Abstract:
Importance:Cardiac dysfunction is a leading cause of morbidity and mortality in Duchenne muscular dystrophy (DMD). This case highlights the importance of steroids in treating cardiac complications of DMD and the dangers of discontinuing or switching between steroid classes. Objective:To recognize the presentation of acute myocardial inflammation, or dystrophinitis, in DMD, which presents as myocarditis and to treat the myocardial inflammation and dilated cardiomyopathy associated with DMD through guideline-directed medical therapy, steroids, and serial surveillance for cardiac dysfunction. Design, Setting, and Participant:A case report of an 18-year-old patient with DMD and with steroid withdrawal-induced myocarditis followed up for 3 years to observe for cardiac function recovery and the natural history of cardiomyopathy in DMD, who was hospitalized in the cardiac care unit and followed up between November 3, 2016, and March 27, 2017. Exposures:Switching from deflazacort to underdosed prednisone for 7 days. Main Outcomes and Measures:Increased myocardial inflammation, edema, and fibrosis after stopping deflazacort abruptly. Results:An 18-year-old male patient with DMD presented to the emergency department with acute-onset chest pain. Ischemic changes were present on electrocardiogram, and elevated cardiac enzymes were detected. Depressed cardiac function and potential evidence of inflammation were seen on cardiac magnetic resonance (CMR) imaging, characterized by elevated T2 values and late gadolinium enhancement. These findings were all consistent with acute myocarditis but without a viral prodrome. Several days prior to presentation, the patient's deflazacort was abruptly discontinued and converted to an equivalent dose of prednisone. After restarting deflazacort, his symptoms improved, and subsequent CMR showed resolution of myocardial edema and improved left ventricular function. Conclusions and Relevance:This case highlights adverse effects associated with changing between corticosteroid classes in DMD cardiomyopathy and also demonstrates the utility of CMR in detecting myocardial inflammation and monitoring response to treatment.
journal_name
JAMA Cardioljournal_title
JAMA cardiologyauthors
Abutaleb ARA,McNally EM,Khan SS,Anderson AS,Carr JC,Wilcox JEdoi
10.1001/jamacardio.2018.2695subject
Has Abstractpub_date
2018-10-01 00:00:00pages
1006-1010issue
10eissn
2380-6583issn
2380-6591pii
2698085journal_volume
3pub_type
杂志文章相关文献
JAMA Cardiology文献大全abstract:Importance:High-resolution stratification of risk of sudden cardiac arrest (SCA) in individual patients is a tool that is necessary for achieving effective and efficient application of data generated by population-based research. This concept is at the core of initiatives for merging cost effectiveness with maximized c...
journal_title:JAMA cardiology
pub_type: 杂志文章,评审
doi:10.1001/jamacardio.2017.0266
更新日期:2017-06-01 00:00:00
abstract:Importance:Cardiac stress testing is often performed prior to noncardiac surgery, although trends in use of preoperative stress testing and the effect of testing on cardiovascular outcomes are currently unknown. Objective:To describe temporal trends and outcomes of preoperative cardiac stress testing from 2004 to 2017...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2020.4311
更新日期:2021-01-01 00:00:00
abstract:Importance:The Department of Veterans Affairs (VA) operates a nationwide system of hospitals and hospital-affiliated clinics, providing health care to more than 2 million veterans with cardiovascular disease. While data permitting hospital comparisons of the outcomes of acute cardiovascular care (eg, myocardial infarct...
journal_title:JAMA cardiology
pub_type: 杂志文章,多中心研究
doi:10.1001/jamacardio.2018.1115
更新日期:2018-07-01 00:00:00
abstract:Importance:Myocardial replacement fibrosis has been reported to occur in one-third of patients with mitral valve prolapse (MVP) and significant mitral regurgitation (MR). However, it remains unknown whether there are detectable changes in myocardial metabolism suggestive of inflammation or ischemia that accompany the d...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2020.1555
更新日期:2020-09-01 00:00:00
abstract:IMPORTANCE:The adverse outcomes associated with atrial fibrillation (AF) have been studied in predominantly white cohorts. Racial differences in outcomes associated with AF merit continued investigation. OBJECTIVE:To evaluate the race-specific associations of AF with stroke, heart failure, coronary heart disease (CHD)...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2016.1025
更新日期:2016-07-01 00:00:00
abstract:Importance:Improvements in symptoms, functional capacity, and quality of life are among the key goals of edge-to-edge transcatheter mitral valve repair (TMVR) for mitral regurgitation. Objective:To examine health status outcomes among patients undergoing TMVR in clinical practice and the factors associated with improv...
