Concentric hypertrophy geometry is a significant determinant of impaired global longitudinal strain in patients with normal cardiac structure and function.

Background: In recent years, the assessment of global longitudinal strain (GLS) derived by speckle-tracking analysis has become a clinically feasible alternative to left ventricular (LV) ejection fraction (LVEF) for the assessment of myocardial function. However, the determinant factors of impaired GLS in structurally and functionally normal patients are unclarified. The objective of this study was to elucidate the determinant factors of impaired GLS in structurally and functionally normal patients. Methods: We evaluated structurally and functionally normal patients scheduled to undergo noncardiac surgery. The evaluated patient characteristics were age, sex, presence of hypertension, presence of diabetes mellitus, presence of hyperlipidemia, systolic blood pressure, and body mass index. The concentrations of B-type natriuretic peptide and high-sensitivity troponin I were measured. Echocardiography was performed to determine the LVEF, GLS, transmitral early diastolic velocity/transmitral atrial velocity ratio, LV mass index (LVMI), and relative wall thickness (RWT). Patients with preserved LVEF (≥50%) were divided into the normal GLS group (GLS ≤ -20%) and the impaired GLS group (GLS > -20%). On the basis of the RWT and LVMI values, the patients were categorized as having four types of LV geometry. Logistic regression analysis was performed to ascertain the determinant factors of impaired GLS. Results: The study cohort comprised 75 structurally and functionally normal patients (age 67.7 ± 12.6 years, 45 men). The GLS was normal in 43 patients and impaired in 32 patients. There was a significant difference in RWT between the impaired and normal GLS groups. The evaluation based on the LV geometry showed that six of seven patients with concentric hypertrophy geometry had impaired GLS, and the GLS was significantly more impaired in patients with concentric hypertrophy geometry than in patients with normal geometry or eccentric hypertrophy geometry. Logistic regression analysis revealed that LV concentric hypertrophy geometry was a significant determinant factor of impaired GLS (odds ratio 22.4, P = 0.042). Conclusions: Global longitudinal strain is more impaired in structurally and functionally normal patients with concentric hypertrophy geometry compared with those with eccentric hypertrophy geometry or normal geometry. In addition, concentric hypertrophy geometry is a significant determinant for impaired GLS in patients with normal cardiac structure and function.

Ventricular tachycardia in patients undergoing 24-h Holter monitoring as preoperative evaluation for noncardiac surgery.

The incidence of ventricular tachycardia (VT) in preoperative evaluation for noncardiac surgery in general hospitals has not been established. The aim of this study was to determine the incidence of VT, characteristics of patients with VT, characteristics of VT, and significance of VT in patients undergoing 24-h Holter monitoring as preoperative evaluation for noncardiac surgery. In 601 patients, VT was detected in 46 patients (7.7%). In patients with VT, left ventricular ejection fraction (LVEF) was lower (62.6 ± 9.3% vs. 66.6 ± 8.9%, p = 0.003), and B-type natriuretic peptide (BNP) was higher compared with patients without VT (median, 52.5 pg/mL vs. 32.8 pg/mL, p = 0.02). The maximum number of consecutive beats of VT was more frequent in the patients with LVEF < 50% than in the patients with LVEF ≥ 50% (median, 11.5 beats vs. 3.0 beats, p = 0.01). Forty patients (87%) underwent scheduled surgery without major complications.

Sarcomere gene mutations are associated with increased cardiovascular events in left ventricular hypertrophy: results from multicenter registration in Japan.

OBJECTIVES: This study investigated the occurrence of cardiovascular events in patients with hypertensive heart disease (HHD) or hypertrophic cardiomyopathy (HCM) with or without sarcomere gene mutations. BACKGROUND: Although HHD and HCM are associated with left ventricular hypertrophy (LVH), few data exist regarding the difference in prognosis between them. METHODS: We enrolled 256 patients with LVH (>13 mm) screened for sarcomere gene mutations. We divided them into 3 groups: the first had HHD without sarcomere gene mutations (group H), the second had sarcomere gene mutations (group G), and the third had neither sarcomere gene mutations nor HHD (group NG). We compared the occurrence of sudden cardiac death, ventricular tachycardia/fibrillation, admission for heart failure, and atrial fibrillation for 1 year. RESULTS: Group G (n = 78, 36 men; mean age, 53.4 years) experienced more total cardiovascular events than group H (n = 45, 32 men; mean age, 67.4 years) (p = 0.042) after adjustments for age and sex, although there was no significant difference in total cardiovascular events between groups H and NG (n = 98, 66 men; mean age, 62.0 years). With Kaplan-Meier analysis, group G exhibited a significantly higher incidence of admission for heart failure (p = 0.017) and atrial fibrillation (p = 0.045) than group H in those 50 years of age and older. Additionally, there was a significant difference in total cardiovascular events between groups G and NG (p = 0.021). CONCLUSIONS: These results demonstrate that HCM with sarcomere gene mutations can be associated with increased cardiovascular events compared with HHD or HCM without sarcomere gene mutations.

