Diet-Induced Obesity in Mice Affects the Maternal Gut Microbiota and Immune Response in Mid-Pregnancy.

Maternal obesity during pregnancy is associated with adverse pregnancy outcomes. This might be due to undesired obesity-induced changes in the maternal gut microbiota and related changes in the maternal immune adaptations during pregnancy. The current study examines how obesity affects gut microbiota and immunity in pregnant obese and lean mice during mid-pregnancy (gestational day 12 (GD12)).

Prognostic significance of left atrial strain in sarcomere gene variant carriers without hypertrophic cardiomyopathy.

Background: Genetic testing of relatives of hypertrophic cardiomyopathy (HCM) patients has led to a large group of genotype-positive, phenotype-negative (G+/Ph-) subjects. Prediction of progression to overt HCM in these subjects is challenging. While left atrial (LA) strain is reduced in HCM patients it is currently unknown whether this parameter can be used to predict HCM phenotype progression.

Contemporary family screening in hypertrophic cardiomyopathy: the role of cardiovascular magnetic resonance.

Aims: Genetic testing in relatives of hypertrophic cardiomyopathy (HCM) patients leads to early identification of pathogenic DNA variant carriers (G+), before the onset of left ventricular hypertrophy. Routine phenotyping consists of electrocardiography (ECG) and transthoracic echocardiography (TTE). Cardiovascular magnetic resonance (CMR) has become valuable in the work-up of HCM.

Relation Between Early Diastolic Mid-Ventricular Flow and Elastic Forces Indicating Aneurysm Formation in Hypertrophic Cardiomyopathy.

Background: The early diastolic paradoxical midventricular flow is suggestive of apical aneurysm (AA) formation in hypertrophic cardiomyopathy (HCM). We aimed to determine whether early diastolic paradoxical midventricular flow may be a useful screening tool in patients, following the time progression of HCM to the aneurysmal stage.

Methods: One hundred twenty-one HCM patients with dominant hypertrophy in the mid and apical segments, based on echocardiography and/or cardiovascular magnetic resonance, were selected from our HCM database, which comprises 1,332 patients.

Novel Morphological Features on CMR for the Prediction of Pathogenic Sarcomere Gene Variants in Subjects Without Hypertrophic Cardiomyopathy.

Carriers of pathogenic DNA variants (G+) causing hypertrophic cardiomyopathy (HCM) can be identified by genetic testing. Several abnormalities have been brought forth as pre-clinical expressions of HCM, some of which can be identified by cardiovascular magnetic resonance (CMR). In this study, we assessed morphological differences between G+/left ventricular hypertrophy-negative (LVH-) subjects and healthy controls and examined whether CMR-derived variables are useful for the prediction of sarcomere gene variants.

BIO FOr CARE: biomarkers of hypertrophic cardiomyopathy development and progression in carriers of Dutch founder truncating MYBPC3 variants-design and status.

Background: Hypertrophic cardiomyopathy (HCM) is the most prevalent monogenic heart disease, commonly caused by truncating variants in the MYBPC3 gene. HCM is an important cause of sudden cardiac death; however, overall prognosis is good and penetrance in genotype-positive individuals is incomplete. The underlying mechanisms are poorly understood and risk stratification remains limited.

Shared genetic pathways contribute to risk of hypertrophic and dilated cardiomyopathies with opposite directions of effect.

The heart muscle diseases hypertrophic (HCM) and dilated (DCM) cardiomyopathies are leading causes of sudden death and heart failure in young, otherwise healthy, individuals. We conducted genome-wide association studies and multi-trait analyses in HCM (1,733 cases), DCM (5,521 cases) and nine left ventricular (LV) traits (19,260 UK Biobank participants with structurally normal hearts). We identified 16 loci associated with HCM, 13 with DCM and 23 with LV traits.

Incremental Value of an Insertable Cardiac Monitor in Patients with Hypertrophic Cardiomyopathy with Low or Intermediate Risk for Sudden Cardiac Death.

Aims: The aim of the present study was to compare the rate of actionable arrhythmic events between patients with hypertrophic cardiomyopathy (HCM) who are monitored with an insertable cardiac monitor (ICM) or Holter monitoring.

Methods: We studied 50 patients (mean age 52 years, 72% men) with HCM at low or intermediate risk for sudden cardiac death (SCD), of whom 25 patients received an ICM between November 2014 and February 2019. We retrospectively identified a control group of 25 patients who were matched on age, sex, and HCM Risk-SCD score category.

Mutation location of HCM-causing troponin T mutations defines the degree of myofilament dysfunction in human cardiomyocytes.

Background: The clinical outcome of hypertrophic cardiomyopathy patients is not only determined by the disease-causing mutation but influenced by a variety of disease modifiers. Here, we defined the role of the mutation location and the mutant protein dose of the troponin T mutations I79N, R94C and R278C.

Methods And Results: We determined myofilament function after troponin exchange in permeabilized single human cardiomyocytes as well as in cardiac patient samples harboring the R278C mutation.