journal_title:JAMA cardiology
pub_type: 杂志文章,多中心研究
doi:10.1001/jamacardio.2018.3359
更新日期:2018-12-01 00:00:00
abstract:Importance:Despite considerable improvements in heart failure care, mortality rates among patients in high-income countries have changed little since the early 2000s. Understanding the reasons underlying these trends may provide valuable clues for developing more targeted therapies and public health strategies. Object...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2019.3593
更新日期:2019-11-01 00:00:00
abstract:Importance:Physicians have been criticized for having an overly enthusiastic response to new device approvals, especially for novel technologies. However, to our knowledge, the rates of new product adoption and patterns of new device usage in clinical practice have not been well described. Objective:To characterize th...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2019.0388
更新日期:2019-06-01 00:00:00
abstract:Importance:Transcatheter aortic valve replacement (TAVR) is an established alternative to surgery for patients with severe symptomatic aortic stenosis. Adjunctive antithrombotic therapy used to mitigate thrombotic risks in patients undergoing TAVR must be balanced against bleeding complications, since both are associat...
journal_title:JAMA cardiology
pub_type: 杂志文章,评审
doi:10.1001/jamacardio.2019.4367
更新日期:2020-01-01 00:00:00
abstract:Importance:Acute mental stress can result in transient endothelial dysfunction, but the prognostic relevance of this phenomenon is unknown. Objective:To determine the association between mental stress-induced impairment in endothelium-dependent relaxation as assessed by brachial artery flow-mediated vasodilation and a...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2019.3252
更新日期:2019-10-01 00:00:00
abstract:Importance:Many of the quality measures used to assess accountable care organization (ACO) performance in the Medicare Shared Savings Program (MSSP) focus on disease control and medication use among patients with cardiovascular disease and diabetes. To date, the association between participation in the MSSP by provider...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2017.2172
更新日期:2017-09-01 00:00:00
abstract:Importance:Early menarche and early menopause are associated with increased risk of cardiovascular disease (CVD) in midlife, but little is known about the association between reproductive life span and the risk of CVD. Objective:To investigate the association between the length of reproductive life span and risk of in...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2020.4105
更新日期:2020-09-16 00:00:00
abstract:Importance:Hutchinson-Gilford progeria syndrome (HGPS) is an ultrarare disorder associated with premature death due to cardiovascular events during the second decade of life. However, because of its rarity (107 identified living patients), the natural history of cardiac disease remains uncharacterized. Therefore, meani...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2017.5235
更新日期:2018-04-01 00:00:00
abstract:Importance:The period following heart failure hospitalization (HFH) is a vulnerable time with high rates of death or recurrent HFH. Objective:To evaluate clinical characteristics, outcomes, and treatment response to vericiguat according to prespecified index event subgroups and time from index HFH in the Vericiguat Gl...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2020.6455
更新日期:2020-11-13 00:00:00
abstract:Importance:In clinical trials, transcatheter aortic valve replacement (TAVR) has been shown to improve symptoms and quality of life. As this technology moves into general clinical practice, evaluation of the health status outcomes among unselected patients treated with TAVR is of critical importance. Objective:To exam...
journal_title:JAMA cardiology
pub_type: 杂志文章,多中心研究
doi:10.1001/jamacardio.2016.5302
更新日期:2017-04-01 00:00:00
abstract:IMPORTANCE:Reduced rates of cardiac catheterization, percutaneous coronary intervention (PCI), and coronary artery bypass graft (CABG) are an unintended consequence of public reporting of cardiogenic shock outcomes in New York. OBJECTIVES:To evaluate whether the referral rates for cardiac catheterization, PCI, or CABG...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2016.0785
更新日期:2016-09-01 00:00:00
abstract:Importance:Somatic mutations causing clonal expansion of hematopoietic cells (clonal hematopoiesis of indeterminate potential [CHIP]) are increased with age and associated with atherosclerosis and inflammation. Age and inflammation are the major risk factors for heart failure, yet the association of CHIP with heart fai...
journal_title:JAMA cardiology
pub_type: 临床试验,杂志文章
doi:10.1001/jamacardio.2018.3965
更新日期:2019-01-01 00:00:00
abstract:Importance:Cardiac biomarkers provide insights into pathophysiologic processes and offer an attractive strategy for the assessment of cardiovascular risk. Objective:To assess the incremental prognostic value of biomarkers that reflect different pathophysiologic processes in patients with type 2 diabetes. Design, Sett...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2016.3030
更新日期:2016-12-01 00:00:00
abstract::P values and hypothesis testing methods are frequently misused in clinical research. Much of this misuse appears to be owing to the widespread, mistaken belief that they provide simple, reliable, and objective triage tools for separating the true and important from the untrue or unimportant. The primary focus in inter...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2016.3312
更新日期:2016-12-01 00:00:00
abstract:Importance:Recent data support percutaneous revascularization as an alternative to coronary artery bypass grafting in unprotected left main (ULM) coronary lesions. However, the relevance of these trials to current practice is unclear, as patterns and outcomes of ULM percutaneous coronary intervention (PCI) in contempor...
