著者
Shuji Moriyama Masahiko Hara Takafumi Hirota Kosuke Nakata Hideki Doi Toshiyuki Matsumura Yushi Araki Yukihiro Inomata Toshihiro Fukui
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-23-0076, (Released:2023-09-07)
参考文献数
29
被引用文献数
1

Background: Acute aortic dissection (AAD) has high morbidity and a high fatality rate for a cardiovascular disease. Recent studies suggested that the incidence of AAD is increasing. However, the actual incidence and mortality rates of AAD are not well known. This study investigated the current epidemiology of AAD within the Yatsushiro medical jurisdictional area.Methods and Results: A population-based review of patients with AAD was performed in a geographically well-defined area. Data were collected retrospectively from January 2011 to December 2020 for a total of 196 patients with AAD (Stanford Type A, n=126 [64.3%]; Stanford Type B, n=70 [35.7%]). The mean patient age was 74.3 years, and 55.6% (109/196) were women. The crude and age-standardized incidence rates of AAD in our medical jurisdictional area were 13.6 and 11.4 per 100,000 inhabitants per year, respectively. The crude and age-standardized 30-day mortality rates of AAD were 4.9 and 4.0 per 100,000 inhabitants per year, respectively. There were upward tendencies for both the incidence and 30-day mortality rate of AAD with age, with both being significantly higher in patients aged ≥85 years (P<0.001).Conclusions: This population-based study detected a higher incidence of AAD than previous studies, but reported a lower incidence of AAD in men than in women. Increasing age was associated with an increased incidence and mortality rate of AAD.
著者
Seiji Takashio Teiji Kato Hikaru Tashima Hiroki Irie Yoshihiro Komohara Tetsuya Oguni Kei Morikawa Naoto Kuyama Noriaki Tabata Shinsuke Hanatani Eiichiro Yamamoto Kenichi Matsushita Mitsuharu Ueda Kenichi Tsujita
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-23-0223, (Released:2023-06-13)
参考文献数
31
被引用文献数
2

Background: Carpal tunnel syndrome (CTS) is considered an early sign of cardiac amyloidosis (CA) because amyloid deposition is often confirmed in the tenosynovium removed during carpal tunnel release (CTR); however, the prevalence of concomitant CA is unclear.Methods and Results: We prospectively examined 700 patients who underwent CTR and evaluated amyloid deposition after tenosynovium removal. Amyloid deposition was observed in 261 (37%) patients, who were significantly older and predominantly male (P<0.05). Of them, 120 agreed to cardiac screening. We performed 99 mTc-labeled pyrophosphate (99 mTc-PYP) scintigraphy in 12 patients who met either of the following criteria: (1) interventricular septal diameter (IVSd) ≥14 mm or (2) 12 mm ≤ IVSd < 14 mm with above-normal limits in high-sensitivity cardiac troponin T (hs-cTnT). Six patients (50%) had positive findings on 99 mTc-PYP scintigraphy and were diagnosed with wild-type transthyretin CA. Concomitant CA was observed in 6/120 (5%) CTR patients with amyloid deposition and 50% (6/12) in patients with left ventricular hypertrophy (≥12 mm) with increased hs-cTnT levels.Conclusions: Amyloid deposition was frequently observed in the removed tenosynovium of elderly men with CTS. Cardiac screening may be useful for early diagnosis of CA in patients undergoing CTR with amyloid deposition.
著者
Shohei Yoshida Tomoaki Nakata Masanao Naya Mitsuru Momose Yasuyo Taniguchi Yoshimitsu Fukushima Masao Moroi Atsutaka Okizaki Akiyoshi Hashimoto Takatoyo Kiko Satoshi Hida Kazuya Takehana Kenichi Nakajima
出版者
The Japanese Circulation Society
雑誌
Circulation Reports (ISSN:24340790)
巻号頁・発行日
pp.CR-23-0031, (Released:2023-04-28)
参考文献数
25

