ライフサイエンスコーパス: LQTS

1 LQTS boys experience a significantly higher rate of fata

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 subjects maintain a high risk for life-threatening

6 1:LQTS type 2, hazard ratio: 9.88; p = 0.03; LQTS type 3:LQTS type 2, hazard ratio: 8.04; p = 0.07),

7 A total of 1,059 LQTS patients with a corrected QT interval > or =450 ms

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

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

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

11 = 0.006) and the LQTS genotypes (LQTS type 1:LQTS type 2, hazard ratio: 9.88; p = 0.03; LQTS type 3:L

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

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

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

15 2, hazard ratio: 9.88; p = 0.03; LQTS type 3:LQTS type 2, hazard ratio: 8.04; p = 0.07), whereas clin

16 rdiac arrest or sudden cardiac death in 3015 LQTS children from the International LQTS Registry who w

17 We analyzed ECG recordings with TdP from 35 LQTS patients (15 c-LQTS and 20 a-LQTS) and compared the

18 ing beat emerged from a T-U-wave in 27 of 35 LQTS patients and in none of 40 control patients.

19 /- 0.9 mm) directly preceded TdP in 34 of 35 LQTS patients and were larger than T-wave amplitude (2.8

20 l QT intervals and with PVCs in 24 of the 35 LQTS patients not related to TdP.

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

22 ith normal-range QTc (</= 440 ms [n = 469]), LQTS with prolonged QTc interval (> 440 ms [n = 1,392]),

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

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

25 The eag domains with Y43A or R56Q (a LQTS locus) mutations showed less regulation of deactiva

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

27 dP from 35 LQTS patients (15 c-LQTS and 20 a-LQTS) and compared them with premature ventricular compl

28 l mechanism underlying inherited or acquired LQTS.

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

30 a-blocker therapy on cardiac events in adult LQTS patients with known cardiac channel mutations.

31 jects displayed gender differences: Affected LQTS women experienced a significantly higher cumulative

32 Risk assessment in affected LQTS patients relies upon a constellation of electrocard

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

34 ge, have therapeutic implications for ageing LQTS patients.

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

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

37 , 556 (92%) patients have not experienced an LQTS-triggered BCE.

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

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

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

41 ent to regulate deactivation gating, that an LQTS mutation perturbed physical interactions between th

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

43 only (LQTS; n = 40, QT >500 ms on drug) and LQTS + TdP (TdP; n = 83).

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

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

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

47 candidate genes for ventricular arrhythmias, LQTS and SCD.

48 ultinational LQTS registries, categorized as LQTS with normal-range QTc (</= 440 ms [n = 469]), LQTS

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

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

51 rol study of 123 adults with drug-associated LQTS.

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

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

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

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

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

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

58 ordings with TdP from 35 LQTS patients (15 c-LQTS and 20 a-LQTS) and compared them with premature ven

59 ons found in definite cases that would cause LQTS) were determined according to mutation type and loc

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

61 rding the outcome of patients with concealed LQTS are limited.

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

63 Genotype-confirmed patients with concealed LQTS make up about 25% of the at-risk LQTS population.

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

65 ts in adult patients with mutation-confirmed LQTS.

66 Congenital LQTS is an important cause of sudden cardiac death.

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

68 e been identified as the cause of congenital LQTS.

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

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

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

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

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

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

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

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

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

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

79 unrelated patients with genetically elusive LQTS.

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

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

82 Records of subjects exhibiting fetal LQTS arrhythmias were reviewed.

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

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

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

86 -specific data are available regarding fetal LQTS.

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

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

89 ify a novel underlying genetic mechanism for LQTS.

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

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

92 io: 6.32; p = 0.006) and the LQTS genotypes (LQTS type 1:LQTS type 2, hazard ratio: 9.88; p = 0.03; L

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

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

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

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

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

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

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

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

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

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

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

104 We compared FHR in LQTS subjects versus normal fetuses.

105 , giant T-U waves separate TdP initiation in LQTS patients from PVCs in other heart disease and from

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

107 thy subjects and severity of presentation in LQTS.

108 n control PVCs (145 +/- 4 ms) and in PVCs in LQTS patients not related to TdP (138 +/- 22 ms).

109 n other heart disease and from other PVCs in LQTS patients.

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

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

112 with polymorphic ventricular tachycardia in LQTS patients.

113 in the interpretation of genetic testing in LQTS.

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

115 ents and larger than the largest T-U-wave in LQTS without TdP (4.7 +/- 0.8 mm).

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 Drug-induced LQTS includes individuals developing marked prolongation

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

120 ying genetic basis for recessively inherited LQTS.

121 Accumulating data from the International LQTS Registry have recently facilitated a comprehensive

122 in 3015 LQTS children from the International LQTS Registry who were followed up from 1 through 12 yea

123 ssed in 2759 subjects from the International LQTS Registry, categorized into electrocardiographically

124 st symptom were drawn from the International LQTS Registry.

