ライフサイエンスコーパス: HCM

1 HCM mutants were hypersensitive and DCM mutants were hyp

2 HCM patients have a significant incidence of screening f

3 HCM patients showed significantly greater E2A in diastol

4 HCM patients with LV apical aneurysms are at high risk f

5 HCM patients with nonobstructive disease appear to exper

6 HCM variant locations predict impaired IHM formation and

7 HCM variants, 72% that changed electrostatic charges, di

8 HCM-related death occurred in 18 patients (3%; 0.54%/y):

9 HCM-related death occurred in 40 patients (4% [0.53%/yea

10 HCM-related mortality among nonobstructive patients was

11 16 years; 92 males and 39 females) with >/=1 HCM risk factor for sudden death underwent S-ICD ECG scr

12 ormed in 19 control subjects, 19 DCM, and 13 HCM patients.

13 en studies were included representing 12,146 HCM patients.

14 ered IHM interaction residues (expected 23%; HCM 54%, p=2.6x10(-19); DCM 26%, p=0.66; controls 20%, p

15 METHODS AND A total of 448 HCM patients were next generation-sequenced (semiconduct

16 s, and then analyzed rare variants from 6112 HCM and 1315 DCM patients and 33,370 ExAC controls.

17 graphic datasets obtained from 77 ATH and 62 HCM patients were used for developing an automated syste

18 eater than the 2.0%/year event rate in 1,847 HCM patients without aneurysms (p < 0.001).

19 ntical to that reported in middle-aged adult HCM patients (98% and 94%, P=0.23).

20 In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary manag

21 ong-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 4

22 ed survival and the clinical course of adult HCM patients.

23 lationship between aneurysm size and adverse HCM-related events.

24 Our results showed that young and aged HCM mice displayed echocardiographic characteristics of

25 addition, when compared with controls, aged HCM females exhibited adrenal gland hypertrophy, reduced

26 ous system activation were increased in aged HCM females vs. controls and correlated with mood disord

27 f the heart disease condition, yet only aged HCM females displayed anxiety- and depression-like behav

28 Our findings explain how the effects of an HCM mutation in the C-domain reflect up into the N-domai

29 e symptoms, other disease complications, and HCM-related mortality, and largely without the requireme

30 ament ADP sensitivity was higher in IDCM and HCM compared with donors, whereas it was lower for MYBPC

31 nd hcTnC may lead to impaired relaxation and HCM.

32 e best predictor for differentiating between HCM ands.

33 ve T1 may be applied to discriminate between HCM and hypertensive heart disease and detect early chan

34 sease by T1 mapping can discriminate between HCM versus hypertensive heart disease as well as to dete

35 T1 was an independent discriminator between HCM and hypertension, over and above extracellular volum

36 RVC) (13%); and hypertrophic cardiomyopathy (HCM) (6%).

37 he technique in hypertrophic cardiomyopathy (HCM) and DCM.

38 icular, between hypertrophic cardiomyopathy (HCM) and increased LV wall thickness because of systemic

39 na is common in hypertrophic cardiomyopathy (HCM) and is associated with abnormal myocardial perfusio

40 ith obstructive hypertrophic cardiomyopathy (HCM) and mild septal hypertrophy, mitral valve (MV) abno

41 NT1 region, six hypertrophic cardiomyopathy (HCM) and two dilated cardiomyopathy (DCM) mutants were s

42 fected genes in hypertrophic cardiomyopathy (HCM) are MYH7 (beta-myosin heavy chain) and MYBPC3 (beta

43 ount for 35% of hypertrophic cardiomyopathy (HCM) cases in the Netherlands.

44 een reported in hypertrophic cardiomyopathy (HCM) caused by Danon disease, Vici syndrome, or LEOPARD

45 features of the hypertrophic cardiomyopathy (HCM) ECG make it a challenge for subcutaneous implantabl

46 scrimination of hypertrophic cardiomyopathy (HCM) from physiological hypertrophy seen in athletes (AT