Background: Definitions of cardiac sarcoidosis (CS) differ among guidelines. Any systemic histological finding of CS is essential for the diagnosis of CS in the 2014 Heart Rhythm Society statement, but not necessary in the Japanese Circulation Society 2016 guidelines. This study aimed to reveal the differences in outcomes by comparing 2 groups, namely CS patients with or without systemic histologically proven granuloma.Methods and Results: This study retrospectively included 231 consecutive patients with CS. CS with granulomas in ≥1 organs was diagnosed in 131 patients (Group G), whereas CS without any granulomas was diagnosed in the remaining 100 patients (Group NG). Left ventricular ejection fraction (LVEF) was significantly reduced in Group NG compared with Group G (44±13% vs. 50±16%, respectively; P=0.001). However, Kaplan-Meier curves showed that major adverse cardiovascular events (MACE)-free survival outcomes were comparable between the 2 groups (log-rank P=0.167). Univariable analyses showed that significant predictors of MACE were Groups G/NG, histological CS, LVEF, and high B-type natriuretic peptide (BNP) or N-terminal pro BNP concentrations, but none of these was significant in multivariable analyses.Conclusions: Overall risks of MACE were similar between the 2 groups despite different manifestations in cardiac dysfunction. The data not only validate the prognostic value of non-invasive diagnosis of CS, but also show the need for careful observation and therapeutic strategy in patients with CS without any granuloma.
著者
Makoto Murata Saya Yanai Shogo Nitta Yuhei Yamashita Tatsunori Shitara Hiroko Kazama Masanori Ueda Yasuyuki Kobayashi Yoshihisa Namasu Hitoshi Adachi
出版者
The Japanese Circulation Society
雑誌
Circulation Reports (ISSN:24340790)
巻号頁・発行日
pp.CR-23-0040, (Released:2023-05-17)
参考文献数
33
被引用文献数
1

Background: The incidence of heart failure (HF) is increasing, and the mortality from HF remains high in an aging society. Cardiac rehabilitation (CR) programs (CRP) increase oxygen uptake (V̇O2) and reduce HF rehospitalization and mortality. Therefore, CR is recommended for every HF patient. However, the number of outpatients undergoing CR remains low, with insufficient attendance at CRP sessions. In this study we evaluated the outcomes of 3 weeks of inpatient CRP (3w In-CRP) for HF patients.Methods and Results: This study enrolled 93 HF patients after acute-phase hospitalization between 2019 and 2022. Patients participated in 30 sessions of 3w In-CRP (30 min aerobic exercise twice daily, 5 days/week). Before and after 3w In-CRP, patients underwent a cardiopulmonary exercise test, and cardiovascular (CV) events (mortality, HF rehospitalization, myocardial infarction, and cerebrovascular disease) after discharge were evaluated. After 3w In-CPR, mean (±SD) peak V̇O2increased from 11.8±3.2 to 13.7±4.1 mL/min/kg (116.5±22.1%). During the follow-up period (357±292 days after discharge), 20 patients were rehospitalized for HF, 1 had a stroke, and 8 died for any reasons. Proportional hazard and Kaplan-Meier analyses demonstrated that CV events were reduced among patients with a 6.1% improvement in peak V̇O2than in patients without any improvement in peak V̇O2.Conclusions: 3w In-CRP for HF patients improved peak V̇O2and reduced CV events in HF patients with a 6.1% improvement in peak V̇O2.
著者
Hajime Saeki Miho Kuramoto Yoshinori Iida Kaori Yasumura Yoh Arita Nobuyuki Ogasawara
出版者
The Japanese Circulation Society
雑誌
Circulation Reports (ISSN:24340790)
巻号頁・発行日
pp.CR-23-0024, (Released:2023-03-31)
参考文献数
28

