Clinical features of quadricuspid aortic valve in middle-aged and elderly patients: Insights from a regional study.

BACKGROUND: Quadricuspid aortic valve (QAV) is a rare congenital disease. The clinical characteristics of this disease remain unclear except for those in relatively young patients reported from tertiary referral hospitals. The aim of this study was to determine the clinical features of QAV in a regional population. METHODS AND RESULTS: We retrospectively investigated 25 340 consecutive patients over middle age (median age, 73 (IQR 65-80) years; range, 45-102 years) who underwent transthoracic echocardiography (TTE) at our institute during the period from April 2008 to December 2023. Eight (0.032%) of the patients (median age, 65 years; range, 47-91 years) were diagnosed with QAV. Six patients suffered from aortic regurgitation (AR), and one patient had mild aortic stenosis at the time of QAV diagnosis. Two patients who had severe AR at referral underwent aortic valve surgery. The severity of AR in the other patients was moderate or less. During a median follow-up period of 27 months (range, 1-171 months), none of the patients other than above two patients had cardiac events. One patient died from a non-cardiac cause at 94 years of age. CONCLUSIONS: Patients diagnosed with QAV after middle age, who do not exhibit severe valve insufficiency at the time of diagnosis, may not experience worse clinical outcomes. However, further research is required for a better understanding of the long-term outcomes.

Genetic Testing for Cardiomyopathy in Japan 2022: Current Status and Issues of Precision Medicine.

Although many causative genes for primary cardiomyopathy have been identified, the use of genetic testing in routine practice is limited in Japan presently. Genetic diagnosis has been reported to be useful for early diagnosis through cascade genetic screening in the family, differentiating secondary cardiomyopathies, and predicting prognosis in some patients; nonetheless, the acquisition of genetic information for cardiomyopathy is stagnating in actual clinical practice. There seem to be a number of reasons for this phenomenon, and although the use of next-generation sequencers has resolved some of the past issues, the importance of pathogenicity studies of variants that are identified is growing. To ensure that patients with cardiomyopathy and their relatives can receive precision medicine, the results of genetic analysis linked to clinical information need to be collected, and a database of variants in Japanese people needs to be established.

External Validation of the Japanese Clinical Score for Mortality Prediction in Patients With Acute Heart Failure.

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.

Association between simple test assessing hand dexterity and mild cognitive impairment in independent older adults.

OBJECTIVES: This study aimed to clarify the association between hand function, including grip strength and hand dexterity, assessed using snap fasteners, and mild cognitive impairment (MCI) in older adults with normal global cognitive function. METHODS: A total of 228 functionally independent older adults (mean age 77.7 ± 6.1 years) participated in this study. None of the participants had a history of dementia diagnosis, and all the participants had a Mini-Mental State Examination score of ≥24. Participants were evaluated for hand function using grip strength and snap fastener tests, and for cognitive function using the National Center for Geriatrics and Gerontology-Functional Assessment Tool. RESULTS: A total of 72 participants (31.6%) were diagnosed with MCI. The slow snap fastener test group had a higher proportion of participants with MCI (p < 0.001) and impairments in memory (p = 0.010), attention (p = 0.043), executive function (p < 0.001), and processing speed (p = 0.044) compared to the fast snap fastener test group. The slow speed of fastening snap fasteners was significantly associated with MCI and impairment in memory, attention, executive function, and processing speed (MCI: adjusted odds ratio (AOR) = 3.88, 95% confidence interval (CI) = 1.64-9.19; memory: AOR = 5.73, 95% CI = 1.58-20.82; attention: AOR = 3.95, 95% CI = 1.10-14.11; executive function: AOR = 7.22, 95% CI = 1.78-29.24; processing speed: AOR = 7.52, 95% CI = 1.19-47.66) according to the multiple logistic regression analysis. Grip strength was not significantly associated with cognitive impairment. CONCLUSIONS: Thus, hand dexterity assessed using the snap fastener test was associated with MCI in older adults with normal global cognitive function. Hand dexterity assessment using the snap fastener test is useful for detecting MCI in apparently healthy older adults.