47 Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse diseas

48 Hypertrophic cardiomyopathy (HCM) is a clinically and genetically heterogeneous disor

49 Hypertrophic cardiomyopathy (HCM) is a genetic disorder that is characterized by left

50 RATIONALE: Hypertrophic cardiomyopathy (HCM) is a prototypic single-gene disease caused mainly b

51 Hypertrophic cardiomyopathy (HCM) is a relatively common genetic heart disease encumb

52 Hypertrophic cardiomyopathy (HCM) is an inherited disease of heart muscle that can be

53 Hypertrophic cardiomyopathy (HCM) is caused by mutations in sarcomere protein genes,

54 Hypertrophic cardiomyopathy (HCM) is caused by mutations in sarcomeric proteins, the

55 ural history of hypertrophic cardiomyopathy (HCM) is complex and may include progressive heart failur

56 ive variants in hypertrophic cardiomyopathy (HCM) is increased in some probands, suggesting different

57 Hypertrophic cardiomyopathy (HCM) is one of the most common cardiomyopathies and a ma

58 Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder, with

59 Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiovascular disease

60 n patients with hypertrophic cardiomyopathy (HCM) older than 25 years.

61 seases, such as hypertrophic cardiomyopathy (HCM) or cardiac channelopathies such as long-QT syndrome

62 nized subset of hypertrophic cardiomyopathy (HCM) patients with left ventricular (LV) apical aneurysm

63 ated (IDCM) and hypertrophic cardiomyopathy (HCM) patients.

64 d reversal of a hypertrophic cardiomyopathy (HCM) phenotype is novel.

65 associated with hypertrophic cardiomyopathy (HCM) remains challenging.

66 Hypertrophic cardiomyopathy (HCM) results from mutations in genes encoding sarcomeric

67 y guidelines of hypertrophic cardiomyopathy (HCM) use a new clinical risk prediction model for sudden

68 to obstructive hypertrophic cardiomyopathy (HCM) who are at unacceptable surgical risk.

69 ndividuals with hypertrophic cardiomyopathy (HCM) who exercise regularly is unknown.

70 y (DCM), 6% for hypertrophic cardiomyopathy (HCM), 12% for restrictive cardiomyopathy, and 23% for le

71 associated with hypertrophic cardiomyopathy (HCM), a disease associated with sudden death in apparent

72 H7) can lead to hypertrophic cardiomyopathy (HCM), a heritable disease characterized by cardiac hyper

73 ndrome (LQT-3), hypertrophic cardiomyopathy (HCM), and ventricular tachycardia-ventricular fibrillati

74 d arrhythmia in hypertrophic cardiomyopathy (HCM), is capable of producing symptoms that impact quali

75 r patients with hypertrophic cardiomyopathy (HCM), largely because of the possibility of sudden death

76 ibited in human hypertrophic cardiomyopathy (HCM), to investigate the influence of HCM over the devel

77 pathy (DCM) and hypertrophic cardiomyopathy (HCM), which are often due to mutations of specific compo

78 associated with hypertrophic cardiomyopathy (HCM).

79 e remodeling in hypertrophic cardiomyopathy (HCM).

80 ommon causes of hypertrophic cardiomyopathy (HCM).

81 f patients with hypertrophic cardiomyopathy (HCM).

82 associated with hypertrophic cardiomyopathy (HCM).

83 didate gene for hypertrophic cardiomyopathy (HCM).

84 nal features of hypertrophic cardiomyopathy (HCM).

85 cal phenotypes (hypertrophic cardiomyopathy, HCM and dilated cardiomyopathy, DCM) associated with mut

86 encoding sarcomere-associated proteins cause HCM.

87 , the underlying molecular mechanism causing HCM is unknown.

88 ion and energetic deficits that characterize HCM.

89 energy consumption, that fully characterizes HCM pathogenesis.

90 stony-elastic consistency typical of classic HCM, and this prompted histological examinations.

91 with left ventricular hypertrophy (clinical HCM; n=36), mutation carriers with normal left ventricul

92 ongstanding history of genetically confirmed HCM presented with rapid development of congestive heart

93 studied long-term outcome in 474 consecutive HCM patients between 7 and 29 years of age presenting at

94 We prospectively studied 573 consecutive HCM patients in 3 centers (44 +/- 17 years; 66% male) wi

95 s retrospectively analyzed 1,940 consecutive HCM patients at 2 centers, 93 of which (4.8%) were ident

96 The DCM/HCM-associated mutants of vinculin occur in the 68-resid

97 P2 Unlike vinculin, wild-type MV and the DCM/HCM-associated R975W mutant bind PIP2 in their inactive

98 uld prevent the development of a deleterious HCM phenotype.