Background: In outpatient center-based cardiac rehabilitation (O-CBCR), moderate-intensity continuous training (MICT) based on the anaerobic threshold (AT) determined by cardiopulmonary exercise stress testing is recommended. However, it is unclear whether differences in exercise intensity within the MICT domain affect peak oxygen uptake (%peakV̇O2).Methods and Results: We retrospectively evaluated patients who underwent O-CBCR at Japan Community Healthcare Organization Osaka Hospital. Those treated with the constant-load method were designated as Group A (n=38), whereas those treated with the variable-load method were designated as Group B (n=48). Although the change in exercise intensity was significantly greater in Group B by approximately 4.5 W, the change in %peakV̇O2was not significantly different between groups. Group A had a significantly longer exercise time than Group B (by approximately 4–5 min). No deaths or hospitalizations occurred in either group. The percentage of episodes with exercise cessation was similar between the 2 groups, but the percentage of episodes with load reduction was significantly higher in Group B, mostly because of the increased heart rate.Conclusions: In supervised MICT based on AT, the variable-load method increased exercise intensity more than the constant-load method without severe complications, but did not improve %peakV̇O2.
著者
Yuki Ikeda Junya Ako Koichi Toda Atsushi Hirayama Koichiro Kinugawa Yoshio Kobayashi Minoru Ono Takashi Nishimura Naoki Sato Takahiro Shindo Morimasa Takayama Satoshi Yasukochi Akira Shiose Yoshiki Sawa J-PVAD Investigators
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.87, no.5, pp.588-597, 2023-04-25 (Released:2023-04-25)
参考文献数
30
被引用文献数
16

Background: The Impella®percutaneous left ventricular assist device has been available in Japan since 2017. This is the first large-scale registry study to analyze the efficacy and safety of Impella in Japanese patients with acute myocardial infarction with cardiogenic shock (AMICS).Methods and Results: The Japanese registry for Percutaneous Ventricular Assist Device (J-PVAD) has registered all consecutive Japanese patients treated with Impella. We extracted data for 593 AMICS patients from J-PVAD and analyzed 30-day survival and safety profiles. Overall 30-day survival was 63.1%. The 30-day survival of the Impella alone and Impella plus venoarterial extracorporeal membrane oxygenation (ECPELLA) groups was 80.9% and 45.7%, respectively. The Impella alone group was older and had a lower rate of cardiac arrest, milder consciousness disturbance, less inotrope use, lower serum lactate concentrations, higher B-type natriuretic peptide concentrations, and higher left ventricular ejection fraction (LVEF) than the ECPELLA group. Cox regression analysis revealed that older age and comorbid renal disturbance were common risk factors affecting 30-day mortality in both groups. Major adverse events were hemolysis (10.8%), hemorrhage/hematoma (7.6%), peripheral ischemia (4.4%), stroke (1.3%), and thrombosis (0.7%). LVEF improved in both groups during support.Conclusions: AMICS treatment with Impella showed favorable 30-day survival and safety profiles. The survival rate of patients treated with Impella alone was particularly high. Further studies are needed to improve outcomes of patients with ECPELLA support.
著者
Rie Hayashi Hiroyasu Iso Kazumasa Yamagishi Hiroshi Yatsuya Isao Saito Yoshihiro Kokubo Ehab S. Eshak Norie Sawada Shoichiro Tsugane for the Japan Public Health Center-Based (JPHC) Prospective Study Group
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.83, no.5, pp.1072-1079, 2019-04-25 (Released:2019-04-25)
参考文献数
43
被引用文献数
24 26

Background: Evidence from prospective cohort studies regarding the relationship between working hours and risk of cardiovascular disease is limited Methods and Results: The Japan Public Health Center-Based Prospective Study Cohort II involved 15,277 men aged 40–59 years at the baseline survey in 1993. Respondents were followed up until 2012. During the median 20 years of follow up (257,229 person-years), we observed 212 cases of acute myocardial infarction and 745 stroke events. Cox proportional hazards models adjusted for sociodemographic factors, cardiovascular risk factors, and occupation showed that multivariable-adjusted hazard ratios (HRs) associated with overtime work of ≥11h/day were: 1.63 (95% confidence interval [CI] 1.01–2.63) for acute myocardial infarction and 0.83 (95% CI 0.60–1.13) for total stroke, as compared with the reference group (working 7 to <9 h/day). In the multivariable model, increased risk of acute myocardial infarction associated with overtime work of ≥11 h/day was more evident among salaried employees (HR 2.11, 95% CI 1.03–4.35) and men aged 50–59 years (HR 2.60, 95% CI 1.42–4.77). Conclusions: Among middle-aged Japanese men, working overtime is associated with a higher risk of acute myocardial infarction.
著者
Takahiro Kamihara Akihiro Hirashiki Manabu Kokubo Atsuya Shimizu
出版者
The Japanese Circulation Society
雑誌
Circulation Reports (ISSN:24340790)
巻号頁・発行日
pp.CR-22-0130, (Released:2023-03-24)
参考文献数
41
被引用文献数
2