Changes in Exercise Tolerance over Time in Patients with Transthyretin Amyloidosis Cardiomyopathy Treated with Tafamidis.

Tafamidis improves the prognosis of patients with transthyretin amyloidosis cardiomyopathy (ATTR-CM). Additionally, it delays the decline in exercise tolerance, as observed in the six-minute walking test. However, the changes in cardiopulmonary function over time based on cardiopulmonary exercise tests are unclear. Thus, this preliminary study investigated the changes in exercise tolerance after one year of tafamidis treatment using cardiopulmonary exercise testing. Eight patients with ATTR-CM (average age: 77 years; male: n = 7) underwent cardiopulmonary exercise testing at baseline and after one year of tafamidis treatment. All eight patients completed a one-year follow-up. At baseline, the anaerobic threshold oxygen uptake (AT VO2: 10.9 ± 1.5) and peak VO2 (14.3 ± 3.0 mL/kg/minute) indicated relatively favorable exercise capacity; however, the minute ventilation/carbon dioxide production (VE/VCO2 slope), which indicates effective ventilation, showed poor performance (33.7 ± 12.8). One year after tafamidis treatment, frailty, as assessed by the Clinical Frailty Scale, had progressed in seven of eight patients (88%) (P < 0.01), and AT VO2 and peak VO2 were significantly reduced (19.2% and 22.3%, respectively; P < 0.05). The VE/VCO2 slope and peak O2 pulse decreased nonsignificantly by approximately 20% (P = 0.47, and P = 0.16, respectively). Further, the structure of the ventricles and atrium and the left ventricle ejection fraction on echocardiography did not change. Thus, exercise tolerance in patients with ATTR-CM was reduced after one year despite tafamidis administration. Not only ATTR-CM progression, but also frailty progression may influence this decrease in exercise tolerance. A comprehensive approach, including tafamidis administration and cardiac rehabilitation, is required for further improvement in the exercise capacity of patients with ATTR-CM.

Assisted Living and Medication Adherence in Super-aged Patients With Heart Failure in the Japanese Population.

ABSTRACT: Poor adherence to medication in patients with heart failure (HF) is associated with poor clinical outcomes. Although social support has been reported to improve medication adherence in patients with HF, the detailed underlying mechanism of this association is unclear. This study investigated appropriate social support types to ensure medication adherence, as well as patient characteristics that benefit from such social support in patients with HF. This was a retrospective observational study investigating the association of social support with medication adherence in 824 patients with HF who were registered in a prospective multicenter database. First, we analyzed the association between social support types and poor medication adherence leading to hospitalization. An interaction analysis was performed to detect patients' characteristics that benefited most from social support in terms of medical adherence. Fifty patients (6.1%) were hospitalized for poor adherence to medications. Multivariable analysis revealed that not receiving assisted living, which was defined as having supporting individuals at least once a week, was independently associated with poor medication adherence-related hospitalization. An interaction analysis revealed that patients with dementia benefited from assisted living significantly, whereas male patients or current smokers did not. Summarily, assisted living at least once a week was appropriate for improving medication adherence in patients with HF and was particularly effective in patients with dementia. Performed in a super-aging region in Japan, this study may also suggest the relevance of social support in preventing HF exacerbation in other developed countries that will experience an aging society in the near future.

Early Experience of Tafamidis Treatment in Japanese Patients With Wild-Type Transthyretin Cardiac Amyloidosis From the Kochi Amyloidosis Cohort.