Terminal stage cardiac findings in patients with cardiac Fabry disease: an electrocardiographic, echocardiographic, and autopsy study.

OBJECTIVES: Fabry disease is caused by deficiency of alpha-galactosidase A, and typically causes multi-organ dysfunction. Patients with manifestations limited to the heart, mainly left ventricular hypertrophy (LVH), have been reported as a disease variation. We have reported a 3% prevalence of this cardiac variant in men with LVH, which we designated 'cardiac Fabry disease'. The purposes of this study were to evaluate the terminal stage cardiac manifestations and autopsy findings in patients with cardiac Fabry disease. METHODS: We examined seven terminal stage patients with cardiac Fabry disease. During hospitalization, standard 12-lead electrocardiograms, Holter electrocardiograms, and echocardiograms were obtained. Autopsies were performed and macroscopic along with microscopic findings were evaluated. RESULTS: Six patients died of heart failure and one of ventricular fibrillation. Electrocardiograms revealed the presence of conduction abnormalities and nonsustained ventricular tachycardia. Echocardiograms and autopsy findings revealed LVH in all patients. Localized basal posterior wall thinning of the left ventricle was detected in the six patients who died of heart failure. All patients had severe left ventricular dysfunction. Histologically, myocardial cells, but not cardiac vascular endothelial cells, showed glycosphingolipid accumulation. No accumulation was observed in other organs or in systemic vascular endothelial cells. CONCLUSIONS: Severe left ventricular dysfunction with associated conduction disturbances and ventricular arrhythmias occur in patients with terminal stage cardiac Fabry disease. Furthermore, LVH is present and associated with thinning of the base of the left ventricular posterior wall. In contrast to typical Fabry disease, accumulation of glycosphingolipids was observed in myocardial cells but not in other organs.

Limitation of angiography to identify the culprit plaque in acute myocardial infarction with coronary total occlusion utility of coronary plaque temperature measurement to identify the culprit plaque.

OBJECTIVES: The purpose of this study was to test the hypothesis that the maximal temperature (Tmax) site, as measured by thermal wire, coincides with the culprit plaque by intravascular ultrasound (IVUS) in patients with acute myocardial infarction (AMI). BACKGROUND: Subsequent thrombosis developing to the proximal region from the site of plaque rupture or erosion can potentially complicate the ability of coronary angiography to identify the accurate culprit plaque in patients with coronary total occlusion. METHODS: In 45 consecutive patients with a first anterior AMI, the Tmax site by thermal wire and the culprit plaque by IVUS were evaluated in the left anterior descending coronary artery (LAD). RESULTS: Twenty-five patients had LAD total occlusion, and the remaining 20 had LAD reperfusion. In both groups of patients, the Tmax site was significantly more distal to the angiographically most stenotic site or occlusive site (reperfusion: mean distance [MD] = 1.1 mm distal, 95% confidence interval [CI] 0.3 to 1.9 mm, p = 0.01; total occlusion: MD = 8.8 mm distal, 95% CI 8.0 to 9.6 mm, p < 0.0001). The culprit plaques by IVUS approximately coincided with those by angiography or thermal wire in patients with reperfusion. However, the angiographic occlusive site was significantly more proximal to the culprit plaque by IVUS (MD = 9.2 mm, 95% CI 7.9 to 10.6 mm, p < 0.0001), but the Tmax site coincided with the culprit plaque by IVUS (MD = 0.3 mm distal, 95% CI 0.3 mm proximal to 1.0 mm distal, p = 0.293) in patients with total occlusion. CONCLUSIONS: Temperature measurement of coronary plaque enables accurate localization of the culprit plaque in AMI with coronary total occlusion.

Intracardiac thrombus, superior vena cava syndrome, and pulmonary embolism in a patient with Behçet's disease: a case report and literature review.

