these children.



How to cite this article:
Ganigara M, Sharma B, Komalla RB, Vyas SY, Mannam G, Rao NK. Unique pattern of late gadolinium enhancement on cardiac magnetic resonance imaging in Duchenne muscular dystrophy.Ann Pediatr Card 2016;9:190-191


How to cite this URL:
Ganigara M, Sharma B, Komalla RB, Vyas SY, Mannam G, Rao NK. Unique pattern of late gadolinium enhancement on cardiac magnetic resonance imaging in Duchenne muscular dystrophy. Ann Pediatr Card [serial online] 2016 [cited 2020 Jun 4 ];9:190-191
Available from: http://www.annalspc.com/text.asp?2016/9/2/190/181496


Full Text

 Case Report



An 11-year-old boy with Duchenne muscular dystrophy (DMD), diagnosed in the first decade of his life, was referred for cardiac evaluation. He was able to ambulate without support. He denied exertional chest discomfort, shortness of breath, palpitations, or syncope. On physical examination, his blood pressure was 100/60 mm Hg, with a heart rate of 82 beats/min. The jugular venous pulse was normal. There were normal cardiac heart sounds with no murmurs. There were no signs of cardiac failure on physical examination. A 12-lead electrocardiogram demonstrated partial right bundle branch block. Transthoracic echocardiography demonstrated normal-sized ventricles with normal ventricular function.

Cardiac magnetic resonance imaging (CMR), performed on a 1.5 Tesla (T) scanner (Achieva, Philips, Best, The Netherlands) showed normal left ventricular (LV) size and systolic function [LV indexed end-diastolic volume of 69 mL/m 2 and LV ejection fraction (LVEF) of 68%]. On late gadolinium enhancement (LGE) imaging there was subepicardial enhancement of the mid- and basal-posterolateral segments of the LV free wall with the involvement of less than one-third of the LV free wall length and no septal involvement [Figure 1] and [Figure 2]. Based on the above findings on CMR he was initiated on therapy with angiotensin-converting enzyme (ACE) inhibitors.{Figure 1}{Figure 2}

 Discussion



With improved magnetic resonance imaging (MRI) technology and protocols, and better understanding of the disease progression, CMR is increasingly being used as the preferred mode for surveillance imaging in children with DMD.[1] The finding of LGE, a marker of myocardial fibrosis, has a unique pattern in patients with DMD as noted in the present case. Hor et al. noted that LGE was more prevalent in the free wall segments compared to the septal segments (42.7% versus 5.3%).[2] LGE was more prevalent at the base and mid-ventricle, and in the anterolateral, inferolateral, and inferior segments. Florian et al. showed that subepicardial and intramural LGE were the most common form of myocardial involvement in DMD.[3] Furthermore, they noted that transmural pattern of LGE and impaired LV systolic function (LVEF <45%) were independent predictors of adverse cardiac events.

It is hypothesized that fibrofatty replacement of the myocardium results in diffuse myocardial fibrosis and is a key step in the development of cardiac dysfunction.[4] Menon et al. note thatLGE on CMR is an independent predictor of adverse cardiac remodelling, ventricular arrhythmias, and death in DMD.[5] Given that CMR is a sensitive tool to detect myocardial fibrosis well before any ventricular dysfunction is evident, this finding has important implications in the management of these patients, including on the time of initiation of medical therapy. Some studies have noted that early institution of ACE inhibition delayed the onset of LV dysfunction and improved mortality.[1]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1McNally EM, Kaltman JR, Benson DW, Canter CE, Cripe LH, Duan D, et al.; Working Group of the National Heart, Lung, and Blood Institute; Parent Project Muscular Dystrophy. Contemporary cardiac issues in Duchenne muscular dystrophy. Circulation 2015;131:1590-8.
2Hor KN, Taylor MD, Al-Khalidi HR, Cripe LH, Raman SV, Jefferies JL, et al. Prevalence and distribution of late gadolinium enhancement in a large population of patients with Duchenne muscular dystrophy: Effect of age and left ventricular systolic function. J Cardiovasc Magn Reson 2013;15:107.
3Florian A, Ludwig A, Engelen M, Waltenberger J, Rösch S, Sechtem U, et al. Left ventricular systolic function and the pattern of late-gadolinium-enhancement independently and additively predict adverse cardiac events in muscular dystrophy patients. J Cardiovasc Magn Reson 2014;16:81.
4Puchalski MD, Williams RV, Askovich B, Sower CT, Hor KH, Su JT, et al. Late gadolinium enhancement: Precursor to cardiomyopathy in Duchenne muscular dystrophy? Int J Cardiovasc Imaging 2009;25:57-63.
5Menon SC, Etheridge SP, Liesemer KN, Williams RV, Bardsley T, Heywood MC, et al. Predictive value of myocardial delayed enhancement in Duchenne muscular dystrophy. Pediatr Cardiol 2014;35:1279-85.