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.

Clinical significance of new-onset atrial fibrillation in patients with hypertrophic cardiomyopathy.

AIMS: There is limited information about the clinical significance of atrial fibrillation (AF), particularly new-onset AF, in patients with hypertrophic cardiomyopathy (HCM) in a community-based patient cohort. This study was carried out to clarify the prevalence and prognostic impact of AF in Japanese HCM patients. METHODS AND RESULTS: In 2004, we established a cardiomyopathy registration network in Kochi Prefecture as a prospective study, and finally, 293 patients with HCM were followed. In the patients' cohort, we recently reported the clinical outcomes including mortality and HCM-related morbid events. HCM-related adverse cardiovascular events were defined in the following: (i) sudden cardiac death (SCD)-relevant events including SCD, spontaneous sustained ventricular tachycardia, and appropriate implantable cardioverter defibrillator discharge; (ii) heart failure (HF) events with the composite of HF death and hospitalization for HF; and (iii) embolic events included embolic stroke-related death and admission for embolic events. In the present study, we focused on AF and conducted a detailed investigation. At registration, the mean age of the patients was 63 ± 14 years, and 86 patients (29%) had documented AF including paroxysmal AF. Patients with AF at registration were characterized by worse clinical profiles including more advanced age, more symptomatic, more advanced left ventricular, and left atrial remodelling at registration. During a mean follow-up period of 6.1 ± 3.2 years, a total of 77 HCM-related adverse events occurred, and the presence of AF at registration was associated with an increased risk of HCM-related adverse events, particularly heart failure events. During the follow-up period, an additional 31 patients (11%) had documentation of AF for the first time, defined as new-onset AF, with an annual incidence of approximately 1.8%, and finally, a total of 117 patients (40%) showed AF. The presence of palpitation and enlarged left atrial diameter, particularly left atrial diameter ≥50 mm, at registration were significant predictors of new-onset AF. Importantly, the incidence of overall HCM-related adverse events was further higher in patients with new-onset AF observed from AF onset than in patients with AF at registration. CONCLUSIONS: In our HCM registry in an aged Japanese community, a significant proportion developed AF. The presence of AF, particularly new-onset AF, was associated with increased incidence of HCM-related events. AF may not be just a marker of disease stage but an important trigger of adverse events.

Questionnaire in patients with aborted sudden cardiac death due to coronary spasm in Japan.

OBJECTIVES: We investigated the medical or mechanical therapy, and the present knowledge of Japanese cardiologists about aborted sudden cardiac death (ASCD) due to coronary spasm. METHODS: A questionnaire was developed regarding the number of cases of ASCD, implantable cardioverter-defibrillator (ICD), and medical therapy in ASCD patients due to coronary spasm. The questionnaire was sent to the Japanese general institutions at random in 204 cardiology hospitals. RESULTS: The completed surveys were returned from 34 hospitals, giving a response rate of 16.7%. All SCD during the 5 years was observed in 5726 patients. SCD possibly due to coronary spasm was found in 808 patients (14.0%) and ASCD due to coronary spasm was observed in 169 patients (20.9%). In 169 patients with ASCD due to coronary spasm, one or two coronary vasodilators was administered in two-thirds of patients [113 patients (66.9%)], while more than 3 coronary vasodilators were found in 56 patients (33.1%). ICD was implanted in 117 patients with ASCD due to coronary spasm among these periods including 35 cases with subcutaneous ICD. Majority of cause of ASCD was ventricular fibrillation, whereas pulseless electrical activity was observed in 18 patients and complete atrioventricular block was recognized in 7 patients. Mean coronary vasodilator number in ASCD patients with ICD was significantly lower than that in those without ICD (2.1 ± 0.9 vs. 2.6 ± 1.0, p < 0.001). Although 16 institutions thought that the spasm provocation tests under the medications had some clinical usefulness of suppressing the next fatal arrhythmias, spasm provocation tests under the medication were performed in just 4 institutions. CONCLUSIONS: In the real world, there was no fundamental strategy for patients with ASCD due to coronary spasm. Each institution has each strategy for these patients. Cardiologists should have the same strategy and the same knowledge about ASCD patients due to coronary spasm in the future.

Remote Management of Pacemaker Patients With Biennial In-Clinic Evaluation: Continuous Home Monitoring in the Japanese At-Home Study: A Randomized Clinical Trial.