A 26-year-old woman with intermittent fever was admitted to our hospital, and gradually developed facial edema. Examinations including computed tomography, transesophageal echocardiography, digital subtraction angiography, and pulmonary perfusion scintigraphy revealed intracardiac thrombus, superior vena cava syndrome, and pulmonary embolism. Clinical findings and laboratory data led us to make a diagnosis of Behçet's disease. Combination of intracardiac thrombus, superior vena cava syndrome, and pulmonary embolism are rare complications in Behçet's disease. Behçet's disease should be considered in the differential diagnosis of intracardiac mass of the right heart, and early diagnosis and treatment are essential for the management of Behçet's disease especially with large-vessel manifestations. In addition to a case report, we review the literature and report the characteristics of intracardiac thrombus in Behçet's disease.

Mutations in the genes for sarcomeric proteins in Japanese patients with onset sporadic hypertrophic cardiomyopathy after age 40 years.

This study was conducted to assess the hypothesis that mutations in the genes for sarcomeric proteins are the molecular cause for older-onset sporadic hypertrophic cardiomyopathy (HC) in Japanese patients. Molecular genetic approaches have demonstrated that familial HC is caused by mutations in genes encoding sarcomeric proteins. Recent studies have shown that sarcomeric gene mutations can also be a molecular cause of older-onset and/or sporadic HC. However, genetic studies to date have examined only a limited number of older Caucasian patients with HC. Clinical evaluations were performed in patients with HC onset after 40 years of age, and the sequence encoding the beta-cardiac myosin heavy chain, cardiac troponin T, cardiac troponin I, cardiac myosin binding protein-C, myosin ventricular regulatory light chain, and myosin ventricular essential light chain genes was analyzed. When a putative mutation was identified, clinical evaluations and genetic studies were subsequently performed on all first-degree relatives. Forty-one patients with sporadic HC onset after 40 years of age (31 men, 10 women; mean age 63+/-10 years at the time of study) were studied. Four novel missense mutations in the cardiac myosin binding protein-C gene (arginine to tryptophan at codon 160, glutamic acid to lysine at codon 334, glycine to arginine at codon 507, and threonine to methionine at codon 1,046) and a previously reported missense mutation in the beta-cardiac myosin heavy chain gene (arginine to histidine at codon 663) were identified in 5 of the 41 patients. No family members carried these mutations or had clinical evidence of HC. In conclusion, mutations in the cardiac myosin binding protein-C are the most common cause of older-onset sporadic HC in Japan.

Usefulness and cost effectiveness of cardiovascular screening of young adolescents.

OBJECTIVES: This study was conducted to assess the usefulness of a screening system for cardiovascular disease in Kagoshima, Japan, and to compare its cost-effectiveness with that of a similar system reported in the United States. BACKGROUND: Preparticipation screening of young athletes has been implemented in many countries to prevent sudden death, but sudden death in young nonathletes remains a problem. In Japan, both athletes and nonathletes have been screened for the presence or absence of cardiovascular diseases for more than 20 yr. METHODS: From 1989 to 1997, all seventh graders in schools in Kagoshima, Japan, were screened for cardiovascular disease using a questionnaire and electrocardiogram before physical examination. They were screened again in the same way 3 yr later. One subject newly diagnosed with cardiovascular disease and recommended to limit athletic participation was defined as "high-risk." Situations leading to cases of sudden death were verified with a report from the school in question. RESULTS: Of the initial study population, 99% participated in the program every year. A total of 37,807 subjects, including nine high-risk subjects, were evaluated consecutively for 6 yr. Of these nine subjects, six, including three patients with hypertrophic cardiomyopathy, were nonathletes. Three sudden deaths occurred during the study period; one student was from the high-risk group. The cost of this screening system was lower than that reported in the United States. CONCLUSIONS: Population-based screening for heart disease in this age range is limited by various factors. To analyze the mechanisms of sudden death in adolescents, we, therefore, are in need of a nationwide registry that includes autopsies for all deadly events.

Prevalence and time of appearance of Brugada electrocardiographic pattern in young male adolescents from a three-year follow-up study.

The prevalence of Brugada's electrocardiographic (ECG) pattern in 7,022 male adolescents in the seventh grade was determined, and the same subjects were reexamined 3 years later, while in tenth grade. Two subjects (0.03%) and 7 subjects (0.10%) showed Brugada's ECG pattern by the conventional criterion (J point or ST-segment >/=0.1 mV in leads V(1) to V(3)), and no subjects (0%) and 2 subjects (0.03%) fulfilled the recent criterion (J point or ST-segment >/=0.2 mV) in the seventh and tenth grades, respectively, indicating that Brugada's ECG pattern begins to appear during junior high school and increases until late adulthood.