ライフサイエンスコーパス: ARVC

1 ARVC desmosomal mutation carriers (n = 84) were evaluate

2 ARVC diagnosis (categories: definite, borderline, possib

3 ARVC is a potentially lethal genetic cardiovascular diso

4 ARVC is a primary disease of the myocardium characterize

5 ARVC is characterized pathologically by fibrofatty infil

6 ARVC was diagnosed in 35 patients at initial evaluation

7 ARVC-derived desmoplakin mutants DSP-1-V30M and DSP-1-S2

8 ardium and skeletal muscle in 171 of the 175 ARVC individuals and in controls with noninflammatory ca

9 on could abolish all Epi-LAVA in 4 ICM and 2 ARVC patients, whereas all patients with NICM required e

10 Among 70 patients who fulfilled the 2010 ARVC/D Revised Task Force Criteria and underwent baselin

11 In 21 (91%) of 23 ARVC patients, RV EVM was abnormal, with a total of 45 e

12 3' untranslated region were sequenced in 38 ARVC families.

13 We studied: 42 ARVC probands, 23 male, aged 42, interquartile range 33-

14 n=38), borderline (n=39), or possible (n=43) ARVC.

15 s: CPVT, 9 (53%) of 17; BrS, 16 (33%) of 49; ARVC, 9 (25%) of 36; LQTS, 48 (20%) of 238; dilated card

16 in 15 (83%) ICM, 2 (13%) NICM, and 11 (73%) ARVC patients, contributing to a potential reduction in

17 ns (ICM: 20 of 71 [28%], NICM: 3 of 39 [8%], ARVC: 25 of 63 [40%]) successfully eliminated the Epi-LA

18 Recently, a ARVC risk score was proposed to predict the 5-year risk

19 Eighty-two additional ARVC cases were obtained from published reports, and add

20 FC are not met by the majority of adolescent ARVC patients, particularly when indexed to body surface

21 (P=0.013) than those who developed HF after ARVC/D presentation.

22 All ARVC samples but no control samples showed a marked redu

23 ardiomyopathy established the North American ARVC Registry and enrolled patients with a diagnosis of

24 In the North American ARVC Registry, the majority of ventricular arrhythmias i

25 lators (ICDs) enrolled in the North American ARVC Registry.

26 The overall yield of mutations among ARVC cases was 58% versus 16% in controls.

27 ical from mechanical tissue substrates among ARVC clinical stages.

28 ears old at last encounter; 33% male) had an ARVC variant (G(+)).

29 In addition, a family member may have an ARVC gene defect and have development of the disease or

30 ate ARVC gene because of its proximity to an ARVC locus at position 2q32 and the connection of the ti

31 utation and endurance training to trigger an ARVC-like phenotype.

32 (n = 45), athletes without TWI (n = 35), and ARVC patients (n = 35).

33 g inherited cardiac disorders in general and ARVC specifically.

34 lly compared in 60 subjects: 30 asymptomatic ARVC gene carriers and 30 healthy controls.

35 latent electrical substrate in asymptomatic ARVC gene carriers that is shared by patients with ARVC

36 itizing medical surveillance in asymptomatic ARVC gene carriers.

37 Management of asymptomatic ARVC gene carriers is challenging because of variable pe

38 Patients with clinical HF before ARVC/D diagnosis (n=31) were older (P=0.005) and met few

39 Our data suggest that buried ARVC mutations destabilize desmoplakin and thereby impai

40 as Naxos disease, which is characterized by ARVC and a cutaneous disorder.

41 a cardiocutaneous syndrome characterized by ARVC and abnormalities of hair and skin.

42 rdiomyopathy gene titin (TTN) as a candidate ARVC gene because of its proximity to an ARVC locus at p

43 right ventricular dysplasia/cardiomyopathy (ARVC) is a genetic disease caused by mutations in desmos

44 right ventricular dysplasia/cardiomyopathy (ARVC), in which the right ventricle is "replaced" by fib

45 imaging in arrhythmogenic RV cardiomyopathy (ARVC) may be inadequate because of the complex contracti

46 ythmogenic right ventricular cardiomyopathy (ARVC) (13%); and hypertrophic cardiomyopathy (HCM) (6%).

