ライフサイエンスコーパス: LQTS

1 LQTS and CPVT predominated in those <24 years of age, 30

2 LQTS is a known cause of sudden death in childhood, adol

3 LQTS is commonly genetic in origin but can also be cause

4 LQTS patients display regions with steep repolarization

5 LQTS patients with a QTc>=480 ms (n=120) had a significa

6 teristics of LQTS patients who have had >/=1 LQTS-related breakthrough cardiac event (BCE) after LCSD

7 Q1 (KV7.1, LQTS type 1), KCNH2 (HERG/KV11.1, LQTS type 2), and SCN5A (NaV1.5, LQTS type 3) were perfo

8 hensive mutational analyses of KCNQ1 (KV7.1, LQTS type 1), KCNH2 (HERG/KV11.1, LQTS type 2), and SCN5

9 matic; 51% LQTS type 1; 33% LQTS type 2; 11% LQTS type 3; 5% multiple mutations) and 50 healthy contr

10 Stellate ganglia of all 12 LQTS/CPVT patients revealed mild but distinct inflammato

11 Although 16 LQTS-susceptibility genes have been discovered, 20% to 2

12 We studied 169 LQTS genotype-positive patients < 50 years of age who pe

13 males; 158 symptomatic; 51% LQTS type 1; 33% LQTS type 2; 11% LQTS type 3; 5% multiple mutations) and

14 7; BrS, 16 (33%) of 49; ARVC, 9 (25%) of 36; LQTS, 48 (20%) of 238; dilated cardiomyopathy, 5 (9%) of

15 Among 42 LQTS subjects, 26 were in Group 1 and 16 in Group 2.

16 ERG/KV11.1, LQTS type 2), and SCN5A (NaV1.5, LQTS type 3) were performed using denaturing high-perfor

17 17 years; 60% females; 158 symptomatic; 51% LQTS type 1; 33% LQTS type 2; 11% LQTS type 3; 5% multip

18 hermore, caveolin-3:p.T78M did not exhibit a LQTS phenotype.

19 Time to a LQTS-associated cardiac event was analyzed using Cox pro

20 nital LQTS but could have a form of acquired LQTS.

21 lar fibrillation is diagnosed with "acquired LQTS" and is discharged with no therapy other than instr

22 Members of a South African LQTS-type 1 founder population (181 noncarriers and 168

23 ge, have therapeutic implications for ageing LQTS patients.

24 As a group, all LQTS-associated CaM mutants (LQTS-CaMs) exhibited reduce

25 AKAP9 has been identified as an LQTS-type 1-modifying gene.

26 , 556 (92%) patients have not experienced an LQTS-triggered BCE.

27 Although the remaining 8 subjects had an LQTS phenotype, evidence suggested that the KCNE2 varian

28 fest the same rhythm after birth and have an LQTS mutation.

29 pathogenic mutations, and 10 did not have an LQTS phenotype.

30 In cases with an LQTS gene mutation, SUDEP may occur as a result of a pre

31 rence-in-means+/-SE: 2.1+/-0.7, P<0.002) and LQTS genotype-positive family members (87.5+/-7.4, diffe

32 nsic ligand affected hERG channel gating and LQTS mutations abolished hERG currents and altered traff

33 In LQTS type 2 (LQT2) and LQTS type 3 (LQT3), T-wave alternans was observed follow

34 FHR varies by GA in both normal and LQTS fetuses.

35 corrected QT interval (QTc), female sex, and LQTS genotype as univariate predictors of symptomatic st

36 ived from patients with LEOPARD syndrome and LQTS has shed light on the molecular mechanisms of disea

37 candidate genes for ventricular arrhythmias, LQTS and SCD.

38 hrough cardiac events (BCEs) were defined as LQTS-attributable syncope or seizures, aborted cardiac a

39 aps all, have been erroneously designated as LQTS-causative mutations.

40 ed as having limited or disputed evidence as LQTS-causative genes.

41 tic LQTS compared to those with asymptomatic LQTS (-52 +/- 38 ms vs. -18 +/- 29 ms; p < 0.0001).

42 g CaM expression and potentially attenuating LQTS-triggered cardiac events, thus initiating a path to

43 patients from the Rochester, New York-based LQTS Registry who were prescribed common beta-blockers (

44 e no association has ever been found between LQTS and isolated CAV3 mutations, we suggest that LQTS9

45 Detailed correlative studies between LQTS mutations and clinical phenotypes are leading the f

46 nd 2 subjects with clinical features of both LQTS and CPVT (p.D132E, p.Q136P).

