Patients’ Characteristics and Clinical Course of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From Kochi RYOMA Study ―

Kubo, Toru; Hirota, Takayoshi; Baba, Yuichi; Ochi, Yuri; Takahashi, A; Yamasaki, Naohito; Hamashige, Naohisa; Yamamoto, Katsuhito; Kondo, Fumiaki; Bando, Kanji; Yamada, Eisuke; Furuno, Takashi; Yabe, Toshikazu; Doi, Yoshinori; Kitaoka, Hiroaki

doi:10.1253/circj.cj-17-0845

Cited by 23 publications

(28 citation statements)

References 32 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Recently, we reported that the survival rate in this cohort was favorable with an HCM-related annual mortality rate of 1.3%. 10 In the present study, we focused on SCD-relevant events and found that the annual mortality rate of SCD was 0.5% and that the rate of SCD-relevant events was 1.0% per year; these findings are in accordance with previously reported data obtained from community-based cohorts for HCM in Western countries. 4,5 Although SCD-relevant events have been considered to be closely associated with young age in HCM patients, most of the patients (89%) in the present HCM population experienced SCD-relevant events after 50 years of age.…”

Section: Regional Hcm Cohort and Scd-relevant Eventssupporting

confidence: 92%

“…Regarding risk factors for overall HCM-related adverse events in this HCM population, we have already reported that NYHA Class III, the presence of AF, LVOT obstruction, and lower LV FS at registration are significant predictors. 10 In the present study, multivariate analysis revealed that the presence of NSVT and D-HCM (at registration or during follow-up) were significant predictors of SCDrelevant events. These 2 factors are known to be associated with SCD.…”

Section: Predictors Of Scd-relevant Eventssupporting

confidence: 49%

See 1 more Smart Citation

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From the Kochi RYOMA Study ―

et al. 2020

Self Cite

View full text Add to dashboard Cite

Background: Sudden cardiac death (SCD) is a most devastating complication of hypertrophic cardiomyopathy (HCM). The aim of this study was to clarify the clinical features of HCM in patients who experienced SCD-relevant events in an aged Japanese community. Methods and Results: In 2004, we established a cardiomyopathy registration network in Kochi Prefecture, and herein report on 293 patients with HCM who are followed as part of the registry. The mean (±SD) age at registration and diagnosis was 63±14 and 56±16 years, respectively. SCD-relevant events occurred in 19 patients during a mean follow-up period of 6.1±3.2 years (incidence rate 1.0%/year): sudden death in 9 patients, successful recovery from cardiopulmonary arrest in 4 patients, and appropriate implantable cardioverter–defibrillator discharge in 6 patients. At registration, 13 patients were in the dilated phase of HCM (D-HCM). During the follow-up period, HCM developed to D-HCM in 21 patients; thus, 34 patients in total had D-HCM. Multivariate analysis revealed that D-HCM at registration or during follow-up and detection of non-sustained ventricular tachycardia (NSVT) during follow-up were significant predictors of SCD-relevant events. Conclusions: In this HCM population in an aged Japanese community, the annual rate of SCD-relevant events was 1.0%. HCM developed to D-HCM in a considerable number of patients, and D-HCM and NSVT were shown to be independently associated with an increased risk of SCD-relevant events.

show abstract

Section: Regional Hcm Cohort and Scd-relevant Eventssupporting

confidence: 92%

Section: Predictors Of Scd-relevant Eventssupporting

confidence: 49%

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From the Kochi RYOMA Study ―

et al. 2020

Self Cite

View full text Add to dashboard Cite

show abstract

“…In echocardiographic examination, echocardiographic findings including severity of LVH and HCM subtypes were assessed by previously reported methods and definitions. 5 Survival and clinical data were collected during serial clinical visits. The study closed on December 31, 2017.…”

Section: Genetic Diagnosismentioning

confidence: 99%

Lifelong Clinical Impact of the Presence of Sarcomere Gene Mutation in Japanese Patients With Hypertrophic Cardiomyopathy