47 ythmogenic right ventricular cardiomyopathy (ARVC) (n = 9 [8%]), and dilated cardiomyopathy (n = 5 [4

48 ythmogenic right ventricular cardiomyopathy (ARVC) can be challenging because the clinical presentati

49 ythmogenic right ventricular cardiomyopathy (ARVC) has a prevalence of at least 1 in 1000, is a leadi

50 ythmogenic right ventricular cardiomyopathy (ARVC) has been suggested, the arrhythmogenic substrate f

51 ythmogenic right ventricular cardiomyopathy (ARVC) have recently been updated, the diagnosis remains

52 ythmogenic right ventricular cardiomyopathy (ARVC) in adolescence is not well established.

53 ythmogenic right ventricular cardiomyopathy (ARVC) in asymptomatic gene carriers.

54 ythmogenic right ventricular cardiomyopathy (ARVC) is a familial heart disease linked to mutations in

55 ythmogenic right ventricular cardiomyopathy (ARVC) is a genetically heterogeneous condition caused by

56 ythmogenic right ventricular cardiomyopathy (ARVC) is a genetically transmitted disease.

57 Arrhythmic right ventricular cardiomyopathy (ARVC) is a hereditary heart muscle disease that causes s

58 ythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death, but it

59 ythmogenic right ventricular cardiomyopathy (ARVC) is a phenotype caused by mutations in desmosomal c

60 ythmogenic right ventricular cardiomyopathy (ARVC) is a primary heart muscle disorder resulting from

61 ythmogenic right ventricular cardiomyopathy (ARVC) is a significant cause of sudden cardiac death in

62 ythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable myocardial disorder associated w

63 ythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease characterized by f

64 ythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disorder mainly caused by

65 ythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ve

66 ythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy, which is associate

67 ythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disorder associated with arrhythmi

68 ythmogenic right ventricular cardiomyopathy (ARVC) is an inherited genetic myocardial disease charact

69 ythmogenic right ventricular cardiomyopathy (ARVC) is associated with pathogenic/likely pathogenic (P

70 ythmogenic right ventricular cardiomyopathy (ARVC) is associated with sudden cardiac death.

71 ythmogenic right ventricular cardiomyopathy (ARVC) is associated with variants in desmosome genes.

72 ythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) an

73 ythmogenic right ventricular cardiomyopathy (ARVC) is fibrofatty scar replacement.

74 ythmogenic right ventricular cardiomyopathy (ARVC) patients.

75 ythmogenic right ventricular cardiomyopathy (ARVC) patients.

76 ythmogenic right ventricular cardiomyopathy (ARVC) phenotype manifestation; however, research is hamp

77 ythmogenic right ventricular cardiomyopathy (ARVC), with skin manifestations, has been associated wit

78 ythmogenic right ventricular cardiomyopathy (ARVC).

79 ythmogenic right ventricular cardiomyopathy (ARVC).

80 ythmogenic right ventricular cardiomyopathy (ARVC).

81 ythmogenic right ventricular cardiomyopathy (ARVC).

82 ythmogenic right ventricular cardiomyopathy (ARVC).

83 ythmogenic right ventricular cardiomyopathy (ARVC).

84 ythmogenic right ventricular cardiomyopathy (ARVC).

85 ythmogenic right ventricular cardiomyopathy (ARVC).

86 ythmogenic right ventricular cardiomyopathy [ARVC]) with sustained VT underwent combined endo- and ep

87 ost aggressive arrhythmogenic cardiomyopathy/ARVC subtype is ARVC type 5 (ARVC5), caused by a p.S358L

88 g intense exertion occurred in 61% of cases; ARVC and left ventricular fibrosis most strongly predict

89 vide evidence that titin mutations can cause ARVC, a finding that further expands the origin of the d

90 the desmosomal plakophilin-2 gene can cause ARVC.

91 nd desmocollin 2 (DSC2), respectively, cause ARVC.

92 WT)) or a truncated (PG(TR)), known to cause ARVC, in the heart; and PG null (PG(-)/(-)) embryos.

93 cardiac desmosomes, have been shown to cause ARVC.