47 ve analysis program in defining breakthrough LQTS arrhythmic risk beyond the QTc value.

48 45 were nonetheless diagnosed as affected by LQTS based on unequivocal ECG abnormalities (QTc, 472+/-

49 l of evidence for 17 genes reported to cause LQTS.

50 e than half of the genes reported as causing LQTS have limited or disputed evidence to support their

51 A1C) had moderate level evidence for causing LQTS.

52 attributed to LQTS, although the most common LQTS rhythm, a fetal heart rate of less than third perce

53 tinguished 83.33% of patients with concealed LQTS from controls, despite having essentially identical

54 idual from a large pedigree with concomitant LQTS, HCM, and congenital heart defects and identified a

55 netism Laboratory for fetuses with confirmed LQTS.

56 ced long QT syndrome (diLQTS) and congenital LQTS (cLQTS) share many features, and both syndromes can

57 y were considered not affected by congenital LQTS and are henceforth referred to as "cases." Furtherm

58 e individuals are not affected by congenital LQTS but could have a form of acquired LQTS.

59 In contrast, LQTS-CaMs did not promote Ca waves and exhibited either

60 phological analysis program to differentiate LQTS patients from healthy controls.

61 eatment intensification following documented LQTS-associated breakthrough cardiac events while on bet

62 romic, genotype-negative, autosomal dominant LQTS in a multigenerational pedigree, and we established

63 erturbations may underlie autosomal dominant LQTS in the absence of Timothy syndrome.

64 l, genetic variants leading to dysfunctional LQTS-associated ion channels in vitro were discovered in

65 ol/L; mean age, 23.4+/-17 years) with either LQTS (n=8) or CPVT (n=4) and serious arrhythmias.

66 mon cardiac channelopathy with 15 elucidated LQTS-susceptibility genes.

67 Thirty-eight genetically elusive LQTS cases underwent whole-exome sequencing to identify

68 Overall, 13% of our genetically elusive LQTS cohort harbored nonsynonymous variants in CaM.

69 variants in a cohort of genetically elusive LQTS, and functionally characterize the novel variants.

70 unrelated patients with genetically elusive LQTS.

71 This study sought to evaluate LQTS outcomes from a single center in the contemporary e

72 We ascertained fetal LQTS subjects by family history (Group 1) or fetal arrhy

73 Records of subjects exhibiting fetal LQTS arrhythmias were reviewed.

74 We studied 43 subjects exhibiting fetal LQTS arrhythmias: TdP+/-2 degrees atrioventricular block

75 Rhythm phenotypes of fetal LQTS have genotype-suggestive features that, along with

76 (FHR)/gestational age (GA) profile of fetal LQTS.

77 -specific data are available regarding fetal LQTS.

78 dycardia has also been associated with fetal LQTS, but little is known of this rhythm manifestation.

79 Of 17 genes reported as being causative for LQTS, 9 (AKAP9, ANK2, CAV3, KCNE1, KCNE2, KCNJ2, KCNJ5,

80 , reappraisal of reported genetic causes for LQTS is required.

81 cardiac mosaicism as a causal mechanism for LQTS and present methods by which the general phenomenon

82 ify a novel underlying genetic mechanism for LQTS.

83 enes have been identified as responsible for LQTS, and elevated risks for EADs may depend on genotype

84 potentially be developed as therapeutics for LQTS and cardiac arrhythmia.

85 ) have emerged as potential therapeutics for LQTS because they are modulators of voltage-gated ion ch

86 eatment options, but no targeted therapy for LQTS exists to date.

87 the 1400 patients evaluated and treated for LQTS, a retrospective review was performed on the 204 pa

88 Two independent predictors of future LQTS-associated cardiac events from the surface ECG were

89 represented significantly in this heretofore LQTS cohort (13.2%) compared with exome aggregation cons

90 the 12 identified genes causal to heritable LQTS, approximately 90% of affected individuals harbor m

91 In LQTS type 2 (LQT2) and LQTS type 3 (LQT3), T-wave altern

92 In LQTS type 2, we observed increasing SMRs starting from a

93 In LQTS type 3, the SMR was increased between age 15 and 19

94 In LQTS, beta-blocker therapy is effective in reducing the

95 ntified only on the day of cardiac arrest in LQTS literature.