BACKGROUND: Tafamidis has emerged as an effective treatment for patients with wild-type transthyretin cardiac amyloidosis (ATTRwt CA). The early experience of tafamidis treatment for Japanese patients with ATTRwt CA is reported here.Methods and Results: Over the past 2 years, in 82 patients with ATTRwt CA (mean age of 81.7±6.0 years), tafamidis treatment was initiated for 38 patients. The remaining 44 patients were not administered tafamidis. The most frequent reason for non-administration of tafamidis was advanced heart failure and the second most reason was the patient's frailty. In patients who received tafamidis treatment, there was no discontinuation of tafamidis due to adverse events, the rate of cardiovascular-related hospitalizations per year was 0.19, and the 1-year survival rate was 92%. In the patients who continued tafamidis for 12-18 months, there was no significant deterioration from baseline for high-sensitivity cardiac troponin T level, plasma B-type natriuretic peptide level, left ventricular ejection fraction, inter-ventricular septum wall thickness, or value of left ventricular longitudinal strain. CONCLUSIONS: Tafamidis treatment was introduced for approximately half of the study patients with ATTRwt CA in real-world practice. Tafamidis is likely to be safe and may maintain the status of disease severity in the short-term in selected Japanese patients with ATTRwt CA. Further research is needed to determine appropriate patient selection for tafamidis treatment and efficacy of tafamidis in the long term.

Clinical Features of Heart Failure in Patients With Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　- Results From the Kochi RYOMA Study.

BACKGROUND: The clinical features of heart failure (HF) in patients with hypertrophic cardiomyopathy (HCM) in Japan have not been fully elucidated.Methods and Results: In 293 patients with HCM (median age at registration, 65 (57-72) years) in a prospective cardiomyopathy registration network in Kochi Prefecture (Kochi RYOMA study), HF events (HF death or hospitalization for HF) occurred in 35 patients (11.9%) (median age, 76 (69-80) years), including 11 HF deaths during a median follow-up of 6.1 years. The 5-year HF events rate was 9.6%. Atrial fibrillation, low percentage of fractional shortening, and high B-type natriuretic peptide level at registration were predictors of HF events. The combination of these 3 factors had a relatively high positive predictive value (55%) for HF events and none of them had a high negative predictive value (99%). There were 4 types of HF profile: left ventricular (LV) systolic dysfunction (40%), severe LV diastolic dysfunction (34%), LV outflow tract obstruction (LVOTO) (20%), and primary mitral regurgitation (MR) (6%). HF deaths occurred in patients with LV systolic dysfunction or LV diastolic dysfunction, but none of patients with LVOTO or primary MR due to additional invasive therapies. CONCLUSIONS: In a Japanese HCM cohort, HF was an important complication, requiring careful follow-up and appropriate treatment.

Safety of early rehabilitation in patients with aneurysmal subarachnoid hemorrhage: A retrospective cohort study.

OBJECTIVE: To investigate the safety and efficacy of early rehabilitation in patients with aneurysmal subarachnoid hemorrhage (aSAH) patients. METHODS: One hundred eleven patients with aSAH admitted between April 2015 and March 2019, were retrospectively evaluated. The early rehabilitation program was introduced in April 2017 to actively promote mobilization and walking training for aSAH patients. Therefore, patients were divided into two groups (The conventional group (n = 55) and the early rehabilitation group (n == 56). Clinical characteristics, mobilization progression, and treatment variables were analyzed. Complications (rebleeding, symptomatic cerebral vasospasm, hydrocephalus, disuse complications,) and a modified Rankin Scale (mRS) at 90 days were compared in two groups. Factors associated with favorable outcomes (mRS≤2) at 90 days were also assessed. RESULTS: The early rehabilitation group had a significantly shorter span to first walking (9 vs. 5 days; P = 0.007). The prevalence of complications was not significantly increased in the early rehabilitation group. Approximately 40% of patients in both groups had pneumonia and urinary tract infections but significantly reduced antibiotic-administration days (13 vs. 6 days; P < 0.001). mRS at 90 days also showed significant improvement in the early rehabilitation group (3 vs. 2; P=0.01). Multivariate logistic regression analysis of favorable outcomes associated that the administration of the early rehabilitation program has a significant independent factor (odds ratio, 3.03; 95% confidence interval, 1.1-8.37). CONCLUSIONS: Early rehabilitation for patients with aSAH can be feasible without increasing complication occurrences. The early rehabilitation program with active mobilization and walking training reduced antibiotic use and was associated with improved independence.

Incidence and Clinical Characteristics of Takotsubo Syndrome in Patients with Subarachnoid Hemorrhage.

