19 research outputs found

    Impact of revised Task Force Criteria: Distinguishing the athlete's heart from ARVC/D using cardiac magnetic resonance imaging

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    Background: Cardiac magnetic resonance (CMR) evaluation of athletes for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is complicated by overlapping features such as right ventricular (RV) volume increase. The revised ARVC/D diagnostic Task Force Criteria (TFC) incorporate cut-off values for RV ejection fraction (EF) and RV end-diastolic volume (EDV) on CMR.Design: To distinguish ARVC/D patients from athletes we compared CMR ventricular volumes, function, TFC cut-off values, and LV/RV ratios since athletes show proportionate, and ARVC/D patients disproportionate, changes in LV and RV.Methods: Quantitative CMR parameters of 33 ARVC/D patients (64% male, mean age 45.4 years, diagnosed by revised TFC), 66 healthy athletes and 66 healthy non-athletes (sex and age matched) were compared using revised TFC and new cut-off values representing LV/RV balance.Results and conclusions: Absolute values for ARVC/D patients/athletes/non-athletes were: in males, RV EDV 149/133/106 ml/m2, ratio EDV LV/RV 0.70/0.91/0.93, RV EF 34/52/54%, LV EF 48/57/58%, ratio EF LV/RV 1.49/1.10/1.09; and in females, RV EDV 115/115/91 ml/m2, ratio EDV LV/RV 0.86/0.94/0.97, RV EF 43/54/58%, LV EF 52/57/61%, ratio EF LV/RV 1.23/1.08/1.04 (p-values < 0.05). Areas under the ROC-curve are 0.68 (RV EDV index), 0.84 (LV/RV EDV ratio) and 0.93 (RV EF), demonstrating significantly (p < 0.001) better performance of RV EF and LV/RV EDV ratio. If a wall motion abnormality is present (observed in 30 ARVC/D patients and not in healthy subjects), RV EF can help distinguish ARVC/D from physiological cardiac adaptation in athletes on CMR whereas RV EDV index cannot. A good alternative in athletes is the LV/RV EDV ratio, representing normal proportionate adaptation of both ventricles

    Europe

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    The foregoing conclusions are broadly consistent with those expressed in the IPCC Special Report on Regional Impacts of Climate Change (1998) and the Second Assessment Report(1996). This survey incorporates much more information than previously reported, corroborating previous conclusions (with which it is broadly consistent) but extending knowledge into other sectors. It is more specific about subregional effects and includes new information concerning adaptive capacity

    Genotype-phenotype analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy:Follow-up of a large series of dutch index-patients and family members

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    Background: In Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) causative mutations in genes encoding 5 desmosomal proteins or TMEM43 are found in the majority of patients. One of the primary clinical challenges in ARVD/C is timely diagnosis of those still asymptomatic. However, previous studies mainly involved overt ARVD/C index-patients. Follow-up data on relatives are scarce. Therefore, we sequenced all 6 genes in a large cohort of ARVD/C families and correlated results with clinical follow-up. Methods: 149 ARVD/C index-patients (111 men, age 49±13 years) according to 2010 Task Force Criteria (TFC) and 302 family members from 93 different families (282 asymptomatic, 135 men, age 44±13 years) were clinically and genetically analyzed. DNA analysis comprised sequencing of PKP2, DSC2, DSG2, DSP, JUP and TMEM43 and multiple ligation-dependent probe amplification (MLPA) to identify large PKP2 deletions. Results: Pathogenic mutations were found in 87 of 149 index-patients (58%): 90% PKP2 and multiple mutations in 4 cases. MLPA revealed 3 large PKP2 deletions (2%). Mutation carriers presented at younger age than non-carriers (35±12 vs 40±14 years; p=0.042). Familial cases were identified in 42 of 93 (45%) of index-patients with relatives screened: 90% with mutations. In total, 57 of 282 asymptomatic relatives (20%) showed signs of ARVD/C (age 47±18 years, 48 with mutations). See table 2. Terminal activation duration (TAD) 7ge;55ms occurred more than negative T waves in V1-3 (12 vs 7%), especially in those age
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