Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort.
View Article and Find Full Text PDFBackground: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease with an estimated prevalence of up to 1:5000 in the general population. Few cases of ARVC during pregnancy are described in literature.
Case Summary: A 32-year-old primigravida was referred to our clinic during the 32nd gestational week.
Importance: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk.
Objective: To develop and validate an SCD risk prediction model that provides individualized risk estimates.
Design, Setting, And Participants: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM.
Background: The Synergy system, a miniature partial circulatory support device, is implanted by an off-pump, minimally invasive surgical approach. The system has been optimized to improve performance in an EU clinical trial for chronic ambulatory heart failure. This therefore offers the possibility of treating elderly chronic heart failure patients who might not usually be considered for long-term circulatory support.
View Article and Find Full Text PDFObjective: The mechanisms by which migraine is linked to ischemic vascular disease remain uncertain and are likely to be complex. The aim of this study was to investigate the correlation between silent myocardial ischemia (SMI) and a history of documented primary headache in a large population of patients with exercise-induced myocardial ischemia.
Methods: The study involved 1,427 consecutive patients (918 symptomatic and 509 asymptomatic patients) with exercise-induced myocardial ischemia and documented coronary artery disease (CAD).
Background: The triggers of ventricular arrhythmias (VAs) leading to sudden cardiac death in hypertrophic cardiomyopathy (HCM) are ill defined. We sought to examine the electrophysiological characteristics of VAs in HCM and study their relation to cardiac phenotype and circadian patterns using stored intracardiac electrocardiograms from implantable cardioverter defibrillators (ICDs).
Methods And Results: A single centre, observational cohort study of 230 consecutively evaluated ICD recipients with HCM [median age 42 years, 97% primary prevention, 51% with anti-tachycardia pacing (ATP)].
Background: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with bradyarrhythmias. We sought to examine the nature of conduction system abnormalities and the indications and determinants of anti-bradycardia pacing in patients with AFD.
Methods And Results: We studied 204 patients with AFD (49% male, mean age 42 years) in an observational, longitudinal, retrospective cohort study.
Objective: Implantable cardioverter defibrillators (ICDs) are routinely used to prevent sudden cardiac death (SCD) in selected hypertrophic cardiomyopathy (HCM) patients, but the determinants of device-related complications, therapies and long-term cardiovascular mortality in ICD recipients are not known.
Design: Retrospective observational cohort study.
Setting: Single-centre tertiary referral cardiomyopathy clinic.
Background: Alterations in coronary vasomotor tone are deemed to play an important role in myocardial infarction (MI), and the ATP-binding cassette transporter C9-ABCC9-may be involved in the regulation of coronary artery vasomotility. We sought to determine whether genetic variations in the coding sequence of ABCC9 gene could be associated with precocious MI (myocardial infarction before the age of 60 years) in humans.
Methods: In this study, we screened using PCR-SSCP analysis the entire coding region of the ABCC9 gene in 45 patients with precocious MI and 45 age- and gender-matched controls.
Aims: Levels of the secreted glycophosphoprotein osteopontin (OPN) have been associated with the presence and extent of coronary artery disease (CAD). The present study assessed the relationship between plasma OPN concentrations and prognosis in patients with chronic stable angina (CSA).
Methods And Results: OPN was measured in baseline plasma samples from 799 patients with stable angina pectoris and angiographically documented CAD.
Objectives: Advanced glycation end-products (AGE) may cause vascular stiffening by forming crosslinks through the collagen molecule or by interaction with their cellular transductional receptor (RAGE). A secreted isoform of RAGE, termed soluble RAGE (sRAGE), may contribute to the removal/detoxification of AGE by acting as a decoy. Here we studied the plasma sRAGE levels in hypertensive and normotensive human subjects.
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