Impact of sex on timing and clinical outcome of septal myectomy for obstructive hypertrophic cardiomyopathy.

Background: Sex disparities are common in hypertrophic cardiomyopathy (HCM). Previous research has shown that at time of myectomy, women are older, have greater impairment of diastolic function and more advanced cardiac remodeling. The clinical impact of these differences is unknown.

Strength of patient cohorts and biobanks for cardiomyopathy research.

In 2011 the Netherlands Heart Foundation allocated funding (CVON, Cardiovasculair Onderzoek Nederland) to stimulate collaboration between clinical and preclinical researchers on specific areas of research. One of those areas involves genetic heart diseases, which are frequently caused by pathogenic variants in genes that encode sarcomere proteins. In 2014, the DOSIS (Determinants of susceptibility in inherited cardiomyopathy: towards novel therapeutic approaches) consortium was initiated, focusing their research on secondary disease hits involved in the onset and progression of cardiomyopathies.

Left-ventricular outflow tract acceleration time is associated with symptoms in patients with obstructive hypertrophic cardiomyopathy.

Aims: Not all obstructive hypertrophic cardiomyopathy (HCM) patients are symptomatic. The relation between obstructive HCM and symptoms is not well understood. The hypothesis of this study is that left-ventricular outflow tract (LVOT) acceleration time (AT) is associated with symptoms.

Frequency and Significance of Coronary Artery Disease and Myocardial Bridging in Patients With Hypertrophic Cardiomyopathy.

The etiology of chest pain in hypertrophic cardiomyopathy (HC) is diverse and includes coronary artery disease (CAD) as well as HC-specific causes. Myocardial bridging (MB) has been associated with HC, chest pain, and accelerated atherosclerosis. We compared HC patients with age-, gender- and CAD pre-test probability-matched outpatients presenting with chest pain to investigate differences in the presence of MB and CAD using coronary computed tomography angiography (CCTA).

Effect of body surface area and gender on wall thickness thresholds in hypertrophic cardiomyopathy.

Background: Family screening for hypertrophic cardiomyopathy (HCM) is based on genetic testing and clinical evaluation (maximal left ventricular wall thickness (MWT) ≥15 mm, or ≥13 mm in first-degree relatives of HCM patients). The aim of this study was to assess the effect of gender and body size on diagnosis of HCM and prediction of clinical outcome.

Methods: This study includes 199 genotype-positive subjects (age 44 ± 15 years, 50% men) referred for cardiac screening.

Correction to: Extra energy for hearts with a genetic defect: ENERGY trial.

Correction to: Neth Heart J 2019 https://doi.org/10.1007/s12471-019-1239-0 In the version of the article originally published online, there was an error in Fig.

Extra energy for hearts with a genetic defect: ENERGY trial.

Aims: Previous studies have shown that hypertrophic cardiomyopathy mutation carriers have a decreased myocardial energy efficiency, which is thought to play a key role in the pathomechanism of hypertrophic cardiomyopathy (HCM). The ENERGY trial aims to determine whether metabolic drugs correct decreased myocardial energy efficiency in HCM mutation carriers at an early disease stage.

Methods: 40 genotype-positive, phenotype-negative MYH7 mutation carriers will be treated for two months with trimetazidine or placebo in a double-blind randomised study design.

Sex differences in hypertrophic cardiomyopathy: new insights.

Purpose Of Review: Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy, diagnosed by left ventricular hypertrophy of at least 15 mm maximal wall thickness (MWT). Recent studies reported a sex difference in clinical presentation, progression and outcome of HCM. This review provides an overview of recent studies into sex differences in HCM.

Effect of Gender and Genetic Mutations on Outcomes in Patients With Hypertrophic Cardiomyopathy.

Gender has been proposed to impact the phenotype and prognosis of hypertrophic cardiomyopathy (HC). Our aims were to study gender differences in the clinical presentation, phenotype, genotype, and outcome of HC. This retrospective single-center cohort study included 1,007 patients with HC (62% male, 80% genotyped) evaluated between 1977 and 2017.

Editor's Choice - Systematic Review and Meta-Analysis of the Outcome of Treatment for Type II Endoleak Following Endovascular Aneurysm Repair.

Objectives: The efficacy and need for secondary interventions for type II endoleaks following endovascular abdominal aortic aneurysm repair (EVAR) remain controversial. This systematic review aimed at investigating the clinical outcomes of different type II endoleak treatments in patients with a persistent type II endoleak after EVAR.

Data Sources: Embase, Medline via Ovid, Web of Science Core Collection, the Cochrane CENTRAL, and Google Scholar.

Eight-Year Prognostic Value of QRS Duration in Patients With Known or Suspected Coronary Artery Disease Referred for Myocardial Perfusion Imaging.

QRS duration is of prognostic relevance in patients with several underlying heart diseases. Short-term data also show the prognostic value of QRS duration in lower risk groups of patients. The aim of this study was to investigate the long-term prognostic value of QRS duration in patients with known or suspected coronary artery disease.