Although takotsubo syndrome (TTS) has been reported in patients with subarachnoid hemorrhage (SAH), its incidence and relation to the severity of SAH are unknown.Of 319 consecutive patients with aneurysmal SAH, 245 patients who underwent both the ECG and echocardiography were analyzed.The incidence of TTS was 6.9% (22 patients (21 women), median age 68 years (range, 60-83) ). Regional wall motion abnormalities were present as apical (64%), mid-ventricular (9%), basal (4%) and focal (23%) forms. Heart failure was found in 10 patients (45%) but there was no cardiac death. Regarding SAH severity, 10 patients (45%) with TTS were in World Federation of Neurosurgical Societies classification grade V, as compared to 40 patients (18%) without TTS (P = 0.005). Seven patients (32%) with TTS died during hospitalization, as compared to 26 patients (12%) without TTS (P = 0.018). Four patients (18%) with TTS were estimated as independent at discharge, as compared to 100 patients (45%) without TTS (P = 0.013).The incidence of TTS in patients with SAH was estimated as 6.9% with significant predominance of women. The severity of SAH was significantly greater in patients with TTS than in those without TTS.

A Validation Study of the Mayo Clinic Phenotype-Based Genetic Test Prediction Score for Japanese Patients With Hypertrophic Cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder with an autosomal-dominant disorder mainly caused by mutations in sarcomere genes. Recently, a phenotype-based genetic test prediction score for patients with HCM was introduced by Mayo Clinic. The genotype score was derived on the basis of the predictive effect of 6 clinical markers, and the total score was shown to be correlated with the yield of genetic testing. However, it has not been determined whether this prediction model is useful in Japanese HCM patients.Methods and Results:The utility of the Mayo Clinic HCM genotype predictor score in 209 Japanese unrelated patients with a clinical diagnosis of HCM who had undergone genetic testing for 6 sarcomere genes was assessed. Overall, 55 patients (26%) had pathogenic or likely pathogenic variants (60% being genotype-positive in familial cases). We divided the patients into 6 groups (groups with scores of from -1 to 5) according to the prediction score. The yields of genetic testing in the groups with scores of -1, 0, 1, 2, 3, 4, and 5 were 8%, 16%, 24%, 48%, 50%, 100%, and 89%, respectively, with an incremental increase in yield between each of the score subgroups (P<0.001). CONCLUSIONS: The Mayo Clinic HCM genotype predictor score is useful for predicting a positive genetic test result in Japanese HCM Patients.

Association between self-reported night sleep duration and cognitive function among older adults with intact global cognition.

OBJECTIVES: The purpose of this study was to clarify the association between self-reported night sleep duration and cognitive functions such as word memory, story memory, attention, executive function, and processing speed of older adults with normal global cognitive function. METHODS: A total of 241 functionally independent older adults (mean age, 75.5 ± 6.4 years) participated in this study. No participants had a history of dementia diagnosis, and each had a Mini-Mental State Examination (MMSE) score ≥ 24. Participants were evaluated for self-reported sleep duration and cognitive function using the National Center for Geriatrics and Gerontology-Functional Assessment Tool (NCGG-FAT). RESULTS: The longest sleep duration group (≥9 h) had lower MMSE scores (p = 0.010), Z-score of word list memory II (delayed recall; p = 0.001), and Z-score of story memory II (delayed recognition; p = 0.002) than the medium sleep duration group (7-8 h). Longest and long sleep duration (8-9 h) was significantly associated with impairment of story memory II (longest sleep duration: adjusted odds ratio [OR] = 3.58, 95% confidence interval [CI] = 1.13-11.37, long sleep duration: adjusted OR = 4.30, 95% CI = 1.34-13.82) with reference to medium sleep duration, but no impairment of MMSE according to multiple logistic regression analysis. Furthermore, short sleep duration (<7 h) was not associated with cognitive impairment. CONCLUSIONS: This study suggests that long sleep duration is associated with cognitive impairment, especially of story memory (delayed recognition) in older adults with normal global cognitive function.

