Prevalence of Inherited Cardiac Conditions in Pediatric First-Degree Relatives of Patients with Idiopathic Ventricular Fibrillation.

Idiopathic ventricular fibrillation (IVF) is diagnosed in out-of-hospital VF survivors after comprehensive investigations have excluded structural heart disease or inherited channelopathies. Current guidelines recommend clinical screening of first-degree relatives of IVF survivors, but this approach has not been validated in children. This study aimed to assess the yield of clinical cardiac screening in child first-degree relatives of IVF victims.

Clinical Features and Natural History of PRKAG2 Variant Cardiac Glycogenosis.

Background: PRKAG2 gene variants cause a syndrome characterized by cardiomyopathy, conduction disease, and ventricular pre-excitation. Only a small number of cases have been reported to date, and the natural history of the disease is poorly understood.

Objectives: The aim of this study was to describe phenotype and natural history of PRKAG2 variants in a large multicenter European cohort.

An International Multicenter Evaluation of Type 5 Long QT Syndrome: A Low Penetrant Primary Arrhythmic Condition.

Background: Insight into type 5 long QT syndrome (LQT5) has been limited to case reports and small family series. Improved understanding of the clinical phenotype and genetic features associated with rare variants implicated in LQT5 was sought through an international multicenter collaboration.

Methods: Patients with either presumed autosomal dominant LQT5 (N = 229) or the recessive Type 2 Jervell and Lange-Nielsen syndrome (N = 19) were enrolled from 22 genetic arrhythmia clinics and 4 registries from 9 countries.

Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology.

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies.

Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors: An Expert Statement.

Carcinoid heart disease is a frequent occurrence in patients with carcinoid syndrome and is responsible for substantial morbidity and mortality. The pathophysiology of carcinoid heart disease is poorly understood; however, chronic exposure to excessive circulating serotonin is considered one of the most important contributing factors. Despite recognition, international consensus guidelines specifically addressing the diagnosis and management of carcinoid heart disease are lacking.

Acute coronary syndromes.

In the UK, there are over 80,000 admissions annually with acute coronary syndromes (ACS). Management of ST-elevation myocardial infarction (STEMI) involves primary percutaneous coronary intervention (PCI), which is delivered via dedicated heart attack centres. Non-ST elevation-ACS (NSTE-ACS) accounts for two-thirds of ACS presentations, affecting an older cohort of patients - often with more complex comorbidities.

Patient-specific parameter estimation in single-ventricle lumped circulation models under uncertainty.

Computational models of cardiovascular physiology can inform clinical decision-making, providing a physically consistent framework to assess vascular pressures and flow distributions, and aiding in treatment planning. In particular, lumped parameter network (LPN) models that make an analogy to electrical circuits offer a fast and surprisingly realistic method to reproduce the circulatory physiology. The complexity of LPN models can vary significantly to account, for example, for cardiac and valve function, respiration, autoregulation, and time-dependent hemodynamics.

On a sparse pressure-flow rate condensation of rigid circulation models.

Cardiovascular simulation has shown potential value in clinical decision-making, providing a framework to assess changes in hemodynamics produced by physiological and surgical alterations. State-of-the-art predictions are provided by deterministic multiscale numerical approaches coupling 3D finite element Navier Stokes simulations to lumped parameter circulation models governed by ODEs. Development of next-generation stochastic multiscale models whose parameters can be learned from available clinical data under uncertainty constitutes a research challenge made more difficult by the high computational cost typically associated with the solution of these models.

Management of high-risk non-ST elevation myocardial infarction in the UK: need for alternative models of care to reduce length of stay and admission to angiography times.

The roll out of the primary percutaneous coronary intervention pathway as the default treatment for patients with ST elevation myocardial infarction (STEMI) across the NHS has led to a paradigm shift in the model of care resulting in a significant improvement in mortality. In comparison, a similar care plan does not exist for non-ST elevation acute coronary syndrome (NSTE-ACS) despite the fact that patients presenting with high-risk non-STEMI carry a similar if not higher mortality at six months in comparison to STEMI. In this article we focus on the contemporary management of NSTE-ACS in the NHS and also look at some of the dedicated pathways already developed and implemented successfully in expediting treatment and decreasing hospital stay without compromising the safety of patients.

Management of cardiovascular conditions in a cohort of patients with HIV: experience from a joint HIV/cardiology clinic.

The aim of this study was to assess cardiovascular diagnoses and management in a cohort of patients diagnosed with HIV, and the performance of a joint HIV/Cardiology Clinic in a tertiary hospital setting. A retrospective analysis was performed on all patients referred to a joint HIV/Cardiology Clinic at our hospital. Data on 120 patients were collected.

Uncertainty quantification in virtual surgery hemodynamics predictions for single ventricle palliation.

The adoption of simulation tools to predict surgical outcomes is increasingly leading to questions about the variability of these predictions in the presence of uncertainty associated with the input clinical data. In the present study, we propose a methodology for full propagation of uncertainty from clinical data to model results that, unlike deterministic simulation, enables estimation of the confidence associated with model predictions. We illustrate this problem in a virtual stage II single ventricle palliation surgery example.