Background: Autophagy may contribute to the maintenance of atrial fibrillation (AF), but no previous study has concurrently surveyed all 3 phases of autophagy, namely autophagosome formation, lysosome formation, and autophagosome-lysosome fusion. Here we aimed to identify disorders involving various phases of autophagy during AF.Methods and Results: We used bioinformatic techniques to analyze publicly available DNA microarray datasets from the left atrium (LA) and right atrium (RA) of 7 patients with AF and 6 patients with normal sinus rhythm who underwent valvular surgeries. We compared gene expression levels in the LA (AF-LA) and RA of patients with AF with those in the LA and RA of patients with normal sinus rhythm. Several differentially expressed genes in the AF-LA sample were significantly associated with the Gene Ontogeny term ‘Autophagy’, indicating that the expression of autophagic genes was specifically altered in this dataset. In particular, the expression of genes known or suspected to be involved in autophagosome formation (autophagy related 5 [ATG5], autophagy related 10 [ATG10], autophagy related 12 [ATG12], and light chain 3B [LC3B]), lysosome formation (lysosomal associated membrane protein 1 [LAMP1] and lysosomal associated membrane protein 2 [LAMP2]), and autophagosome-lysosome fusion (synaptosome associated protein 29 [SNAP29], SNAP associated protein [SNAPIN], and syntaxin 17 [STX17]) was significantly upregulated in the LA-AF dataset.Conclusions: Autophagy is activated excessively in, and may perpetuate, AF.
著者
Yuki Ikeda Junya Ako Koichi Toda Atsushi Hirayama Koichiro Kinugawa Yoshio Kobayashi Minoru Ono Takashi Nishimura Naoki Sato Takahiro Shindo Morimasa Takayama Satoshi Yasukochi Akira Shiose Yoshiki Sawa J-PVAD Investigators
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-22-0476, (Released:2023-01-20)
参考文献数
30
被引用文献数
16

Background: The Impella®percutaneous left ventricular assist device has been available in Japan since 2017. This is the first large-scale registry study to analyze the efficacy and safety of Impella in Japanese patients with acute myocardial infarction with cardiogenic shock (AMICS).Methods and Results: The Japanese registry for Percutaneous Ventricular Assist Device (J-PVAD) has registered all consecutive Japanese patients treated with Impella. We extracted data for 593 AMICS patients from J-PVAD and analyzed 30-day survival and safety profiles. Overall 30-day survival was 63.1%. The 30-day survival of the Impella alone and Impella plus venoarterial extracorporeal membrane oxygenation (ECPELLA) groups was 80.9% and 45.7%, respectively. The Impella alone group was older and had a lower rate of cardiac arrest, milder consciousness disturbance, less inotrope use, lower serum lactate concentrations, higher B-type natriuretic peptide concentrations, and higher left ventricular ejection fraction (LVEF) than the ECPELLA group. Cox regression analysis revealed that older age and comorbid renal disturbance were common risk factors affecting 30-day mortality in both groups. Major adverse events were hemolysis (10.8%), hemorrhage/hematoma (7.6%), peripheral ischemia (4.4%), stroke (1.3%), and thrombosis (0.7%). LVEF improved in both groups during support.Conclusions: AMICS treatment with Impella showed favorable 30-day survival and safety profiles. The survival rate of patients treated with Impella alone was particularly high. Further studies are needed to improve outcomes of patients with ECPELLA support.
著者
Kensuke Takabayashi Tomoyuki Hamada Toru Kubo Kotaro Iwatsu Tsutomu Ikeda Yohei Okada Tetsuhisa Kitamura Shouji Kitaguchi Takeshi Kimura Hiroaki Kitaoka Ryuji Nohara
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-22-0652, (Released:2022-12-28)
参考文献数
22
被引用文献数
1