96 Adherence to beta-blockers in LQTS is suboptimal in half of those with LQTS 1 and 2.

97 rong or definitive evidence for causality in LQTS with atypical features, including neonatal atrioven

98 fter left cardiac sympathetic denervation in LQTS or catecholaminergic polymorphic ventricular tachyc

99 educing the risk of a first cardiac event in LQTS, their efficacy differed by genotype; nadolol was t

100 to risk of breakthrough arrhythmic events in LQTS, particularly LQT2.

101 -blockers with the risk of cardiac events in LQTS.

102 We compared FHR in LQTS subjects versus normal fetuses.

103 However, in LQTS type 1 (LQT1), once a PVC occurred, it always immed

104 ely a common mechanism for PVT initiation in LQTS.

105 o trigger or enhance electric instability in LQTS/CPVT patients who are already genetically predispos

106 thy subjects and severity of presentation in LQTS.

107 unified therapy for arrhythmia prevention in LQTS.

108 The QTc-PRS in LQTS probands (n=137; 89.3+/-6.8) was significantly grea

109 The average QTc-PRS in LQTS was 88.0+/-7.2 and explained only ~2.0% of the QTc

110 chanisms of spontaneous initiation of PVT in LQTS.

111 fying a fetal proband in Group 2 resulted in LQTS diagnosis in 9 unsuspected members of 6 families.

112 ion, which could pose arrhythmogenic risk in LQTS patients.

113 with polymorphic ventricular tachycardia in LQTS patients.

114 ity of the heart, can be equally variable in LQTS patients, posing well-described diagnostic dilemmas

115 irty-nine fetuses had pathogenic variants in LQTS genes: 27 carried the family variant, 11 had de nov

116 patients with a complex phenotype including LQTS, HCM, and congenital heart defects annotated as car

117 Caucasian patients experiencing drug-induced LQTS (dLQTS) and 87 Caucasian controls from the DARE (Dr

118 otypes of fetuses with de novo and inherited LQTS variants and identify risk factors for sudden death

119 n CaM that is mutated in a form of inherited LQTS.

120 ying genetic basis for recessively inherited LQTS.

121 ing the KCNQ1-A341V mutation and 122 Italian LQTS patients with impaired (I(Ks)-, 66 LQT1) or normal

122 Therefore KCNQ1 LQTS patients may exhibit increased insulin secretion.

123 nts, from six families, diagnosed with KCNQ1 LQTS were individually matched to two randomly chosen BM

124 The phenotype of patients with KCNQ1 LQTS, caused by mutations in KCNQ1, includes, besides lo

125 This study investigates variants in a known LQTS-causative gene, AKAP9, for potential LQTS-type 1-mo

126 Mutations in known LQTS genes were found in 95% of subjects tested.

127 Beta-blockers are the mainstay in managing LQTS.

128 tes from a patient with D130G-CALM2-mediated LQTS, thus creating a platform with which to devise and

129 tes from a patient with D130G-CALM2-mediated LQTS, thus creating a platform with which to devise and

130 CaM and KCNQ1 that may explain CaM-mediated LQTS.

131 As a group, all LQTS-associated CaM mutants (LQTS-CaMs) exhibited reduced Ca affinity, whereas CPVT-a

132 cluding exome analysis, in genotype-negative LQTS probands.

133 ced at least 1 subsequent, albeit nonlethal, LQTS-triggered cardiac event.

134 The malignancy of de novo LQTS variants was remarkably high and demonstrate that t

135 Approximately 20% of LQTS cases remain genetically elusive.

136 ty genes have been discovered, 20% to 25% of LQTS remains genetically elusive.

137 It could distinguish 86.8% of LQTS patients from healthy controls.

138 are unlikely to explain arrhythmogenicity of LQTS-CaM mutations.

139 S, we calculated a bradycardia index as % of LQTS FHR recordings either </=110 beats per minute (obst

140 rcentile for GA may improve ascertainment of LQTS in fetuses, neonates, and undiagnosed family member

141 hift mutations in 4 of 33 unrelated cases of LQTS (12%).

142 assification of these genes for causation of LQTS after assessment of the evidence scored by the inde

143 e sought to determine the characteristics of LQTS patients who have had >/=1 LQTS-related breakthroug

144 d with LQTS and with overlapping features of LQTS and CPVT.

145 The underlying basis of this form of LQTS is a disruption of Ca(2+)/calmodulin (CaM)-dependen

146 strategies for the treatment of this form of LQTS.

