Carnitine Administration and I-BMIPP Washout Rate in Hemodialysis Patients with Triglyceride Deposit Cardiomyovasculopathy.

Triglyceride deposit cardiomyovasculopathy (TGCV) is an emerging rare heart disease with high mortality, characterized by defective intracellular lipolysis of triglycerides (TG). We developed diagnostic criteria for TGCV, in which low washout rate of BMIPP (BMIPP-WR) is a key factor. The working group of the Japan Society of Nuclear Cardiology recently published practice recommendations for measuring BMIPP-WR.

Machine learning-based detection of sleep-disordered breathing in hypertrophic cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is often concomitant with sleep-disordered breathing (SDB), which can cause adverse cardiovascular events. Although an appropriate approach to SDB prevents cardiac remodelling, detection of concomitant SDB in patients with HCM remains suboptimal. Thus, we aimed to develop a machine learning-based discriminant model for SDB in HCM.

Feasibility of Short-Term Aggressive Lipid-Lowering Therapy with the PCSK9 Antibody in Acute Coronary Syndrome.

Background: The guideline-recommended low-density lipoprotein cholesterol target level of <70 mg/dL may not be achieved with statin administration in some patients with acute coronary syndrome (ACS). Therefore, the proprotein convertase subtilisin-kexin type 9 (PCSK9) antibody can be added to high-risk patients with ACS. Nevertheless, the optimal duration of PCSK9 antibody administration remains unclear.

Rapidly progressive myocardial calcification following sepsis.

Sepsis is a clinical syndrome caused by a dysregulated host response to infection that can lead to multiple organ dysfunction and death. Cardiovascular abnormalities are frequent in sepsis and may result in myocardial injury unrelated to coronary artery disease. Myocardial calcification is a rare complication of sepsis, which shows rapid-onset extensive myocardial calcifications.

Plasma Globotriaosylsphingosine Level as a Primary Screening Target for Fabry Disease in Patients With Left Ventricular Hypertrophy.

Background: Although previous studies have suggested a certain prevalence of Fabry disease (FD) in left ventricular hypertrophy (LVH) patients, the screening of FD is difficult because of its wide-ranging clinical phenotypes. We aimed to clarify the utility of combined measurement of plasma globotriaosylsphingosine (lyso-Gb3) concentration and α-galactosidase A activity (α-GAL) as a primary screening of FD in unexplained LVH patients.

Methods and results: Between 2014 and 2016, both lyso-Gb3 and α-GAL were measured in 277 consecutive patients (male 215, female 62, age 25-79 years) with left ventricular wall thickness >12 mm on echocardiogram: 5 patients (1.

Unusual Serial Electrocardiographic Changes which Progressed to Arrhythmogenic Right Ventricular Cardiomyopathy.

Left ventricular (LV) involvement in the advanced stage of arrhythmogenic right ventricular cardiomyopathy (ARVC) is a well recognized phenomenon. T wave inversion in the lateral leads has been reported to be an electrocardiographic marker of LV involvement. Variants of ARVC that preferentially affect the left ventricle (left-dominant subtype of arrhythmogenic cardiomyopathy) have recently been recognized.

Low WSS and High OSI Measured by 3D Cine PC MRI Reflect High Pulmonary Artery Pressures in Suspected Secondary Pulmonary Arterial Hypertension.

Purpose: Institutional Review Board (IRB)-approved prospective study was conducted to test whether objective and quantitative hemodynamic markers wall shear stress (WSS) and oscillatory shear index (OSI) measured by three-dimensional (3D) cine phase-contrast (PC) can reflect pulmonary arterial hypertension (PAH).

Patients And Methods: Seventeen consecutive patients of suspected secondary PAH were examined for pulmonary artery pressures (PAPs) with right heart catheterization (RHC) and three-dimensional (3D) cine PC MR. Based on the RHC data, patients were subdivided into two groups of 12 non-PAH (median age of 74.