99 ters alone are insufficient to differentiate HCM from physiological LVH and should be complemented by

100 sitivity and specificity for differentiating HCM from physiological LVH: 13% had a left ventricular c

101 A recently discovered HCM-associated 25-base pair deletion in MYBPC3 is inheri

102 e demonstrate that there is an embryological HCM phenotype.

103 ing as the most prominent predictor of fatal HCM.

104 ilar to G+ HCM but worse than G- HCM and FG+ HCM diagnosed in the context of family screening.

105 w-up of FG+ and G+ HCM versus G- HCM and FG+ HCM in relatives.

106 Clinical phenotype and outcome of FG+ HCM was similar to G+ HCM but worse than G- HCM and FG+

107 and outcome of MYBPC3 founder mutation (FG+) HCM with nonfounder genotype-positive (G+) and genotype-

108 n may be a valuable therapeutic approach for HCM.

109 in 3 European tertiary referral centers for HCM from July 2013 to December 2016.

110 at hyperdynamic contraction is essential for HCM pathobiology and that inhibitors of sarcomere contra

111 p.Ala13Thr variant in MYL2, a known gene for HCM.

112 subclinical changes in patients at risk for HCM and to evaluate the effects of interventions.

113 Cardiac samples from HCM patients, harboring thick-filament (MYH7mut, MYBPC3m

114 d for more intensive follow-up of FG+ and G+ HCM versus G- HCM and FG+ HCM in relatives.

115 y in FG+ probands with HCM was similar to G+ HCM (22% versus 14%; log-rank P=0.14), but higher than G

116 ype and outcome of FG+ HCM was similar to G+ HCM but worse than G- HCM and FG+ HCM diagnosed in the c

117 us 14%; log-rank P=0.14), but higher than G- HCM (22% versus 6%; log-rank P<0.001) and FG+ relatives

118 HCM was similar to G+ HCM but worse than G- HCM and FG+ HCM diagnosed in the context of family scree

119 ensive follow-up of FG+ and G+ HCM versus G- HCM and FG+ HCM in relatives.

120 ype-positive (G+) and genotype-negative (G-) HCM.

121 earlier disease onset than patients who have HCM with variants elsewhere.

122 However, HCM is also an important cause of sudden cardiac death,

123 Human HCM demonstrates calcium mishandling through both genoty

124 ays for calcium regulatory pathways in human HCM surgical samples with (n=25) and without (n=10) sarc

125 d the electromechanical dysfunction of human HCM myocardium in vitro.

126 ients with diagnoses of HCM or hypertension (HCM, n=95; hypertension, n=69) and G+P- subjects (n=23)

127 that features of the human pre-hypertrophic HCM phenotype occur in the mouse.

128 iac beta-myosin variants cause hypertrophic (HCM) or dilated (DCM) cardiomyopathy by disrupting sarco

129 unction (DCM), and extent of LV hypertrophy (HCM).

130 k factors may thus be useful for identifying HCM patients who might benefit from early diagnosis and

131 In HCM, mutation-silencing therapies are likely to be ineff

132 Perfusion abnormalities in HCM are not simply a consequence of supply/demand mismat

133 C), could detect tissue-level alterations in HCM sarcomere mutation carriers with and without left ve

134 e central role of the late sodium current in HCM, and introduce the scientific rationale and executio

135 AF is an uncommon primary cause of death in HCM virtually limited to embolic stroke, supporting a lo

136 [(3)H]ryanodine binding was not different in HCM, consistent with no major modification of the ryanod

137 hypothesis that the missing causal genes in HCM is, in part, because of an oligogenic cause, wherein

138 Coronary hemodynamics in HCM were characterized by a very large backward compress

139 volume fraction were significantly higher in HCM compared with patients with hypertension (P<0.0001),

140 ain 3 (LC3)-II protein levels were higher in HCM septal myectomies than in nonfailing control hearts

141 ription factor activity was not increased in HCM, suggesting that calcineurin pathway activation is n

142 As such, unmet medical need in HCM has been highlighted by the National Heart, Lung, an

143 ci syndrome, or LEOPARD syndrome, but not in HCM caused by mutations in genes encoding sarcomeric pro

144 ence of AF on clinical course and outcome in HCM remains incompletely resolved.

145 itical role of the early postnatal period in HCM pathogenesis and suggest that mutant sarcomeres mani

146 at altered myofilament structures present in HCM-D166V mice were mitigated in S15D-D166V rescue mice.

