Pediatric-Onset Arrhythmogenic Cardiomyopathy
نویسندگان
چکیده
منابع مشابه
Arrhythmogenic cardiomyopathy
Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed. It is a cell-to-cell junction cardiomyopathy, typically caused by genetically determined abno...
متن کاملArrhythmogenic Right Ventricular Cardiomyopathy
Arrythmogenic right ventricular (RV) cardiomyopathy (ARVC) is a cardiomyopathy characterized pathologically by fibrofatty replacement primarily of the RV and clinically by life-threatening ventricular arrhythmias in apparently healthy young people. The prevalence of the disease has been estimated at 1 in 5,000 individuals, although this estimate will likely increase as awareness of the conditio...
متن کاملArrhythmogenic left ventricular cardiomyopathy.
A 53-year-old white man was identified through family screening as having arrhythmogenic right ventricular cardiomyopathy caused by a frameshift mutation in the desmoplakin gene (DSP S1015fsX1017). Direct questioning revealed a previously undisclosed history of palpitations. The electrocardiogram (ECG) showed anterolateral T-wave inversion (Figure 1). Holter monitoring detected an asymptomatic ...
متن کاملLeft-Dominant Arrhythmogenic Cardiomyopathy
Background In the absence of coronary disease and left ventricular (LV) systolic dysfunction, lateral T-wave inversion and arrhythmia of LV origin are often considered benign. Similarly, chest pain with enzyme release might be attributed to viral myocarditis. We hypothesized that these abnormalities might be manifestations of the “left-dominant” subtype of arrhythmogenic right ventricular cardi...
متن کاملArrhythmogenic right ventricular cardiomyopathy.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) has evolved from postmortem pathology at to a diagnosable clinical condition, and holds promise for definitive genetic diagnosis. Its prevalence is between 1/1,000 and 1/5,000, with 10% of deaths occurring before age 19 and 50% before age 35. When analyzed against age-specific norms, the electrocardiography (ECG) and signal-averaged ECG (SA...
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ژورنال
عنوان ژورنال: Journal of the American College of Cardiology
سال: 2019
ISSN: 0735-1097
DOI: 10.1016/j.jacc.2019.05.023