Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients

Cirino, Raphael Henrique Déa; Scola, Rosana Hermínia; Ducci, Renata Dal‐Prá; Wermelinger, Ana Cristina Camarozano; Kay, Cláudia Suemi Kamoi; Lorenzoni, Paulo José; Werneck, Lineü Cesar; Carmes, Eliane Ribeiro; Cunha, Cristiane Martins

doi:10.1002/mus.26102

Cited by 8 publications

(9 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The genetic diagnosis was mandatory for enrolling patients in this study. The most common prevalent exon mutations were from 45 to 50 that is matching with previous studies [19]. In this study exon mutations, 45-47 and 51-54 were significantly associated with EF < 60%.…”

Section: Discussionsupporting

confidence: 90%

“…Moreover, a significant moderate inverse correlation was found between the age of DMD patients and the decline in GLS. This is supported by Nigro et al and Cirino et al who found that cardiac problems typically start as latent cardiomyopathy without symptoms evolving into clinically overt cardiac disease in 14% of patients under the age of 14 years, to be present in 57% of patients over 18 years of age [ 19 , 22 ]. This study presents global longitudinal strain as a simple potential tool that could be used in the periodic follow-up of patients and carriers with a lower cost in comparison with CMR to detect subtle LV systolic dysfunction that may allow early initiation of anti-failure treatments that improve their prognosis.…”

Section: Discussionmentioning

confidence: 79%

“…In this study exon mutations, 45–47 and 51–54 were significantly associated with EF < 60%. Variable exon mutations were previously related to cardiomyopathy; however, these results may be misleading due to the small number of patients with the same exon mutations [ 19 , 20 ]. In the present study, the number of exon mutations was not correlated with LV systolic or diastolic functions.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Global longitudinal strain detects subtle left ventricular systolic dysfunction in Duchenne muscular dystrophy patients and carriers

et al. 2021

View full text Add to dashboard Cite

Background With the continuous improvement of the respiratory care of Duchenne muscular dystrophy patients, cardiac manifestations (heart failure and arrhythmias) become the leading causes of morbidity and mortality. Early identification of cardiac muscle affection is crucial to start anti-failure drugs that reverse remodeling and improve prognosis. This study aimed to detect subtle cardiac changes in Duchenne muscular dystrophy patients and carriers using electrocardiography and echocardiography. Results This study included genetically diagnosed Duchenne muscular dystrophy patients (28 males) and carriers (25 females) and compared them to healthy gender-matched control groups. All study participants underwent clinical assessment, 12-lead electrocardiography, and global longitudinal strain augmented echocardiography. In the current study, Duchenne muscular dystrophy patients had higher heart rates, smaller left ventricular internal diameters, left atrial diameter, lower ejection fraction, and worse left ventricular global longitudinal strain in comparison with the control group. The global longitudinal strain inversely correlated with the age of Duchenne muscular dystrophy patients. The number of exon mutations did not affect electrocardiography and echocardiographic findings. Exon mutations 45–47 and 51–54 were significantly associated with an ejection fraction less than 60%. Duchenne muscular dystrophy carriers had smaller left ventricular wall diameters, left ventricular end-diastolic diameter, left atrial diameter, and worse left ventricular global longitudinal strain in comparison with the control group. Conclusions Left ventricular global longitudinal strain could detect subtle left ventricular systolic dysfunction in Duchenne muscular dystrophy patients and carriers before the decline of left ventricular ejection fraction.

show abstract

Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Global longitudinal strain detects subtle left ventricular systolic dysfunction in Duchenne muscular dystrophy patients and carriers

et al. 2021

View full text Add to dashboard Cite

show abstract

“…In this context, echocardiography is an essential tool in MD management because, in addition to being widely available and of low cost, it assesses anatomic-functional characteristics of the heart and detects preclinical changes. 3 Due to the development of new specific therapies, the increased survival of patients with some types of MD has made cardiological complications the leading cause of morbidity and mortality. For this reason, the early detection of cardiac changes and the introduction of cardiomyopathy-directed therapy have become essential.…”

Section: General Aspects Related To the Clinical And Echocardiographi...mentioning

confidence: 99%

My approach to diagnosis of and echocardiographic follow-up for muscular dystrophies

Cirino¹,

Ducci²

2022

Arq Bras Cardiol: Imagem Cardiovasc

View full text Add to dashboard Cite

“…7 Thus, earlier detection of cardiac dysfunction is even more important. Recently, Cirino et al 8 sought to find predictors of early left ventricular systolic dysfunction (LVSD) in patients with DMD. They analyzed myocardial strain using transthoracic echocardiology (TTE) and determined predictors of early LVSD in those with abnormal myocardial strain.…”

Section: Muscular Dystrophymentioning

confidence: 99%

What is in the Myopathy Literature?

Lacomis¹

2018

Journal of Clinical Neuromuscular Disease

View full text Add to dashboard Cite

We review the development of exon 51 skipping therapy with eteplirsen for Duchenne muscular dystrophy, including the recent report of longterm, sustained dystrophin production. Studies of the late-life health profile of patients with Duchenne muscular dystrophy, early detection of left ventricular systolic dysfunction, and caregiver burden are also covered. A study of skeletal muscle magnetic resonance imaging in dysferlinopathies provides an extensive, detailed map of the involved muscles and consistency across phenotypes. Regarding the category of autoimmune myopathies, we discuss an article on the clinical and laboratory features associated with PM/Scl antibodies in comparison with other autoimmune myopathy subgroups. Finally, the overall increase in mortality in inflammatory myopathies is highlighted in a recent report from Sweden.

show abstract

Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients

Abstract: Myocardial strain measurements are useful for the early diagnosis of LVSD in DMD patients. Older age, use of corticosteroids, and mutations within the "hot-spot" region of the DMD gene are associated with early LVSD. Muscle Nerve, 2018.

Cited by 8 publications

References 36 publications

Global longitudinal strain detects subtle left ventricular systolic dysfunction in Duchenne muscular dystrophy patients and carriers

Global longitudinal strain detects subtle left ventricular systolic dysfunction in Duchenne muscular dystrophy patients and carriers

My approach to diagnosis of and echocardiographic follow-up for muscular dystrophies

What is in the Myopathy Literature?

Contact Info

Product

Resources

About