The original designation of “Arrhythmogenic right ventricular (dysplasia/) cardiomyopathy”(ARVC) was used by the scientists who first discovered the disease, in the pre-genetic and pre-cardiac ...magnetic resonance era, to describe a new heart muscle disease predominantly affecting the right ventricle, whose cardinal clinical manifestation was the occurrence of malignant ventricular arrhythmias. Subsequently, autopsy investigations, genotype-phenotype correlations studies and the increasing use of contrast-enhancement cardiac magnetic resonance showed that the fibro-fatty replacement of the myocardium represents the distinctive phenotypic feature of the disease that affects the myocardium of both ventricles, with left ventricular involvement which may parallel or exceed the severity of right ventricular involvement. This has led to the new designation of “Arrhythmogenic Cardiomyopathy” (ACM), that represents the evolution of the original term of ARVC. The present International Expert Consensus document proposes an upgrade of the criteria for diagnosis of the entire spectrum of the phenotypic variants of ACM. The proposed “Padua criteria” derive from the diagnostic approach to ACM, which has been developed over 30 years by the multidisciplinary team of basic researchers and clinical cardiologists of the Medical School of the University of Padua. The Padua criteria are a working framework to improve the diagnosis of ACM by introducing new diagnostic criteria regarding tissue characterization findings by contrast-enhanced cardiac magnetic resonance, depolarization/repolarization ECG abnormalities and ventricular arrhythmia features for diagnosis of the left ventricular phenotype. The proposed diagnostic criteria need to be further validated by future clinical studies in large cohorts of patients.
•Fibro-fatty myocardial replacement is the distinctive phenotypic feature of Arrhythmogenic cardiomyopathy (ACM);•ACM can affect both the right and left ventricle;•The 2010 International Task Force diagnostic criteria lacked specific criteria for diagnosis of left-sided variants of ACM;•The proposed Padua diagnostic criteria encompass dominant-right, biventricular and dominant-left ACM.
In 1982 a nationwide program of pre-participation screening including 12-lead electrocardiography (ECG) was launched in Italy. The aim of this article is to examine whether this 25-year screening ...program should be considered a valid and advisable public health strategy. The analysis of data coming from the long-running Italian experience indicates that ECG screening has provided adequate sensitivity and specificity for detection of potentially lethal cardiomyopathy or arrhythmias and has led to substantial reduction of mortality of young competitive athletes by approximately 90%. Screening was feasible thanks to the Italian Health System, which is developed in terms of health care and prevention services, and because of the limited costs of cardiovascular evaluation in the setting of a mass program. On the basis of current scientific evidence the implementation of a mass-screening program aimed to prevent athletic-field sudden cardiac death should be at least carefully considered by public health administrators worldwide.
Abstract Objectives The aim of this study was to assess the impact of sex and different sports on right ventricular (RV) remodeling and compare the derived upper limits with widely used revised Task ...Force (TF) reference values. Background Uncertainties exist regarding the extent and physiological determinants of RV remodeling in highly trained athletes. The issue is important, considering that in athletes RV size occasionally exceeds the cutoff limits proposed to diagnose arrhythmogenic RV cardiomyopathy. Methods A total of 1,009 Olympic athletes (mean age 24 ± 6 years; n = 647 64% males) participating in skill, power, mixed, and endurance sport were evaluated by 2-dimensional echocardiography and Doppler/tissue Doppler imaging. The right ventricular outflow tract (RVOT) in parasternal long-axis (PLAX) and short-axis views, fractional area change, sʹ velocity, and morphological features were assessed. Results Indexed RVOT PLAX was greater in females than in males (15.3 ± 