Background: To predict mortality in patients with acute heart failure (AHF), we created and validated an internal clinical risk score, the KICKOFF score, which takes physical and social aspects, in addition to clinical aspects, into account. In this study, we validated the prediction model externally in a different geographic area.Methods and Results: There were 2 prospective multicenter cohorts (1,117 patients in Osaka Prefecture [KICKOFF registry]; 737 patients in Kochi Prefecture [Kochi YOSACOI study]) that had complete datasets for calculation of the KICKOFF score, which was developed by machine learning incorporating physical and social factors. The outcome measure was all-cause death over a 2-year period. Patients were separated into 3 groups: low risk (scores 0–6), moderate risk (scores 7–11), and high risk (scores 12–19). Kaplan-Meier curves clearly showed the score’s propensity to predict all-cause death, which rose independently in higher-risk groups (P<0.001) in both cohorts. After 2 years, the cumulative incidence of all-cause death was similar in the KICKOFF registry and Kochi YOSACOI study for the low-risk (4.4% vs. 5.3%, respectively), moderate-risk (25.3% vs. 22.3%, respectively), and high-risk (68.1% vs. 58.5%, respectively) groups.Conclusions: The unique prediction score may be used in different geographic areas in Japan. The score may help doctors estimate the risk of AHF mortality, and provide information for decisions regarding heart failure treatment.
著者
Ryuichi Matsukawa Hirohide Matsuura Masaki Tokutome Arihide Okahara Ayano Hara Kousuke Okabe Shunsuke Kawai Yasushi Mukai
出版者
The Japanese Circulation Society
雑誌
Circulation Reports (ISSN:24340790)
巻号頁・発行日
vol.4, no.8, pp.345-352, 2022-08-10 (Released:2022-08-10)
参考文献数
32

Background: Acute coronary syndrome (ACS) patients with solid lesions often require predilatation before stenting. Predilatation with high pressure may increase the risk of distal embolism, whereas direct stenting increases the risk of stent underexpansion. We recently reported that, in severely calcified lesions, using a cutting balloon (CB) can provide greater acute gain compared with other scoring balloons. Therefore, we hypothesized that predilatation with CB may reduce the incidence of distal embolism in ACS patients with solid lesions.Methods and Results: This study retrospectively analyzed data for 175 ACS patients who required predilatation, either with a conventional balloon (n=136) or CB (n=39). The occurrence of distal embolism was significantly lower in the CB than conventional balloon group (10.3% vs 32.4%, respectively; P=0.007). Multivariate analysis showed that the occurrence of distal embolism was positively associated with Thrombolysis in Myocardial Infarction (TIMI) grade and the presence of attenuated plaque, but negatively associated with the use of a CB. To support this clinical observation, we compared thrombus dispersal using a CB and non-compliant balloon in an ex vivo experimental model using a pseudo-thrombus. In this model, pseudo-thrombus dispersal was significantly smaller when a CB rather than non-compliant balloon was used (1.8±1.0% vs 2.6±1.2%, respectively; n=20, for each; P=0.002).Conclusions: In ACS patients with solid lesions that require predilatation, predilatation with a CB may reduce the incidence of distal embolism.
著者
Akihiko Nogami Kyoko Soejima Itsuro Morishima Kenichi Hiroshima Ritsushi Kato Satoru Sakagami Fumiharu Miura Keisuke Okawa Tetsuya Kimura Takashi Inoue Atsushi Takita Kikuya Uno Koichiro Kumagai Takashi Kurita Masahiko Gosho Kazutaka Aonuma for the RYOUMA Investigators
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-22-0290, (Released:2022-08-20)
参考文献数
41
被引用文献数
6