94 In a cohort of well-characterized ARVC subjects, neither beta-blockers nor sotalol seemed

95 We studied 23 consecutive ARVC patients (16 males; mean age, 38+/-12 years) who un

96 an age 36 years [22-52]) from 44 consecutive ARVC families undergoing comprehensive genetic screening

97 We studied 69 consecutive ARVC/D patients (47 males; median age 35 years [28-45])

98 Compared with 20 healthy controls, ARVC patients had longer ventricular activation duration

99 Definite ARVC or post-inflammatory cardiomyopathy was diagnosed i

100 and together with JUP mutation met definite ARVC diagnosis.

101 assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe.

102 ients with possible, borderline, or definite ARVC compared with controls for parasternal long-axis vi

103 ignificantly lower in patients with definite ARVC (-21+/-4%) and disease subgroups versus controls (-

104 A total of 864 patients with definite ARVC (40+/-16 years; 53% male) were included.

105 e of CMR phenotype in patients with definite ARVC and to evaluate the effectiveness of the novel 5-ye

106 diagnosis in 10 of 11 subjects with definite ARVC on the basis of clinical criteria and correctly rul

107 A total of 140 patients with definite ARVC were enrolled (mean age 42 +/- 17 years, 97 males)

108 leven of the 23 patients (48%) with definite ARVC would not have been in this group if CMR had not be

109 ts, respectively, all of whom had definitive ARVC.

110 into mice resulted in an exercise-dependent ARVC phenotype.

111 values of isoproterenol testing to diagnose ARVC were 91.4%, 88.9%, 43.2%, and 99.1%, respectively.

112 of 8.5% of RV free wall was used to diagnose ARVC with 94% sensitivity (95% confidence interval [CI]:

113 EAM-guided biopsy diagnosed ARVC in 11 (46%), myocarditis in 8 (33%), and idiopathic

114 amined the contribution of CMR to diagnosing ARVC using the rTFC in a pediatric population.

115 lement of the rTFC contributed to diagnosing ARVC was determined using a c-statistics model.

116 Different ARVC stages were characterized by distinct RV deformatio

117 Among the many documented ARVC-related genetic variants, a striking hotspot of nin

118 ost likely cause for the genesis of dominant ARVC due to mutations in PKP2.

119 right ventricular cardiomyopathy/dysplasia (ARVC) genetic test and to determine genetic associations

120 right ventricular cardiomyopathy/dysplasia (ARVC/D) is controversial.

121 right ventricular cardiomyopathy/dysplasia (ARVC/D) that facilitated recognition and interpretation

122 right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well

123 right ventricular cardiomyopathy/dysplasia (ARVC/D).

124 al RV deformation has been reported in early ARVC without structural abnormalities.

125 ocardiograms, manually over-read to evaluate ARVC diagnostic criteria, and performed a PheWAS (phenom

126 hout the proposed modifications for familial ARVC; and 2) gene-carrier status in 35 individuals from

127 with ATWI fulfilled diagnostic criteria for ARVC after further evaluation.

128 Diagnostic criteria for ARVC are nonspecific in such individuals.

129 ients with no or only minor CMR criteria for ARVC diagnosis.

130 sess the accuracy of diagnostic criteria for ARVC when applied to athletes exhibiting electrocardiogr

131 itional patients met diagnostic criteria for ARVC.

132 k Force and modified diagnostic criteria for ARVC.

133 more accurate set of diagnostic criteria for ARVC.

134 the diagnostic yield of echocardiography for ARVC.

135 dies of 142 pediatric patients evaluated for ARVC between 2005 and 2009 were reviewed.

136 ograms from 120 adolescents investigated for ARVC (13+/-4 years) were retrospectively analyzed.

137 ed plakoglobin signal level was specific for ARVC, we analyzed myocardium from 15 subjects with hyper

138 a sensitive and specific diagnostic test for ARVC, we performed immunohistochemical analysis of human

139 y sensitive and specific diagnostic test for ARVC.

140 Survival free from ARVC diagnosis was significantly lower in the positive i

141 rcise-induced arrhythmogenic remodeling from ARVC and post-inflammatory cardiomyopathies.

142 rcise-induced arrhythmogenic remodeling from ARVC and post-inflammatory cardiomyopathy.

143 METHODS AND We studied 20 genotyped ARVC patients with a broad spectrum of disease using ele

144 Hence, ARVC is the first identified disease of disrupted differ

145 heart-biopsy samples from the Johns Hopkins ARVC registry.