147 RCA2A mRNA transcript levels were reduced in HCM regardless of genotype, as was sarcoplasmic endoplas

148 tein kinase type II (CaMKII) were similar in HCM samples compared with controls.

149 5 randomized studies of medical therapies in HCM.

150 eeded to conduct rigorous clinical trials in HCM has recently emerged because of the heightened aware

151 take assay showed reduced uptake velocity in HCM regardless of genotype (P=0.01).

152 3 mutations and in a Mybpc3-targeted knockin HCM mouse model, as well as the effect of autophagy modu

153 tomatic Hypertrophic Cardiomyopathy (LIBERTY-HCM) trial, the largest randomized, double-blind, placeb

154 e significantly improved survival with a low HCM-related mortality of 0.5% per year across all ages,

155 from a large pedigree with concomitant LQTS, HCM, and congenital heart defects and identified a novel

156 nts with a complex phenotype including LQTS, HCM, and congenital heart defects annotated as cardiac-o

157 responsible for a complex phenotype of LQTS, HCM, sudden cardiac death, and congenital heart defects.

158 Human cerebral malaria (HCM) is a serious complication of Plasmodium falciparum

159 12-dependent methylmalonyl-CoA mutase (MCM), HCM has a highly conserved domain architecture.

160 ase of amyloidosis, which classically mimics HCM.

161 12-dependent 2-hydroxyisobutyryl-CoA mutase (HCM) is a radical enzyme catalyzing the stereospecific i

162 g protein C), is the most frequently mutated HCM gene.

163 nce was increased only in sarcomere-mutation HCM (P<0.001).

164 .5-fold increased only in sarcomere-mutation HCM (P=0.01), as was autophosphorylated CaMKII (P<0.01),

165 muscle cells (HCASMC) and cardiac myocytes (HCM), leading to upregulation of antioxidant defense enz

166 th sarcomere mutation-positive and -negative HCM.

167 re compared with sarcomere mutation-negative HCM (HCMsmn) and nonfailing donors.

168 no sarcomere mutation, that is, nonfamilial HCM.

169 Nonobstructive HCM patients were less likely to experience advanced lim

170 ological exercise (<30 mm Hg; nonobstructive HCM) and retrospectively assembled clinical follow-up da

171 A small minority of nonobstructive HCM patients progress to heart transplant.

172 set out to functionally characterize a novel HCM-associated mutation (K206I-TNNI3) and elucidate the

173 nts with a clinical diagnosis of obstructive HCM referred for surgical management of LVOTO were obser

174 agement of severely symptomatic, obstructive HCM patients.

175 y for patients with symptomatic, obstructive HCM.

176 ry heart failure symptoms due to obstructive HCM (New York Heart Association functional class III).

177 verely symptomatic patients with obstructive HCM and mild septal hypertrophy.

178 in 39 consecutive patients with obstructive HCM.

179 In approximately 40% of HCM patients, the causal genes remain to be identified.

180 Approximately 40% of HCM probands have a nonfamilial subtype, with later onse

181 FLNC candidate variants in a large cohort of HCM patients who were also sequenced for the main sarcom

182 al course and prognosis of a large cohort of HCM patients with LV apical aneurysms over long-term fol

183 .0001), including in subgroup comparisons of HCM subjects without evidence of late gadolinium enhance

184 concentrations prevented the development of HCM-related cardiac phenotype, including thickening of t

185 he involvement of FLNC in the development of HCM.

186 Patients with diagnoses of HCM or hypertension (HCM, n=95; hypertension, n=69) and

187 variant, in whom an unambiguous diagnosis of HCM could not be made because of concomitant severe aort

188 cutive patients with a clinical diagnosis of HCM underwent septal myectomy.

189 Four percent of patients with AF died of HCM-related causes (n=11), with annual mortality 0.7%; m

190 -8 mo) female mice to examine the effects of HCM on the development of anxiety- and depression-like b

191 The histological features of HCM include myocyte hypertrophy and disarray, as well as

192 variants were associated with mild forms of HCM and a reduced penetrance, with few affected in the f

193 probands with no reported family history of HCM, including 166 (40% of total) probands with no sarco

194 of LQTS plus a personal or family history of HCM-like phenotypes and identified 2 additional pedigree

195 pathy (HCM), to investigate the influence of HCM over the development of anxiety and depression.