BACKGROUND: Current expert consensus recommends remote monitoring for cardiac implantable electronic devices, with at least annual in-office follow-up. We studied safety and resource consumption of exclusive remote follow-up (RFU) in pacemaker patients for 2 years. METHODS: In Japan, consecutive pacemaker patients committed to remote monitoring were randomized to either RFU or conventional in-office follow-up (conventional follow-up) at twice yearly intervals. RFU patients were only seen if indicated by remote monitoring. All returned to hospital after 2 years. The primary end point was a composite of death, stroke, or cardiovascular events requiring surgery, and the primary hypothesis was noninferiority with 5% margin. RESULTS: Of 1274 randomized patients (50.4% female, age 77±10 years), 558 (RFU) and 550 (Conventional follow-up) patients reached either the primary end point or 24 months follow-up. The primary end point occurred in 10.9% and 11.8%, respectively (P=0.0012 for noninferiority). The median (interquartile range) number of in-office follow-ups was 0.50 (0.50-0.63) in RFU and 2.01 (1.93-2.05) in conventional follow-up per patient-year (P<0.001). Insurance claims for follow-ups and directly related diagnostic procedures were 18 800 Yen (16 500-20 700 Yen) in RFU and 21 400 Yen (16 700-25 900 Yen) in conventional follow-up (P<0.001). Only 1.4% of remote follow-ups triggered an unscheduled in-office follow-up, and only 1.5% of scheduled in-office follow-ups were considered actionable. CONCLUSIONS: Replacing periodic in-office follow-ups with remote follow-ups for 2 years in pacemaker patients committed to remote monitoring does not increase the occurrence of major cardiovascular events and reduces resource consumption. Registration: URL: https://clinicaltrials.gov; Unique identifier: NCT01523704.

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

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.

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.

Patients' Characteristics and Clinical Course of Hypertrophic Cardiomyopathy in a Regional Japanese Cohort　- Results From Kochi RYOMA Study.

BACKGROUND: There have been few studies on the clinical course of hypertrophic cardiomyopathy (HCM) in a community-based patient cohort in Japan.Methods and Results:In 2004, we established a cardiomyopathy registration network in Kochi Prefecture (the Kochi RYOMA study) that consisted of 9 hospitals, and finally, 293 patients with HCM were followed. The ages at registration and at diagnosis were 63±14 and 56±16 years, respectively, and 197 patients (67%) were male. HCM-related deaths occurred in 23 patients during a mean follow-up period of 6.1±3.2 years. The HCM-related 5-year survival rate was 94%. In addition, a total of 77 cardiovascular events that were clinically severe occurred in 70 patients, and the HCM-related 5-year event-free rate was 80%. Multivariate Cox proportional hazards model analysis showed that the presence of NYHA class III at registration was a significant predictor of HCM-related deaths and that the presence of atrial fibrillation, lower fractional shortening and presence of left ventricular outflow tract obstruction in addition to NYHA class III were significant predictors of cardiovascular events. CONCLUSIONS: In our unselected registry in an aged Japanese community, HCM mortality was favorable, but one-fifth of the patients commonly suffered from HCM-related adverse cardiovascular events during the 5-year follow-up period. Careful management of HCM patients is needed, particularly for those with the above-mentioned clinical determinants.

Mechanisms of changes in functional mitral regurgitation by preload alterations.

BACKGROUND: This study aimed to investigate the mechanisms of acute changes in functional mitral regurgitation (FMR) by preload alterations. METHODS: Twenty-two consecutive patients with left ventricular ejection fraction <40% and at least mild FMR underwent transthoracic echocardiography. Passive leg lifting and sublingual administration of nitroglycerin were performed to alter preload. Mitral regurgitant volume (MRV) was assessed using the Doppler method. RESULTS: MRV changed in parallel with preload alterations. MRV correlated better with tenting height (TH) than with mitral annular area (MAA) at baseline, whereas the difference in the correlate coefficients was not statistically significant (R=0.69 and R=0.40, respectively; p=0.19). On the other hand, changes in MRV between each sequential stage correlated better with those in MAA than with those in TH (R=0.68 and R=0.44, respectively; p=0.043). Multiple regression analysis revealed that baseline TH was the independent determinant of baseline MRV (R=0.69, p=0.0004), whereas changes in MAA with preload alteration were the independent determinant of the changes in MRV (R=0.68, p<0.0001). Changes in left atrial (LA) volume were the independent determinant of the changes in MAA (R=0.30, p=0.0063). CONCLUSIONS: Acute changes in FMR with preload alterations resulted from the transverse changes in MAA rather than the longitudinal changes in tethering-tenting of mitral geometry, and mitral annular deformation was determined by changes in LA volume. Preload reduction might help heart failure treatment through the reduction in FMR resulting from the decrease in LA and mitral annular size.

Efficacy and safety of rivaroxaban in extreme elderly patients with atrial fibrillation: Analysis of the Shikoku Rivaroxaban Registry Trial (SRRT).