What we believed to be a right upper pulmonary vein by transthoracic echocardiography is actually a right lower pulmonary vein.

BACKGROUND: Current guidelines recommend that pulmonary vein (PV) velocity should be recorded by using the right upper pulmonary vein (RUPV) during transthoracic echocardiography (TTE) evaluation of left ventricular diastolic function. However, it is uncertain whether the PV displayed during TTE is truly measuring the upper PV. This study aimed to identify the actual site of each PV that is usually detected during TTE. METHODS: We retrospectively studied 105 patients who underwent cardiac computed tomography (CT) and TTE, reconstructed images three-dimensionally, and measured the angles between each PV and the left ventricle (LV) that would correspond to the Doppler incident angle of the apical four-chamber view on TTE. We also performed TTE during catheter ablation to confirm the exact site of the PV. RESULTS: Apical four-chamber views on TTE revealed that one certain PV was detectable on the right side of the vertebra. CT scans revealed that the median angle of the axes between the LV and right lower pulmonary vein (RLPV) was smaller than that of RUPV {32.1˚ [interquartile range (IQR): 21.7˚-42.1˚] vs. 62.5˚ (IQR: 51.6˚-70.6˚), P < .001}. During catheter ablation for treatment of atrial fibrillation, in the most well-displayed PV on TTE, we detected the ablation catheter placed in the RLPV. CONCLUSIONS: The most well-displayed PV in an apical four-chamber view by TTE was not the RUPV but the RLPV which showed the smallest angle of incidence toward the LV apex. The RLPV is suitable for evaluation of PV velocity to assess LV diastolic function.

Hypertrophic Cardiomyopathy　- A Heterogeneous and Lifelong Disease in the Real World.

Hypertrophic cardiomyopathy (HCM) is the most frequent hereditary cardiomyopathy, showing an autosomal-dominant f inheritance. A great deal of attention has been paid to genetics, left ventricular tract obstruction and the prediction and prevention of sudden cardiac death in HCM. Needless to say, these are very important, but we should recognize the heterogeneity in etiology, morphology, clinical course and management of this unique cardiomyopathy. Another important perspective is that HCM causes left ventricular remodeling over time and is a disease that requires lifelong management in the real world.

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

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.

Effects of Switching from Treatment with Amlodipine and Atorvastatin Using Two Pills to an Equal Dose of Single-Pill Therapy in Japanese Outpatients.

This study examined whether switching from amlodipine and atorvastatin treatment using two pills to an equal dose of single-pill therapy is useful in Japanese outpatients. We retrospectively reviewed data obtained from 94 outpatients for whom treatment with two pills, namely amlodipine and atorvastatin, was switched to an equal dose of single-pill therapy in 11 hospitals. The criterion for enrollment in this study was that patients had switched their medication without changing other anti-hypertensive or anti-cholesterol drugs. Neither systolic nor diastolic blood pressure changed significantly after switching to an equal dose of single-pill therapy, whereas low-density lipoprotein (LDL) cholesterol levels significantly decreased after the medication was switched from 94±24 mg/dl to 89±17 mg/dl (p=0.015). A switch from medication with two separate pills of amlodipine and atorvastatin to an equal dose of single-pill therapy resulted in an overall decrease in LDL cholesterol. The results indicated that the switch to single-pill therapy might be a useful treatment.

Preoperative Clinical Features and High Pulmonary Wedge Pressure with a Discordant Pattern as Prognostic Factor in Hemodialysis Patients with Severe Aortic Valve Stenosis.