Background: Optimal periprocedural oral anticoagulant (OAC) therapy before catheter ablation (CA) for atrial fibrillation (AF) and the safety profile of OAC discontinuation during the remote period (from 31 days and up to 1 year after CA) have not been well defined.Methods and Results: The RYOUMA registry is a prospective multicenter observational study of Japanese patients who underwent CA for AF in 2017–2018. Of the 3,072 patients, 82.3% received minimally interrupted direct-acting OACs (DOACs) and 10.2% received uninterrupted DOACs. Both uninterrupted and minimally interrupted DOACs were associated with an extremely low thromboembolic event rate. Female, long-standing persistent AF, low creatinine clearance, hepatic disorder, and high intraprocedural heparin dose were independent factors associated with periprocedural major bleeding. At 1 year after CA, DOAC was continued in 55.9% of patients and warfarin in 56.4%. The incidence of thromboembolic and major bleeding events for 1 year was 0.3% and 1.2%, respectively. Age ≥73 years, dementia, and AF recurrence were independently associated with major bleeding events. Univariate analyses revealed that warfarin continuation and off-label overdose of DOACs were risk factors for major bleeding after CA.Conclusions: High intraprocedural dose of heparin was associated with periprocedural major bleeding events. At 1 year after CA, over half of the patients had continued OAC therapy. Thromboembolic events were extremely low; however, major bleeding occurred in 1.2%. Age ≥73 years, dementia, and AF recurrence were independently associated with major bleeding after CA.
著者
Mitsutaka Nakashima Machiko Tanakaya Toru Miyoshi Takaaki Saito Yusuke Katayama Satoru Sakuragi Hiroshi Ito
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-22-0109, (Released:2022-08-04)
参考文献数
34
被引用文献数
6

Background: The fibrosis-4 (FIB-4) index is used to evaluate liver disease patients. It can also be used to evaluate the prognosis for heart disease patients; however, its ability to determine the prognosis of severe isolated tricuspid regurgitation (TR) patients is unclear. This study aimed to clarify the association between FIB-4 index scores and the cardiovascular prognosis for severe isolated TR patients.Methods and Results: This was a dual-center, retrospective study. From 2011 to 2019, 111 consecutive outpatients with severe isolated TR (mean age, 68.6 years; 53.2% male) were evaluated. Major adverse cardiovascular events (MACEs) were defined as the composite of cardiovascular death, hospitalization for heart failure, myocardial infarction, and stroke. The association between FIB-4 index scores and echocardiography was also evaluated. During a median follow up of 3.0 years, 24 patients were lost to follow up and 40 MACEs occurred. Baseline FIB-4 index scores for patients with MACEs were significantly higher than those for patients without MACEs. A multivariate analysis revealed that FIB-4 index scores are significantly associated with MACEs (hazard ratio, 1.89; 95% confidence interval, 1.01–3.54; P=0.046). A linear regression analysis indicated that FIB-4 index scores were correlated with echocardiographic parameters, including the left atrial volume index and left ventricular end-diastolic diameter.Conclusions: The FIB-4 index score may be a useful predictor of MACEs for patients with severe isolated TR.
著者
Hayato Tada Hirofumi Okada Atsushi Nohara Masakazu Yamagishi Masayuki Takamura Masa-aki Kawashiri
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
vol.85, no.11, pp.2073-2078, 2021-10-25 (Released:2021-10-25)
参考文献数
23
被引用文献数
1 24

Background:Recent studies suggest that cumulative exposure to low-density lipoprotein-cholesterol (LDL-C) leads to the development of atherosclerotic cardiovascular disease (ASCVD). However, few studies have investigated whether this link extends to individuals with familial hypercholesterolemia (FH), a relevant patient population.Methods and Results:We retrospectively investigated the health records of 1,050 patients with clinical FH diagnosis between April 1990 and March 2019. We used Cox proportional hazards models adjusted for established ASCVD risk factors to assess the association between cholesterol-year-score and major adverse cardiovascular events (MACEs), including death from any cause or hospitalization due to ASCVD events. Cholesterol-year-score was calculated as LDL-C max × [age at diagnosis/statin initiation] + LDL-C at inclusion × [age at inclusion − age at diagnosis/statin initiation]. The median follow-up period for MACE evaluation was 12.3 (interquartile range, 9.1–17.5) years, and 177 patients experienced MACEs during the observation period. Cholesterol-year-score was significantly associated with MACEs (hazard ratio, 1.35; 95% confidence interval, 1.07–1.53; P=0.0034, per 1,000 mg-year/dL), independent of other traditional risk factors including age and LDL-C, based on cross-sectional assessment. Cholesterol-year-score improved the discrimination ability of other traditional risk factors for ASCVD events (C-index, 0.901 vs. 0.889; P=0.00473).Conclusions:Cumulative LDL-C exposure was strongly associated with MACEs in Japanese patients with FH, warranting early diagnosis and treatment initiation in these patients.