journal_title:JAMA cardiology
pub_type: 杂志文章,多中心研究
doi:10.1001/jamacardio.2018.4376
更新日期:2019-02-01 00:00:00
abstract:Importance:Persons with human immunodeficiency virus (HIV) that is treated with antiretroviral therapy have improved longevity but face an elevated risk of myocardial infarction (MI) due to common MI risk factors and HIV-specific factors. Despite these elevated MI rates, optimal methods to predict MI risks for HIV-infe...
journal_title:JAMA cardiology
pub_type: 杂志文章,多中心研究
doi:10.1001/jamacardio.2016.4494
更新日期:2017-02-01 00:00:00
abstract:Importance:If we assume that women and men exhibit variations of the same fundamental vascular physiology, then conventional analyses of subclinical measures would suggest that women catch up to men by midlife in the extent of potentially important vascular disease. Alternatively, under the assumption that vascular phy...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2019.5306
更新日期:2020-03-01 00:00:00
abstract:Importance:Echocardiographic left ventricular global longitudinal strain (GLS) detects early subclinical ventricular dysfunction and can be used in patients receiving potentially cardiotoxic chemotherapy. A meta-analysis of the prognostic value of GLS for cancer therapy-related cardiac dysfunction (CTRCD) has not been ...
journal_title:JAMA cardiology
pub_type: 杂志文章,meta分析
doi:10.1001/jamacardio.2019.2952
更新日期:2019-10-01 00:00:00
abstract:Importance:Use of transcatheter aortic valve replacement (TAVR) for severe aortic stenosis is growing rapidly. However, to our knowledge, the durability of these prostheses is incompletely defined. Objective:To determine the midterm hemodynamic performance of balloon-expandable transcatheter heart valves. Design, Set...
journal_title:JAMA cardiology
pub_type: 杂志文章,随机对照试验
doi:10.1001/jamacardio.2017.3306
更新日期:2017-11-01 00:00:00
abstract:Importance:Little is known about the influence of comprehensive public health initiatives according to out-of-hospital cardiac arrest (OHCA) location, particularly at home, where resuscitation efforts and outcomes have historically been poor. Objective:To describe temporal trends in bystander cardiopulmonary resuscita...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2017.3471
更新日期:2017-11-01 00:00:00
abstract:Importance:Observational studies have reported an association between low serum vitamin D levels and elevated risk of cardiovascular disease (CVD) events, but such studies cannot prove causation because of possible unmeasured confounding. Objective:We conducted a meta-analysis of randomized clinical trials that tested...
journal_title:JAMA cardiology
pub_type: 杂志文章,meta分析
doi:10.1001/jamacardio.2019.1870
更新日期:2019-08-01 00:00:00
abstract:Importance:Bioresorbable scaffolds were designed to provide clinical benefits after their complete bioresorption. Prior studies demonstrated early risks with the Absorb polymeric bioresorbable vascular scaffold (BVS). Whether this risk profile changes over time during the course of its bioresorption is unknown. Object...
journal_title:JAMA cardiology
pub_type: 杂志文章,meta分析
doi:10.1001/jamacardio.2019.4101
更新日期:2019-12-01 00:00:00
abstract:Importance:One-third of Medicare beneficiaries are enrolled in Medicare Advantage (MA), Medicare's private plan option. Medicare Advantage incentivizes performance on evidence-based care, but limited information exists using reliable clinical data to determine whether this translates into better quality for patients wi...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2019.0007
更新日期:2019-03-01 00:00:00
abstract:Importance:Cardiovascular disease (CVD) is the leading cause of death in the United States, but regional variation within the United States is large. Comparable and consistent state-level measures of total CVD burden and risk factors have not been produced previously. Objective:To quantify and describe levels and tren...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2018.0385
更新日期:2018-05-01 00:00:00
abstract:Importance:Hospitalizations for worsening heart failure (WHF) represent an enormous public health and financial burden, with physicians, health systems, and payers placing increasing emphasis on hospitalization prevention. In addition, maximizing time out of the hospital is an important patient-centered outcome. In thi...
journal_title:JAMA cardiology
pub_type: 杂志文章
doi:10.1001/jamacardio.2017.5250
更新日期:2018-03-01 00:00:00