146 itulated the clinical manifestation of human ARVC: ventricular dilation and aneurysm, cardiac fibrosi

147 scs in cardiomyocytes from four of six human ARVC cases show reduced or loss of iASPP.

148 ical relevance of an incidentally identified ARVC loss-of-function variant.

149 Genetic reclassification impacted ARVC diagnosis.

150 In ARVC, 5 causative desmosomal genes have been identified,

151 Fat extent was 16.5% +/- 6.1 in ARVC and 4.6% +/- 2.7 in non-ARVC (P < .0001).

152 The findings suggest that adipocytes in ARVC, at least in part, originate from c-Kit+ CPCs.

153 oimmunity, we searched for AHAs and AIDAs in ARVC.

154 rotecting against ventricular arrhythmias in ARVC, but no studies have provided data in a group rigor

155 of RV strain and dyssynchrony assessment in ARVC using feature-tracking CMR analysis.

156 ture ventricular contractions were common in ARVC patients with variable initiation sites in both ven

157 especially exertional dyspnea, are common in ARVC/D; yet, classic left-sided signs are typically abse

158 ological substrate properties that differ in ARVC patients compared with healthy controls.

159 globin signal level was reduced diffusely in ARVC samples, including those obtained in the left ventr

160 t a biologically plausible candidate gene in ARVC pathogenesis.

161 determine prevalence and predictors of HF in ARVC/D.

162 sonance (CE-CMR) for imaging scar lesions in ARVC patients.

163 powerful predictor of arrhythmic outcome in ARVC/D, independently of history and RV dilatation/dysfu

164 To investigate the role of plakoglobin in ARVC, we generated an inducible cardiorestricted knockou

165 stic value of beta-adrenergic stimulation in ARVC.

166 genes for arrhythmic risk stratification in ARVC.

167 minant involvement of the right ventricle in ARVC.

168 rrhythmogenic cardiomyopathy/ARVC subtype is ARVC type 5 (ARVC5), caused by a p.S358L mutation in TME

169 nds tested previously for mutations in known ARVC genes and found an additional likely pathogenic var

170 relatives or if they be a result of manifest ARVC.

171 ctively adjudicated for 289 patients meeting ARVC/D Task Force Criteria.

172 ow identification of RV scar lesions in most ARVC patients.

173 , with posterolateral RV wall being the most ARVC-specific area.

174 tic substrates by studying genotype-negative ARVC families.

175 e subsequently screened 73 genotype-negative ARVC probands tested previously for mutations in known A

176 s had definite, borderline, possible, and no ARVC, respectively, applying the rTFC.

177 finitive," "borderline," "possible," or "no" ARVC diagnostic groups based on the rTFC.

178 6.5% +/- 6.1 in ARVC and 4.6% +/- 2.7 in non-ARVC (P < .0001).

179 enic heterozygosity was identified in 16% of ARVC-causing desmosomal gene mutation carriers and was a

180 d missense mutations versus a similar 21% of ARVC cases.

181 by 0.5% of control individuals versus 43% of ARVC cases, while 16% of controls hosted missense mutati

182 rrect identification of 88%, 75%, and 63% of ARVC patients with no or only minor CMR criteria for ARV

183 Improved noninvasive assessment of ARVC and better understanding of the disease substrate a

184 The strong association of ARVC and left ventricular fibrosis with exercise-induced

185 These data suggest that the genetic basis of ARVC includes reduced penetrance with compound and digen

186 ent of the titin spring is a likely cause of ARVC and constitutes a novel mechanism underlying myocar

187 te CDH2 mutations as novel genetic causes of ARVC and contribute to a more complete identification of

188 The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement.

189 dy sought to describe the clinical course of ARVC and occurrence of life-threatening arrhythmic event

190 tions should be monitored for development of ARVC.

191 are important contributors to a diagnosis of ARVC in children, using the rTFC.

192 None had an existing diagnosis of ARVC in the electronic health record, nor significant di

193 Timely diagnosis of ARVC is difficult as clinical findings may be subtle and

194 Once the diagnosis of ARVC is established, the single most important clinical

195 in strenuous exercise after the diagnosis of ARVC, hemodynamically tolerated sustained monomorphic ve

196 ve (sensitivity, 91.4%) for the diagnosis of ARVC, particularly in its early stages.