196 The majority of HCM-associated mutations are found in genes encoding sar

197 udy was to use a regulatable murine model of HCM to study the reversibility of pathological LVH.

198 rcomeric proteins, which account for most of HCM cases.

199 tudy in the setting of the HCMNet network of HCM clinical centers.

200 , most data have supported the occurrence of HCM at about 1 in 500.

201 derstanding of the molecular pathogenesis of HCM and have stimulated efforts designed to identify new

202 will discuss the complex pathophysiology of HCM, review the current therapeutic landscape, describe

203 tal trajectory is altered by the presence of HCM truncating Mybpc3 gene mutation.

204 Rate of HCM-related deaths combined with life-saving aborted dis

205 y, we have analyzed the crystal structure of HCM from Aquincola tertiaricarbonis in complex with coen

206 utations represent a nonfamilial subgroup of HCM.

207 implications of this nonfamilial subgroup of HCM.

208 We posit that a subset of HCM might be oligogenic caused by multiple pathogenic va

209 METHODS AND One hundred and thirty-one HCM patients (age, 50+/-16 years; 92 males and 39 female

210 Five- and 10-year survival (considering only HCM deaths) was high (97% and 94%, respectively), virtua

211 th controls, the SIC was 61% higher in overt HCM and 47% higher in subclinical HCM (P<0.001 for both)

212 atively different across subjects with overt HCM, subclinical HCM, and healthy controls (P<0.001).

213 In the majority of patients, HCM has a relatively benign course.

214 ere stiffness in sarcomere mutation-positive HCM samples was irrespective of the phosphorylation back

215 y is specific to sarcomere mutation-positive HCM, whereas sarcoplasmic endoplasmic reticular calcium

216 ts (P=0.04) compared with sarcomere-positive HCM probands.

217 inant mutations in vinculin can also provoke HCM, causing acute cardiac failure.

218 otype in mice carrying a clinically relevant HCM-related mutation.

219 n combination with another therapy to rescue HCM caused by MYBPC3 mutations.

220 e diseases also cause a phenotype resembling HCM (genocopy or phenocopy).

221 enotype-negative subjects carrying high-risk HCM-related mutations.

222 is required to ensure that the highest risk HCM patients can benefit from S-ICD implantation.

223 nd validate a risk prediction model for SCD (HCM Risk-SCD [hypertrophic cardiomyopathy risk-SCD]).

224 es with HCM were compared with 101 sedentary HCM patients.

225 10.7+/-3.9, P<0.001) compared with sedentary HCM patients.

226 ated probands with HCM seen in a specialized HCM center between 2002 and 2015 and genetic testing per

227 across subjects with overt HCM, subclinical HCM, and healthy controls (P<0.001).

228 r in overt HCM and 47% higher in subclinical HCM (P<0.001 for both).

229 left ventricular wall thickness (subclinical HCM; n=28), and healthy controls (n=10).

230 in FG+/phenotype-negative relatives; subtle HCM developed in 11% during 6 years of follow-up.

231 ogical approach in patients with symptomatic HCM.

232 In conclusion, prolonged systemic HCM stress can lead to development of mood disorders, po

233 Our previous studies on transgenic (Tg) HCM-RLC mice revealed that the D166V (Aspartate166 --> V

234 t failure (n=12), 1.8%/y, 3-fold higher than HCM mortality.

235 They suggest that HCM is more common than previously estimated, which may

236 The HCM Risk-SCD model improves the risk stratification of p

237 er a molecular-level explanation for how the HCM mutation cTnI-R145G reduces the modulation of cTn by

238 te II, thus opening up new insights into the HCM phenotype.

239 Therefore, we introduced the HCM mutation E56G into a single-cysteine (C16) hVELC con

240 the causal genes in approximately 40% of the HCM cases remain unknown, typically in small families an

241 ther accounting for approximately 50% of the HCM families.

242 TAxalphaMHCR403Q, in which expression of the HCM-causing Arg403Gln mutation in the alpha-myosin heavy

243 for sudden cardiac death (SCD), based on the HCM Risk-SCD study.

244 m in mutant cTnC that may play a role to the HCM phenotype.

245 ir structural defects has been mapped to the HCM phenotype.

246 rter-defibrillator therapy, identical to the HCM Risk-SCD end point.

247 The 5-year SCD risk was calculated using the HCM Risk-SCD formula.

248 e hypercontractile phenotype induced by this HCM mutation in myosin.