Nakashima

Kubo

Sugiura

et al. 2020

Circ J

Self Cite

View full text Add to dashboard Cite

Background: Hypertrophic cardiomyopathy (HCM) is mainly caused by mutations in sarcomere genes. Regarding the clinical implications of genetic information, little is known about the lifelong clinical effect of sarcomere mutations in Japanese HCM patients. Methods and Results: We studied 211 consecutive Japanese patients with HCM who had agreed to genetic testing between 2003 and 2013. Genetic analyses were performed by direct DNA sequencing in the 6 common sarcomere genes (MYH7, MYBPC3, TNNT2, TNNI3, TPM1, ACTC). Through variant filtering, 21 mutations were identified in 67 patients. After excluding 8 patients whose variants were determined as having uncertain significance, finally 203 patients (130 men, age at study entry: 61.8±14.1 years) were investigated for clinical presentation and course. At the time of study entry, patients with mutations were younger, had more frequent non-sustained ventricular tachycardia, had greater interventricular wall thickness, were more frequently in the dilated phase and less frequently had apical HCM. Through their lifetimes, a total of 98 HCM-related morbid events occurred in 72 patients. Survival analysis revealed that patients with sarcomere gene mutations experienced those morbid events significantly more frequently, and this tendency was more prominent for lethal arrhythmic events. Conclusions: In our HCM cohort, patients with sarcomere gene mutations had poorer lifelong outcome. Genetic information is considered important for better management of HCM.

show abstract

“…Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder that is generally associated with mild disability and normal life expectancy if sudden death can be prevented. [1][2][3][4][5] On the other hand, it is well known that HCM in a subset of patients progresses to 'end-stage' or 'dilated' phase characterized by left ventricular (LV) systolic dysfunction, and patients with the end-stage phase of HCM (end-stage HCM) have a poor prognosis. [6][7][8][9][10] It is important to predict the clinical course in order to provide adequate interventions for those patients.…”

Section: Introductionmentioning

confidence: 99%

Elevation of high‐sensitivity cardiac troponin T and left ventricular remodelling in hypertrophic cardiomyopathy

et al. 2020

Self Cite

View full text Add to dashboard Cite

Aims Hypertrophic cardiomyopathy (HCM) is generally associated with mild disability and normal life expectancy. On the other hand, once the end-stage phase of HCM characterized by left ventricular (LV) ejection fraction < 50% is established, patients with this subtype have a poor prognosis. This study clarifies the clinical parameters associated with progression to end-stage HCM. Methods and results We retrospectively studied 157 HCM patients (age 59.9 ± 14.2 years, 104 men) with preserved LV systolic function in whom subsequent echocardiographic data were obtained for a period of >1 year. HCM progressed to end-stage HCM in 13 patients (8.3%) of the 157 patients during a mean follow-up period of 6.3 ± 2.8 years. Compared with patients who did not reach end-stage HCM at the last evaluation, patients with progression to the end-stage phase had lower ejection fraction, larger LV size, more enlarged left atrial diameter, longer follow-up period, and higher frequency of an elevated concentration of high-sensitivity cardiac troponin T (hs-cTnT; >0.014 ng/mL) at registration. Multivariate analysis revealed that elevated hs-cTnT was a significant predictor independent of lower LV ejection fraction for progression to end-stage HCM. Furthermore, in patients with elevated hs-cTnT levels, LV ejection fraction became significantly lower, LV end-diastolic diameter increased, and LV wall thickness decreased during the follow-up period, whereas those parameters did not change in the normal hs-cTnT group. Conclusions In patients with HCM, an elevated hs-cTnT was associated with progression of LV remodelling, and this biomarker can be useful for predicting progression to the end-stage phase.

show abstract

Patients’ Characteristics and Clinical Course of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From Kochi RYOMA Study ―

Cited by 23 publications

References 32 publications

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From the Kochi RYOMA Study ―

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From the Kochi RYOMA Study ―

Lifelong Clinical Impact of the Presence of Sarcomere Gene Mutation in Japanese Patients With Hypertrophic Cardiomyopathy

Elevation of high‐sensitivity cardiac troponin T and left ventricular remodelling in hypertrophic cardiomyopathy

Contact Info

Product

Resources

About

Patients’ Characteristics and Clinical Course of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort ― Results From Kochi RYOMA Study ―

Cited by 23 publications

References 32 publications

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort ― Results From the Kochi RYOMA Study ―

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort ― Results From the Kochi RYOMA Study ―

Lifelong Clinical Impact of the Presence of Sarcomere Gene Mutation in Japanese Patients With Hypertrophic Cardiomyopathy

Elevation of high‐sensitivity cardiac troponin T and left ventricular remodelling in hypertrophic cardiomyopathy

Contact Info

Product

Resources

About

Patients’ Characteristics and Clinical Course of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From Kochi RYOMA Study ―

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From the Kochi RYOMA Study ―

Sudden Cardiac Death-Relevant Events of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　― Results From the Kochi RYOMA Study ―