197 ms better than RV volume in the diagnosis of ARVC.

198 ry and enrolled patients with a diagnosis of ARVC.

199 is of utmost importance in the diagnosis of ARVC.

200 the right ventricle.Phenotypic expression of ARVC is variable and a significant number of patients ma

201 sudden cardiac death, displaying features of ARVC.

202 frequently nonspecific clinical features of ARVC/D.

203 c modalities and advances in the genetics of ARVC/D.

204 Almost half of ARVC patients have a mutation in genes encoding cell adh

205 The phenotypic hallmark of ARVC is fibroadipocytic replacement of cardiac myocytes,

206 difference that increased with likelihood of ARVC.

207 This novel model of ARVC demonstrates for the first time how plakoglobin aff

208 duced pluripotent stem cell (hiPSC) model of ARVC.

209 ded further insights for the pathogenesis of ARVC and potential therapeutic interventions.

210 n molecular genetics and the pathogenesis of ARVC could afford the opportunity for a genetic-based di

211 dysfunction involved in the pathogenesis of ARVC remain poorly understood.

212 w molecular insight into the pathogenesis of ARVC.

213 tein plakoglobin (PG) in the pathogenesis of ARVC.

214 esponsible mechanism for the pathogenesis of ARVC.

215 molecular mechanisms in the pathogenesis of ARVC.

216 have been implicated in the pathogenesis of ARVC.

217 ns of this pathway to the pathophysiology of ARVC, not only through perturbation of cardiac patternin

218 ings highlighted age-dependent penetrance of ARVC.

219 were present in the early concealed phase of ARVC.

220 Different CMR presentations of ARVC are associated with different prognoses.

221 The prevalence of ARVC loss-of-function variants is ~1:435 in a general cl

222 lar EVM characterized a low-risk subgroup of ARVC/D patients.

223 A subset of ARVC is categorized as Naxos disease, which is character

224 and cardiac dysfunction similar to those of ARVC patients.

225 early diagnosis and noninvasive follow-up of ARVC patients.

226 is study evaluated the impact of exercise on ARVC cardiac manifestations in mice after adeno-associat

227 tients with ARVC and to assess the impact on ARVC diagnosis.

228 The p.C796R and other ARVC-related PKP2 mutations indicate loss of function ef

229 of clinical criteria and correctly ruled out ARVC in 10 of 11 subjects without ARVC, for a sensitivit

230 vidence of cardiac disease, but met possible ARVC diagnosis with one major criterion (the JUP mutatio

231 e needed to clarify whether they may predict ARVC development in healthy relatives or if they be a re

232 Radical mutations are high-probability ARVC-associated mutations, whereas rare missense mutatio

233 rdiac death (SCD) in desmosomal gene-related ARVC.

234 important pathogenic pathway for Jup-related ARVC.

235 sults may not be generalizable to lower-risk ARVC/D cohorts.

236 y of familial and in almost half of sporadic ARVC.

237 Subclinical and electrical staged ARVC subjects already showed signs of local mechanical a

238 nt definitions of electrical and subclinical ARVC stages.

239 patients undergoing evaluation for suspected ARVC.

240 ricular contractions evaluation or suspected ARVC.

241 ound to be high in patients with symptomatic ARVC: new epsilon waves appeared in 3 of 18 (17%), super

242 ects with desmosomal mutations confirms that ARVC is a disease of the desmosome and cell junction.

243 ance of this decision reflects the fact that ARVC is a common cause of sudden death in young people a

244 Genetic variation among the ARVC susceptibility genes has not been systematically ex

245 enetic variation in healthy controls for the ARVC susceptibility genes.

246 ers of lipid droplets were identified in the ARVC-CMs that displayed the more severe desmosomal patho

247 fied widened and distorted desmosomes in the ARVC-hiPSC-CMs.

248 nt decrease in the expression of PKP2 in the ARVC-hiPSC-CMs.

249 d prolonged field potential rise time in the ARVC-hiPSC-CMs.