249 e 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not differen

250 s before application of prophylactic ICDs to HCM, or were without conventional risk factors.

251 n of hydroxylated acyl-CoA esters, wild-type HCM as well as HcmA I90V and I90A mutant enzymes could a

252 that the majority of patients affected with HCM can achieve normal or near-normal life expectancy wi

253 ients with genetic mutations associated with HCM.

254 Most athletes with HCM (96%) exhibited T-wave inversion and had milder LVH

255 Only a minority of athletes with HCM constitute the conventional gray zone of mild, conce

256 Athletes with HCM exhibit less LVH, larger left ventricular cavities,

257 A subset of athletes with HCM exhibiting morphologically mild (13-16 mm), concentr

258 s from 106 young (14-35 years) athletes with HCM were compared with 101 sedentary HCM patients.

259 In athletes with HCM, LVH was frequently (36%) confined to the apex and o

260 the clinical profile of young athletes with HCM.

261 ation and reduced mobility, contrasting with HCM, which showed reduced mobility with altered diastoli

262 The male index case was diagnosed with HCM after an out-of-hospital cardiac arrest, which was f

263 ds with HCM, 54 FG+ relatives diagnosed with HCM after family screening, 74 FG+/phenotype-negative re

264 examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom t

265 ere clinical deterioration in a patient with HCM due to superimposed amyloid light-chain amyloidosis.

266 terior descending artery in 33 patients with HCM and 20 control patients at rest and during hyperemia

267 occurs over several years for patients with HCM and can be detected at relatively early stages, the

268 AND We evaluated autophagy in patients with HCM carrying MYBPC3 mutations and in a Mybpc3-targeted k

269 at (1) autophagy is altered in patients with HCM carrying MYBPC3 mutations, (2) autophagy is impaired

270 ves the risk stratification of patients with HCM for primary prevention of SCD, and calculating an in

271 Patients with HCM had a lower coronary flow reserve than control subje

272 Coronary flow in patients with HCM is deranged.

273 in the absence of red flags in patients with HCM older than 25 years.

274 and phenotypic data from 2,913 patients with HCM to identify regions of disease enrichment within bet

275 Nevertheless, for those patients with HCM who are at risk for (or experience) disease-related

276 Etiology of patients with HCM who underwent surgical myectomy.

277 Notably, patients with HCM with variants in the enriched regions have earlier d

278 th AF and age- and sex-matched patients with HCM without AF.

279 of a consecutive cohort of 706 patients with HCM without prior SCD event, from 2 tertiary referral ce

280 Of the 1558 patients with HCM, 304 (20%) had episodes of AF, of which 226 (74%) we

281 Among patients with HCM, age, NYHA functional class, family history of sudde

282 d therapy for the treatment of patients with HCM, and to date there have been only 5 randomized studi

283 ive lifestyle for thousands of patients with HCM, while providing many with a measure of reassurance

284 ing perfusion abnormalities in patients with HCM.

285 nostic factors for survival in patients with HCM.

286 ole risk factor identified for patients with HCM.

287 hundred and thirteen unrelated probands with HCM seen in a specialized HCM center between 2002 and 20

288 ardiovascular mortality in FG+ probands with HCM was similar to G+ HCM (22% versus 14%; log-rank P=0.

289 inical phenotype of FG+ and G+ probands with HCM was similar.

290 FG+ carriers included 134 FG+ probands with HCM, 54 FG+ relatives diagnosed with HCM after family sc

291 d 680 271 FG+ carriers, 132 G+ probands with HCM, and 277 G- probands with HCM.

292 diastolic dysfunction than FG+ probands with HCM.

293 probands with HCM, and 277 G- probands with HCM.

294 6%; log-rank P<0.001) and FG+ relatives with HCM (22% versus 4%; P=0.009).

295 FG+ relatives with HCM had less hypertrophy, smaller left atria, and less s

296 The TTN variant segregated with HCM, except in a 7-year-old boy, who had a normal phenot

297 METHODS AND A clinically affected trio with HCM underwent clinical evaluation, electrocardiography,

298 ed the considerable mortality rate for young HCM patients remains unresolved.

299 In a large hospital-based cohort of young HCM patients, representing an age group considered at gr

300 adjusted analysis was undertaken in younger HCM patients and compared with ATH with left ventricular