Hemodialysis (HD) is one of the important risks for the development of cardiovascular disease, including aortic valve stenosis (AS). Although aortic valve replacement (AVR) is a beneficial treatment for AS, HD patients are known to show a high rate of mortality after AVR than non-HD patients.We retrospectively studied 109 patients who underwent AVR for severe AS, 18 of which were HD patients. Survival rate after AVR, preoperative clinical data, and surgical procedure were investigated.In preoperative clinical features, left ventricular end-diastolic diameter was larger, intraventricular septum thickness (IVST) was thicker, left ventricular mass index (LVMI) was higher, left ventricular ejection fraction was lower, E/e' was higher, and pulmonary arterial wedge pressure (PAWP) was higher in the HD group than in the non-HD group. During a follow-up period of 3.2 ± 2.3 years after AVR, patients receiving HD had a worse prognosis than those without HD treatment: the 3-year survival rate after surgery in the HD group was 36.2% and that in the non-HD group was 84.9%. With regard to prognostic factors in the whole cohort, significant differences were found in IVST, LVMI, E/e', PAWP, and HD. In patients receiving HD, abnormally high PAWP for their right atrial pressure (RAP) was observed, suggesting that PAWP and RAP were discordant, and univariate analysis revealed that high PAWP was the only predictor of mortality in HD patients after surgery.Preoperative PAWP with a discordant pattern in HD patients might be an important prognostic predictor after AVR.

International External Validation Study of the 2014 European Society of Cardiology Guidelines on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (EVIDENCE-HCM).

BACKGROUND: Identification of people with hypertrophic cardiomyopathy (HCM) who are at risk of sudden cardiac death (SCD) and require a prophylactic implantable cardioverter defibrillator is challenging. In 2014, the European Society of Cardiology proposed a new risk stratification method based on a risk prediction model (HCM Risk-SCD) that estimates the 5-year risk of SCD. The aim was to externally validate the 2014 European Society of Cardiology recommendations in a geographically diverse cohort of patients recruited from the United States, Europe, the Middle East, and Asia. METHODS: This was an observational, retrospective, longitudinal cohort study. RESULTS: The cohort consisted of 3703 patients. Seventy three (2%) patients reached the SCD end point within 5 years of follow-up (5-year incidence, 2.4% [95% confidence interval {CI}, 1.9-3.0]). The validation study revealed a calibration slope of 1.02 (95% CI, 0.93-1.12), C-index of 0.70 (95% CI, 0.68-0.72), and D-statistic of 1.17 (95% CI, 1.05-1.29). In a complete case analysis (n= 2147; 44 SCD end points at 5 years), patients with a predicted 5-year risk of <4% (n=1524; 71%) had an observed 5-year SCD incidence of 1.4% (95% CI, 0.8-2.2); patients with a predicted risk of ≥6% (n=297; 14%) had an observed SCD incidence of 8.9% (95% CI, 5.96-13.1) at 5 years. For every 13 (297/23) implantable cardioverter defibrillator implantations in patients with an estimated 5-year SCD risk ≥6%, 1 patient can potentially be saved from SCD. CONCLUSIONS: This study confirms that the HCM Risk-SCD model provides accurate prognostic information that can be used to target implantable cardioverter defibrillator therapy in patients at the highest risk of SCD.

Clinical Profile of Thromboembolic Events in Patients With Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　- Results From Kochi RYOMA Study.

BACKGROUND: There is limited information about the clinical profiles of patients with hypertrophic cardiomyopathy (HCM) and thromboembolic events in a community-based Japanese patient cohort.Methods and Results:In 2004, we established a cardiomyopathy registration network in Kochi Prefecture that comprised 9 hospitals, and finally 293 patients with HCM were followed. The mean age at registration was 63±14 years, and 197 patients (67%) were men. At registration, 86 patients (29%) had documented atrial fibrillation (AF). During a mean follow-up period of 6.1±3.2 years, thromboembolic events, including 3 embolic stroke deaths, occurred in 23 patients. The 5-year embolic event rate was 5.5%. During the follow-up period, an additional 31 patients (11%) had documentation of AF and finally a total of 117 patients (40%) developed AF. The 5-year embolic event rate in those 117 patients with AF was 12.3%. Of the 23 patients with embolic events, 12 had AF prior to the embolic complications and another 6 had documented AF after thromboembolism. AF was not detected in the remaining 5 patients. The CHADS2score did not correlate with the embolic outcome in HCM patients. CONCLUSIONS: In this community-based registry, thromboembolic events were not rare in patients with HCM. All patients with HCM in whom AF develops should be given anticoagulation therapy regardless of their CHADS2score.