BACKGROUND: The Shikoku Rivaroxaban Registry Trial (SRRT) is a retrospective survey of the use of rivaroxaban for stroke prevention in elderly patients in Shikoku, Japan. METHODS: The SRRT enrolled 1339 patients from 8 hospitals. Patients were divided into two groups according to their age, the extreme elderly group (453 patients aged ≧80 years) and the control group (886 patients aged <80 years). RESULTS: In the extreme elderly group, 41.5% of the patients had low body weight (<50kg) and 65.1% had abnormal renal function (creatinine clearance <50ml/min). The mean CHADS2, CHA2DS2-VASc, and HAS BLED scores were 2.7, 4.4, and 2.3, respectively. There were 333 (73.5%) patients who met the dosing criteria, and of these patients, 81.2% received rivaroxaban 10mg daily. Thromboembolic events occurred in 4 patients (0.94%/person year) and intracranial hemorrhage occurred in 4 patients (0.89%/person year). The incidence of these events was not significantly different from the control group. In addition, all patients with cerebral infarction had been treated with a smaller dose of rivaroxaban than recommended by the dosing criteria, suggesting that dosing criteria should be adhered to. CONCLUSION: These results suggest that rivaroxaban is effective and safe in extreme elderly patients with atrial fibrillation.

The impairment of endocardial radial strain is related to aortic stenosis severity in patients with aortic stenosis and preserved LV ejection fraction using two-dimensional speckle tracking echocardiography.

BACKGROUND: Myocardial function is heterogeneous in different myocardial layers. Recently, two-dimensional speckle tracking echocardiography has been used to define myocardial deformation parameters of the left ventricular (LV) segment. This study aimed to investigate strain in subendocardial and subepicardial layers in patients with aortic stenosis (AS) and preserved LV ejection fraction (LVEF) using speckle tracking echocardiography. METHODS: Parasternal short-axis and apical long-axis views of the left ventricle were acquired at the mid-papillary level in 35 control subjects and 32 patients with AS and preserved LVEF. Radial, circumferential, and longitudinal strain in subendocardial and subepicardial layers at the posterior and anteroseptal segments were calculated. RESULTS: There was no significant difference in circumferential strain in subendocardial and subepicardial layers between the control subjects and the patients with AS. Similarly, there was no significant difference in epicardial radial strain at the posterior and anteroseptal segments between the control subjects and the patients with AS. Longitudinal strain at both the posterior and anteroseptal segments was significantly decreased in the AS group compared with that in the control group. AS patients had significantly decreased values of endocardial radial strain compared with those in controls (anteroseptal: 18.2 ± 11.2 vs. 34.5 ± 14.8, P < 0.005; posterior: 25.2 ± 14.8 vs. 32.6 ± 12.6, P < 0.05). In the AS group, endocardial radial strain in the posterior and anteroseptal segments was significantly correlated with the aortic valve area (posterior: r = 0.41, P < 0.05; anteroseptal: r = 0.33, P < 0.05). CONCLUSION: Patients with AS and preserved LVEF have impaired longitudinal strain and endocardial radial strain, although circumferential strain and epicardial radial strain are preserved. Despite preserved LVEF, endocardial radial strain was associated with AS severity.

Successful treatment in a case of acute myocardial infarction due to spontaneous coronary artery dissection.

Spontaneous coronary dissection is a rare cause of myocardial ischemia, myocardial infarction, and sudden death. In the present case, we performed stenting only of the severe stenotic lesion of the dissection with a short bare metal stent, and left the remaining long length of dissection vessel untreated. Six months after coronary stenting, follow-up angiography revealed complete healing of the coronary dissection and no significant in-stent restenosis. This case clearly demonstrates the usefulness of intravascular ultrasound (IVUS) imaging in the diagnosis of coronary dissection and IVUS imaging guides to the choice of the best stenting.

Cor triatriatum sinister disclosed after diagnosis of acute myocardial infarction.

We present a case of cor triatriatum sinister diagnosed occasionally after acute anterior myocardial infarction. For management of the acute myocardial infarction (AMI), urgent reperfusion therapy was successfully performed through the left anterior descending coronary artery. Thereafter, no complication associated with AMI occurred. Cor triatriatum sinister was diagnosed and assessed later by means of several modalities. Finally, medical observation was indicated for this patient. This case illustrates the importance of awareness of this congenital disease in an adult when echocardiography shows an abnormal linear echo in the mid portion of the left atrium.

Complete detachment of cardiac myxoma causing aortic saddle embolization and cerebral infarction.

A 30-year-old previously healthy male was brought to our emergency room with loss of consciousness and convulsion. Physical examination showed that the femoral arterial pulses were absent. Two-dimensional transthoracic and transesophageal echocardiography showed no evidence of intracardiac thrombus or tumor or of aortic dissection. Brain magnetic resonance imaging study showed cerebral infarction involving anterior and bilateral hemispheres. He died of multiple organ failure after admission. Autopsy showed aortic saddle embolization caused by a myxomatous-like material. Histological examination of the embolic material confirmed the diagnosis of myxoma. There was a stalk on the left atrial septum which might possibly represent the site of attachment of the myxoma. This case suggested that a cardiac myxoma completely detached from the left atrial septum and caused aortic saddle embolization.