147 2) have been associated with severe forms of LQTS and CPVT, with life-threatening arrhythmias occurri

148 were significantly higher in the ganglia of LQTS/CPVT cases than in healthy controls (P=0.0018 and P

149 enerally well tolerated in this age group of LQTS patients.

150 The authors describe how the histories of LQTS and BrS went through the same stages, but in differ

151 validation study, patients with a history of LQTS-associated life-threatening cardiac events had a mo

152 Identification of LQTS patients more likely to be symptomatic remains elus

153 Further, the majority of LQTS patients have a corrected QT interval below this th

154 In models of LQTS type 2 (LQTS2) using human induced pluripotent stem

155 itional patients with a similar phenotype of LQTS plus a personal or family history of HCM-like pheno

156 ction responsible for a complex phenotype of LQTS, HCM, sudden cardiac death, and congenital heart de

157 To identify FHR predictors of LQTS, we calculated a bradycardia index as % of LQTS FHR

158 channelopathy with a 1% to 5% annual risk of LQTS-triggered syncope, aborted cardiac arrest, or sudde

159 r recordings, could modulate the severity of LQTS type 1 (LQT1) in 46 members of a South-African LQT1

160 We analyzed left stellectomy specimens of LQTS and CPVT patients for signs of inflammatory activit

161 e early detection and risk stratification of LQTS, particularly for fetuses with double mutations, at

162 r by sports medicine doctors on suspicion of LQTS because of marked repolarization abnormalities on t

163 ants associated with CPVT (N54I and N98S) or LQTS (D96V, D130G, and F142L).

164 ure to QT-prolonging stressors, 10 had other LQTS pathogenic mutations, and 10 did not have an LQTS p

165 strategies in molecularly defined pediatric LQTS type 1 and (LQT1) and type 2 (LQT2) patients.

166 defined and appropriately treated pediatric LQTS mutation carriers.

167 2 LQT5) with genotype and phenotype positive LQTS underwent ECG imaging.

168 ases of genotype-negative/phenotype-positive LQTS.

169 with "genotype-negative/phenotype-positive" LQTS.

170 wn LQTS-causative gene, AKAP9, for potential LQTS-type 1-modifying effects.

171 that prenatal rhythm phenotype might predict LQTS genotype and facilitate improved risk stratificatio

172 ease-network algorithms as the most probable LQTS-susceptibility gene and involves a conserved residu

173 The 3 putative LQTS susceptibility missense mutations (KCNQ1, p.A283T;

174 n after LCSD, approximately 50% of high-risk LQTS patients have experienced >/=1 post-LCSD breakthrou

175 sk for ACA or SCD in this overall lower risk LQTS subgroup.

176 riants showed a lower incidence of signature LQTS rhythm (6/27=22%), including TdP (3/27=11%).

177 nd perinatal death: 9 (82%) showed signature LQTS rhythms, 6 (55%) showed TdP, 5 (45%) were stillborn

178 rt rate, and rhythm, including the signature LQTS rhythms: functional 2 degrees atrioventricular bloc

179 It is not uncommon to suspect LQTS among individuals actively practicing sports based

180 EMW negativity in patients with symptomatic LQTS compared to those with asymptomatic LQTS (-52 +/- 3

181 5 (51%) of 225 RSCA cases: long QT syndrome (LQTS) (n = 48 [42%]), hypertrophic cardiomyopathy (HCM)

182 channel function underlie long QT syndrome (LQTS) and are associated with cardiac arrhythmias and su

183 enervation reduces risk in long-QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tach

184 ythmias such as congenital long-QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tach

185 Long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tach

186 different beta-blockers in long QT syndrome (LQTS) and in genotype-positive patients with LQT1 and LQ

187 yopathy, and most recently long-QT syndrome (LQTS) and sudden infant death syndrome.

188 Patients with long QT syndrome (LQTS) are predisposed to life-threatening arrhythmias.

189 of disease severity in the long-QT syndrome (LQTS) as their identification may contribute to refineme

190 The heart rhythm disorder long QT syndrome (LQTS) can result in sudden death in the young or remain

191 Inherited long QT syndrome (LQTS) caused by loss-of-function mutations, or unintende

192 Long QT syndrome (LQTS) exhibits great phenotype variability among family

193 As genetic testing for long QT syndrome (LQTS) has become readily available, important advances a

194 hythmias characteristic of long QT syndrome (LQTS) include torsades de pointes (TdP) and/or 2 degrees

