scholarly journals Co-occurring Duchenne muscular dystrophy and hypertrophic cardiomyopathy in an adult with atypical cardiac phenotype

2014 ◽  
Vol 25 (2) ◽  
pp. 355-357 ◽  
Author(s):  
Animesh Tandon ◽  
Michael D. Taylor ◽  
Linda H. Cripe
Keyword(s):  
Late Gadolinium Enhancement ◽  
Duchenne Muscular Dystrophy ◽  
Hypertrophic Cardiomyopathy ◽  
Muscular Dystrophy ◽  
Systolic Function ◽  
Left Ventricular Systolic Function ◽  
Left Ventricular ◽  
Gadolinium Enhancement ◽  
Cardiac Phenotype ◽  
Septal Hypertrophy

AbstractWe present the case of a 29-year-old man with mutation-positive Duchenne muscular dystrophy and mutation-positive hypertrophic cardiomyopathy. His cardiac phenotype has characteristics of both disorders; he manifests sub-epicardial left ventricular free wall late gadolinium enhancement that is consistent with Duchenne cardiomyopathy, as well as asymmetric ventricular septal hypertrophy, hyperdynamic left ventricular systolic function, and septal mid-myocardial late gadolinium enhancement, which are characteristic of hypertrophic cardiomyopathy.

scholarly journals CARDIAC COMPLICATIONS IN DUCHENNE MUSCULAR DYSTROPHY IN CHILDREN

2015 ◽  
Vol 64 (4) ◽  
pp. 378-382
Author(s):  
Ioana Grigore ◽  
◽  
Mariana Frasin ◽  
Georgeta Diaconu ◽  
◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Systolic Function ◽  
Clinical Manifestations ◽  
Left Ventricular Systolic Function ◽  
Left Ventricular ◽  
Cardiac Complications ◽  
Echocardiographic Examination ◽  
Neurological Exam ◽  
Tissue Changes

Cardiac complications are frequently diagnosed in Duchenne muscular dystrophy (DMD), clinical manifestations generally appear after age 10. ECG recordings objectified various changes in these patients, the most common being represented by extensive R waves in V1, left deep Q waves, conduction abnormalities and arrhythmias. Echocardiographic examination may objective the presence of subclinical cardiac dysfunction in children under 12 years old diagnosed with DMD. In patients where standard echocardiographic examination is normal, myocardial performance index is a parameter useful in early detection of asymptomatic cardiac abnormalities. Cardiovascular magnetic resonance provides information both on left ventricular systolic function and myocardial tissue changes and occurrence of fibrosis, lesions rarely objectified before the age of 10 years. All these explorations should be considered in children with DMD at the end of a complete neurological exam.

scholarly journals Patterns of Left Ventricular Hypertrophy and Late Gadolinium Enhancement on Cardiac MRI in Patients with Hypertrophic Cardiomyopathy and their Prognostic Significance – An Experience from a South Asian Country

2021 ◽  
Vol 11 ◽  
pp. 14
Author(s):  
Fateh Ali Tipoo Sultan ◽  
Sheema Saadia
Keyword(s):  
Late Gadolinium Enhancement ◽  
Hypertrophic Cardiomyopathy ◽  
Prognostic Significance ◽  
Multivariable Analysis ◽  
Left Ventricular ◽  
University Hospital ◽  
Cardiac Events ◽  
Gadolinium Enhancement ◽  
Common Pattern ◽  
Septal Hypertrophy

Objectives: Cardiac magnetic resonance (CMR) imaging is very pertinent in the diagnosis and risk stratification of patients with hypertrophic cardiomyopathy (HCM). We aimed to assess the patterns of left ventricular (LV) hypertrophy, late gadolinium enhancement (LGE), and their prognostic significance in HCM patients in Pakistani population, as no such data are available from Pakistan. Material and Methods: This was a retrospective, single center study. All patients who had confirmed diagnosis of HCM on CMR at Aga Khan University Hospital during the period of 2011–2019 were identified and included in the study. Results: A total of 74 patients were included with the mean age of 45.6 ± 15 years and the majority 71.6 % (n = 53) being male. Maximal LV wall thickness was 21.1 ± 5 mm, asymmetrical septal hypertrophy being the most common pattern (62.2%, n = 46). LGE was present in 75.7% (n = 56) with most common site being septum plus LV free wall (24.3%, n =18). Mean ejection fraction% was found to be lower in patients with LGE (P < 0.001). Major adverse cardiac events (MACE) were observed in 40.5% (n = 30). Presence of LGE and right ventricular involvement was found to have a statistically significant association with MACE (P value 0.018 and 0.046, respectively). In multivariable analysis, only LGE was significantly associated with MACE (odd ratio: 4.65; 95% CI: 1.21–17.88). Conclusion: Asymmetrical septal hypertrophy was the most common pattern of hypertrophy. LGE was present in three fourth of the study population and it was significantly associated with MACE.

scholarly journals Effects of candesartan on left ventricular hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Maqsood ◽  
H.A Shakeel ◽  
H.F Shoukat ◽  
M.D Khan ◽  
S.A.Y Shah ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Hypertrophic Cardiomyopathy ◽  
Interstitial Fibrosis ◽  
Angiotensin Receptor Blockers ◽  
Left Ventricular ◽  
Funding Source ◽  
Gadolinium Enhancement ◽  
Percent Change ◽  
Lv Mass ◽  
Significant Difference

Abstract Introduction Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) hypertrophy in the absence of pressure overload. Manifestations of the disease include heart failure associated with diastolic dysfunction and atrial and ventricular tachyarrhythmias. Pathological features of HCM include myocyte hypertrophy, interstitial fibrosis, and myocyte disarray and are mediated by angiotensin II. Purpose This study aimed to evaluate the effects of candesartan on left ventricular (LV) hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy (HCM). Methods In double-blind fashion, 30 patients (6 women, 24 men; age: 55±11 years) with HCM were randomly assigned to receive placebo (n=13) or candesartan 50 mg twice a day (n=17) for 1 year. To measure LV mass and extent of fibrosis, cardiac magnetic resonance imaging was performed at baseline and 1 year as assessed by late gadolinium enhancement. Results There was a trend toward a significant difference in the percent change in LV mass (median: +5% with placebo vs. −5% with candesartan; p=0.06). There was a significant difference in the percent change in the extent of late gadolinium enhancement, with the placebo group experiencing a larger increase (+30±27% with placebo vs. −22±44% with candesartan; p=0.03). Conclusion Our study concludes reduction of the progression of myocardial hypertrophy and fibrosis with candesartan in patients with hypertrophic cardiomyopathy. Our study population was limited so we warrant larger trials to confirm a place for angiotensin receptor blockers in the management of patients with hypertrophic cardiomyopathy. Figure 1 Funding Acknowledgement Type of funding source: Other. Main funding source(s): Self funding

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