2.2 mm/m2 vs. 14.4 ± 1.9 mm/m2 ; p < 0.001). Both RVOT PLAX and parasternal short-axis view were significantly different among skill, power, mixed, and endurance sports: 14.3 ± 2.1 mm/m2 versus 14.7 ± 1.9 mm/m2 versus 14.0 ± 1.8 mm/m2 versus 15.7 ± 2.2 mm/m2 , respectively (p < 0.001); and 15.2 ± 2.7 mm/m2 versus 15.3 ± 2.4 mm/m2 versus 14.8 ± 2.1 mm/m2 versus 16.2 ± 2.5 mm/m2 , respectively (p < 0.001). The 95th percentile for indexed RVOT PLAX and parasternal short-axis view was 18 mm/m2 and 20 mm/m2 , respectively. Fractional area change and sʹ velocity did not differ among the groups (p = 0.34 for both). RV enlargement compatible with major and minor TF diagnostic criteria for arrhythmogenic RV cardiomyopathy was observed in 41 (4%) and 319 (32%) athletes. A rounded apex was described in 823 (81%) athletes, prominent trabeculations in 378 (37%) athletes, and a prominent/hyperreflective moderator band in 5 (0.5%) athletes. Conclusions RV remodeling occurs in Olympic athletes, with male sex and endurance practice playing the major impact. A significant subset (up to 32%) of athletes exceeds the normal TF limits; therefore, we recommend referring to the 95th percentiles here reported as referral values; alternatively, only major diagnostic TF criteria for arrhythmogenic RV cardiomyopathy may be appropriate.
Sudden cardiac death (SCD) is the leading cause of death in athletes during sport. Whether obtained for screening or diagnostic purposes, an ECG increases the ability to detect underlying ...cardiovascular conditions that may increase the risk for SCD. In most countries, there is a shortage of physician expertise in the interpretation of an athlete's ECG. A critical need exists for physician education in modern ECG interpretation that distinguishes normal physiological adaptations in athletes from abnormal findings suggestive of pathology. On 13-14 February 2012, an international group of experts in sports cardiology and sports medicine convened in Seattle, Washington, to define contemporary standards for ECG interpretation in athletes. The objective of the meeting was to develop a comprehensive training resource to help physicians distinguish normal ECG alterations in athletes from abnormal ECG findings that require additional evaluation for conditions associated with SCD.
The clinical profile and arrhythmic outcome of competitive athletes with isolated nonischemic left ventricular (LV) scar as evidenced by contrast-enhanced cardiac magnetic resonance remain to be ...elucidated.
We compared 35 athletes (80% men, age: 14-48 years) with ventricular arrhythmias and isolated LV subepicardial/midmyocardial late gadolinium enhancement (LGE) on contrast-enhanced cardiac magnetic resonance (group A) with 38 athletes with ventricular arrhythmias and no LGE (group B) and 40 healthy control athletes (group C). A stria LGE pattern with subepicardial/midmyocardial distribution, mostly involving the lateral LV wall, was found in 27 (77%) of group A versus 0 controls (group C; P<0.001), whereas a spotty pattern of LGE localized at the junction of the right ventricle to the septum was respectively observed in 11 (31%) versus 10 (25%; P=0.52). All athletes with stria pattern showed ventricular arrhythmias with a predominant right bundle branch block morphology, 13 of 27 (48%) showed ECG repolarization abnormalities, and 5 of 27 (19%) showed echocardiographic hypokinesis of the lateral LV wall. The majority of athletes with no or spotty LGE pattern had ventricular arrhythmias with a predominant left bundle branch block morphology and no ECG or echocardiographic abnormalities. During a follow-up of 38±25 months, 6 of 27 (22%) athletes with stria pattern experienced malignant arrhythmic events such as appropriate implantable cardiac defibrillator shock (n=4), sustained ventricular tachycardia (n=1), or sudden death (n=1), compared with none of athletes with no or LGE spotty pattern and controls.
Isolated nonischemic LV LGE with a stria pattern may be associated with life-threatening arrhythmias and sudden death in the athlete. Because of its subepicardial/midmyocardial location, LV scar is often not detected by echocardiography.
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