195 Long QT syndrome (LQTS) is a genetic disease characterized by a prolonged

196 Long QT syndrome (LQTS) is a leading cause of sudden cardiac death in earl

197 Long QT syndrome (LQTS) is a potentially lethal but highly treatable cardi

198 Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy with

199 Although the hallmark of long-QT syndrome (LQTS) is abnormal cardiac repolarization, there are vary

200 Congenital Long QT syndrome (LQTS) is an arrhythmogenic disorder that causes syncope

201 Long QT syndrome (LQTS) is an inherited or drug induced condition associat

202 Fetal long QT syndrome (LQTS) is associated with complex arrhythmias including t

203 Inherited long-QT syndrome (LQTS) is associated with risk of sudden death.

204 Long QT syndrome (LQTS) is caused by the abnormal function of ion channels

205 Long-QT syndrome (LQTS) is characterized by a prolonged heart rate-correct

206 Congenital long QT syndrome (LQTS) is characterized by QT prolongation.

207 Long-QT syndrome (LQTS) is characterized by such striking clinical heterog

208 The diagnosis of long QT syndrome (LQTS) is rather straightforward.

209 A puzzling feature of the long QT syndrome (LQTS) is that family members carrying the same mutation

210 Long QT syndrome (LQTS) is the first described and most common inherited a

211 Long QT syndrome (LQTS) is the most common cardiac channelopathy with 15 e

212 Long QT syndrome (LQTS) may contribute to this problem.

213 Long-QT syndrome (LQTS) may result in syncope, seizures, or sudden cardiac

214 Brugada syndrome (BrS) and long-QT syndrome (LQTS) present as congenital or acquired disorders with d

215 clinics and the Rochester long-QT syndrome (LQTS) registry.

216 dentify high- and low-risk long QT syndrome (LQTS) type 1 (LQT1) patients.

217 In long-QT syndrome (LQTS) type 1, severely increased mortality risk during a

218 n these channels can cause Long QT Syndrome (LQTS) which increases the risk for ventricular fibrillat

219 hogenic variants linked to long QT syndrome (LQTS) with early and severe expressivity.

220 apable of producing severe long-QT syndrome (LQTS) with mutations involving CALM1, CALM2, or CALM3.

221 Long-QT syndrome (LQTS), a cardiac arrhythmia disorder with variable pheno

222 mutations associated with long-QT syndrome (LQTS), a potentially lethal cardiac arrhythmia.

223 f the RAS/MAPK pathway and long-QT syndrome (LQTS), and future directions for the field.

224 ion of the QT interval, or long QT syndrome (LQTS), are at risk of life-threatening ventricular arrhy

225 clinical importance in the long QT syndrome (LQTS), however, little genotype-specific data are availa

226 s review will focus on the long QT syndrome (LQTS), the most common of the potentially lethal inherit

227 e KCNH2 gene implicated in Long QT Syndrome (LQTS), which occurred once in 500 whole genome sequences

228 ighest phenotypic risk for long QT syndrome (LQTS)-associated life-threatening cardiac events remains

229 eading cause of congenital long QT syndrome (LQTS).

230 ardiac arrhythmia disease, long QT syndrome (LQTS).

231 reduces cardiac events in long QT syndrome (LQTS).

232 n a 10-day-old infant with long QT syndrome (LQTS).

233 ations in KCNQ1 have KCNQ1 long QT syndrome (LQTS).

234 tentially lethal mendelian long-QT syndrome (LQTS).

235 ular tachycardia (CPVT) or long QT syndrome (LQTS).

236 nagement of the fetus with long QT syndrome (LQTS).

237 in the Mendelian disorder long QT syndrome (LQTS).

238 eta-blockers in congenital long QT syndrome (LQTS).

239 NOS1AP affect drug-induced long QT syndrome (LQTS).

240 tentials, is a hallmark of long-QT syndrome (LQTS).

241 arrhythmia syndromes like long QT syndrome (LQTS).

242 ac channelopathies such as long-QT syndrome (LQTS); however, the underlying molecular mechanisms are

243 Long QT syndromes (LQTS) arise from many genetic and nongenetic causes with

244 n regarding mutation characteristics and the LQTS genotype, identify increased risk for ACA or SCD in

245 edictive for future fatal arrhythmias in the LQTS.

246 We integrated the LQTS network with GWAS loci from the corresponding commo

247 s the risk of AF and its relationship to the LQTS genotype and the long-term prognosis in patients wi

248 We used the LQTS protein network to filter weak GWAS signals by iden

249 These LQTS-causative variants reduce CaM affinity to Ca(2+) an

250 lar block (AVB) are not always attributed to LQTS, although the most common LQTS rhythm, a fetal hear

251 that a resultant fetal loss is attributed to LQTS.