125 Patients were divided into LQTS only (LQTS; n = 40, QT >500 ms on drug) and LQTS +

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

127 Therefore KCNQ1 LQTS patients may exhibit increased insulin secretion.

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

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

130 were negative for mutations in the 11 known LQTS-susceptibility genes.

131 Hundreds of causative mutations in 12 known LQTS-susceptibility genes have been identified.

132 ects who were genetically tested for a known LQTS mutation (hazard ratio 4.76, P=0.02).

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

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

135 Beta-blockers are the mainstay in managing LQTS.

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

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

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

139 ical dysfunction for sodium-channel-mediated LQTS (LQT3).

140 were similar in the 2 groups (381 +/- 38 ms [LQTS] vs. 388 +/- 43 ms [TdP]).

141 ,386 genotyped subjects from 7 multinational LQTS registries, categorized as LQTS with normal-range Q

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

143 utation in 1/269 unrelated genotype-negative LQTS patients that was absent in 400 control alleles.

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

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

146 Approximately 20% of LQTS cases remain genetically elusive.

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

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

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

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

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

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

153 score >or=4 and/or QTc >or=480 ms) cases of LQTS and >1300 healthy controls for each gene.

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

155 ha1-syntrophin, was performed in a cohort of LQTS patients that were negative for mutations in the 11

156 indicated that the phenotypic expression of LQTS is time dependent and age specific, warranting cont

157 le penetrance, and pleiotropic expression of LQTS mutations.

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

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

160 strategies for the treatment of this form of LQTS.

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

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

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

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

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

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

167 The management of LQTS has become subtype-specific due to the availability

168 etic testing, subtype-specific management of LQTS has become the standard of care.

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

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

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

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

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

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

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

176 gement strategies in the high-risk subset of LQTS patients presenting with syncope.

177 Patients were divided into LQTS only (LQTS; n = 40, QT >500 ms on drug) and LQTS + TdP (TdP; n

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

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

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

181 defined and appropriately treated pediatric LQTS mutation carriers.

182 2 LQT5) with genotype and phenotype positive LQTS underwent ECG imaging.

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

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

185 Here, comprehensive postmortem LQTS genetic testing was performed in a cohort of SUD ca

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

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

188 y be defined confidently as high-probability LQTS-causing mutations.

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

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

191 and suggest that SNTA1 be considered a rare LQTS-susceptibility gene.

192 cealed LQTS make up about 25% of the at-risk LQTS population.

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

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

195 d proteins cause inherited long QT syndrome (LQTS) and account for approximately 75-80% of cases (LQT

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

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

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

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

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

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

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

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

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

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

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

207 Genetic testing for long-QT syndrome (LQTS) has diagnostic, prognostic, and therapeutic implic

208 n patients with congenital long-QT syndrome (LQTS) have focused mainly on the first 4 decades of life

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

210 The hereditary long QT syndrome (LQTS) is a genetic channelopathy with variable penetranc

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

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

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

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

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

216 The congenital long-QT syndrome (LQTS) is an important cause of sudden cardiac death in c

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

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

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

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

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

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

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

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

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

226 s for fatal arrhythmias in long QT syndrome (LQTS) patients presenting with syncope.

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

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

229 nical course of women with long QT syndrome (LQTS) throughout their potential childbearing years.

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

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

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

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

234 ng events in patients with long-QT syndrome (LQTS) with normal corrected QT (QTc) intervals.

235 firmed adult patients with long QT syndrome (LQTS), 2) to study life-threatening cardiac events as a

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

237 deactivation are linked to long QT syndrome (LQTS), a catastrophic cardiac arrhythmia.

238 ry subunit, associate with long QT syndrome (LQTS), a defect in ventricular repolarization.

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

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

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

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

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

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

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

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

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

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

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

250 d (a-) and congenital (c-) long QT syndrome (LQTS).

251 for inherited and acquired long-QT syndrome (LQTS).

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

253 ients with drug-associated long QT syndrome (LQTS).

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

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

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

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

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

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

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

261 related to gender, clinical history, and the LQTS genotype.

262 ions: hazard ratio: 6.32; p = 0.006) and the LQTS genotypes (LQTS type 1:LQTS type 2, hazard ratio: 9

263 edictive for future fatal arrhythmias in the LQTS.

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

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

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

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

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

269 re a novel pharmacological approach to treat LQTS.

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

271 .1 loss-of-function consistent with in utero LQTS type 1, whereas the HERG1b-R25W mutation (33.2-week

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

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

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

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

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

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

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

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

280 10.0+/-10 years; mean QTc, 528+/-74 ms) with LQTS who underwent LCSD between 2005 and 2010 (mean age

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

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

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

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

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

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

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

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

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

290 of risk for cardiac events in patients with LQTS.

291 mutations in a large cohort of patients with LQTS.

292 tion against cardiac events in patients with LQTS.

293 ach for risk stratification 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.