250 SC-CMs were demonstrated to recapitulate the ARVC phenotype in the dish, provide mechanistic insights

251 d in clear RV dysfunction that resembled the ARVC phenotype (impaired global RV systolic function and

252 rol subjects, matched for age and sex to the ARVC group, were included for comparison purpose.

253 out the background noise associated with the ARVC genetic test.

254 on that showed complete segregation with the ARVC phenotype in 1 large family.

255 Thus, ARVC, at least in a subset, is a disease of desmosomes.

256 RV strain was significantly associated with ARVC diagnosis and its likelihood (multivariable odds ra

257 n TMEM43 and TGFB1 have been associated with ARVC.

258 to the specificity of their association with ARVC/D.

259 When athletes were compared with ARVC patients, markers of physiological remodeling inclu

260 .686A>C, p.Gln229Pro) that cosegregated with ARVC in affected family members.

261 e sequencing was performed on 2 cousins with ARVC.

262 ere genotyped for 93 probands diagnosed with ARVC from the Netherlands and 427 ostensibly healthy con

263 At that time, clinical experience with ARVC/D was dominated by symptomatic index cases and sudd

264 encoding plakoglobin in a German family with ARVC but no cutaneous abnormalities.

265 Thirty-six patients with ARVC (mean age +/- standard deviation, 46 years +/- 15;

266 s positive in 32 of 35 (91.4%) patients with ARVC and in 42 of 377 (11.1%) patients without ARVC (P<0

267 unction and RV dyssynchrony in patients with ARVC and provides incremental value over conventional ci

268 distribution differed between patients with ARVC and those without, with posterolateral RV wall bein

269 reclassify genetic variants in patients with ARVC and to assess the impact on ARVC diagnosis.

270 oncerns risk stratification in patients with ARVC and to place this literature in the framework of th

271 LTVA events in patients with ARVC can be predicted by a novel simple prediction model

272 ere significantly higher among patients with ARVC compared with patients with RV outflow tract arrhyt

273 Frequently, patients with ARVC have >1 genetic defect in the same gene (compound h

274 but because only 30% to 50% of patients with ARVC have 1 of these gene abnormalities, it is assumed t

275 Thirty-two consecutive patients with ARVC referred to CMR imaging were included.

276 ife-threatening arrhythmias in patients with ARVC spans from adolescence to advanced age, reaching it

277 ical course of 301 consecutive patients with ARVC using the Kaplan-Meier method adjusted to avoid the

278 f the study, ETT results of 25 patients with ARVC with histories of sustained ventricular arrhythmia

279 ene carriers that is shared by patients with ARVC with histories of ventricular arrhythmia.

280 broblasts were obtained from 2 patients with ARVC with plakophilin-2 (PKP2) mutations, reprogrammed t

281 In patients with ARVC, fat correlated to RV volume (R = 0.63, P < .0001),

282 ant ventricular arrhythmias in patients with ARVC.

283 edict incident sustained VA in patients with ARVC.

284 of ventricular arrhythmias in patients with ARVC.

285 ers and therapeutic targets in patients with ARVC.

286 in unrelated genotype-negative patients with ARVC.

287 time experiences in caring for patients with ARVC.

288 an increased risk for MACE in patients with ARVC/D with advanced disease and a high risk for adverse

289 aphy in risk stratification in patients with ARVC/D, although our results may not be generalizable to

290 first-degree family members of a person with ARVC to have genetic testing but only if there is a know

291 If the affected family member (proband) with ARVC does not have a genetic defect identified, then it

292 N-cadherin were normal in all subjects with ARVC.

293 irst tested myocardium from 11 subjects with ARVC; of these samples, 8 had desmosomal gene mutations.

294 VC and in 42 of 377 (11.1%) patients without ARVC (P<0.0001).

295 ruled out ARVC in 10 of 11 subjects without ARVC, for a sensitivity of 91%, a specificity of 82%, a

296 The 5-year ARVC risk score is valid for the estimation of risk in p

297 aluate the effectiveness of the novel 5-year ARVC risk score to predict cardiac events in different C

298 The novel 5-year ARVC risk score was retrospectively calculated using the

299 ent, a LV-dominant phenotype, and the 5-year ARVC risk score were independent predictors of major eve

300 ncluded patients from the Multicenter Zurich ARVC Registry who hosted a genetic variant deemed to be