252 the L-type calcium channel Ca(V)1.2 leads to LQTS in patients with Timothy syndrome.

253 among patients with LQTS and its relation to LQTS manifestations are lacking.

254 re a novel pharmacological approach to treat LQTS.

255 were curated as definitive genes for typical LQTS.

256 The underlying LQTS genotype was LQT1 in 36 (56%) and LQT2 in 20 (31%).

257 e life saving for the fetus and unsuspecting LQTS family members.

258 .1 loss-of-function consistent with in utero LQTS type 1, whereas the HERG1b-R25W mutation (33.2-week

259 a loss of function consistent with in utero LQTS type 2.

260 The commonest CID identified after RSCA was LQTS; the most common CID cause of RSCA for those >40 ye

261 voke arrhythmogenic Ca disturbances, whereas LQTS-CaMs do not.

262 ophysiological substrate and examine whether LQTS patients display regional heterogeneities in repola

263 CALM2 mutations can be associated with LQTS and with overlapping features of LQTS and CPVT.

264 fest rhythms not known to be associated with LQTS increases the difficulty of echocardiographic diagn

265 al death, missense mutations associated with LQTS susceptibility were discovered in 3 cases (3.3%) an

266 ontrol study including 112 patient duos with LQTS from France, Italy, and Japan, 25 polymorphisms wer

267 of risk stratification within families with LQTS, leading to more targeted management.

268 LM1-3 should be pursued for individuals with LQTS, especially those with early childhood cardiac arre

269 ct the clinical outcomes of individuals with LQTS.

270 10.0+/-10 years; mean QTc, 528+/-74 ms) with LQTS who underwent LCSD between 2005 and 2010 (mean age

271 covered in a 10-year-old female patient with LQTS with a QTc of 500 milliseconds who experienced recu

272 edian QTc did not change among patients with LQTS (461+/-60 to 476+/-54 ms; P=0.49).

273 Genotype-positive patients with LQTS (784 LQT1, 746 LQT2, and 233 LQT3) were compared wi

274 We analyzed a cohort of 651 patients with LQTS (age 26 +/- 17 years; 60% females; 158 symptomatic;

275 ctive study comprising the 606 patients with LQTS (LQT1 in 47%, LQT2 in 34%, and LQT3 in 9%) who were

276 Patients with LQTS (N=40) and catecholaminergic polymorphic ventricula

277 sing data were reviewed for 90 patients with LQTS 1 and 2 who reside in Auckland, New Zealand, during

278 rdiac sympathetic denervation, patients with LQTS and CPVT have high levels of postoperative satisfac

279 scale studies of AF risk among patients with LQTS and its relation to LQTS manifestations are lacking

280 EMW was found among nearly all patients with LQTS compared to controls, with more profound EMW negati

281 1-time monotherapy for certain patients with LQTS requires further evaluation.

282 and predictors thereof, among patients with LQTS types 1 and 2.

283 eview was performed on the 204 patients with LQTS who underwent LCSD at our institution since 2005 to

284 e probability of ACA or SCD in patients with LQTS with normal-range QTc intervals (4%) was significan

285 mptomatic and 162 asymptomatic patients with LQTS, and a meta-analysis was performed.

286 mittee approved use of EMW for patients with LQTS, making it a routinely reported echocardiographic f

287 of risk for cardiac events in patients with LQTS.

288 mutations in a large cohort of patients with LQTS.

289 tion against cardiac events in patients with LQTS.

290 ach for risk stratification in patients with LQTS.

291 therapy for carefully selected patients with LQTS.

292 versus asymptomatic status in patients with LQTS.

293 and the long-term prognosis in patients with LQTS.

294 Probands with LQTS (n=167) were screened for mutations in CAV3 using d

295 sociated loci in 298 unrelated probands with LQTS identified coding variants not found in controls bu

296 t-generation sequencing in two siblings with LQTS in a Spanish family of African ancestry.

297 ssense mutations in CALM2 in 3 subjects with LQTS (p.N98S, p.N98I, p.D134H) and 2 subjects with clini

298 in LQTS is suboptimal in half of those with LQTS 1 and 2.

299 repolarization gradients between and within LQTS types.

300 owards 'mutation-specific' management within LQTS subtypes.