Physical exercise barriers and needs in adults with congenital heart disease: a qualitative study.

Objective: Regular physical exercise has well-known health benefits and is generally considered safe for adults with congenital heart disease (ACHD). However, many individuals with ACHD remain insufficiently physically active. This study explored the barriers and needs related to physical exercise as experienced by people with ACHD to inform the development of tailored strategies that support and promote increased physical activity.

Aortic Function in a Longitudinal 4D Flow MRI Study in Marfan Syndrome Patients Receiving Resveratrol.

Background: New treatment strategies are required to reduce aortic events in Marfan syndrome (MFS). Resveratrol is a dietary supplement that intervenes in aortic wall cellular metabolism and may benefit MFS patients.

Purpose: To evaluate whether treatment with Resveratrol affects aorta hemodynamics derived from 4D flow MRI in MFS.

Effects of resveratrol on aortic growth in patients with Marfan syndrome: a single-arm open-label multicentre trial.

Background: Resveratrol, a dietary supplement that intervenes in cellular metabolism, has been shown to reduce aortic growth rate in a mouse model of Marfan syndrome (MFS), a condition associated in humans with life-threatening aortic complications, often preceded by aortic dilatation. The primary objective of this study was to investigate the effects of resveratrol on aortic growth rate in patients with MFS .

Methods: In this investigator-initiated, single-arm open-label multicentre trial, we analysed resveratrol treatment in adults aged 18-50 years with MFS.

Tolerability and beneficial effects of sacubitril/valsartan on systemic right ventricular failure.

Objective: Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are prone to sRV dysfunction. Pharmacological options for sRV failure remain poorly defined. This study aims to investigate the tolerability and effects of sacubitril/valsartan on sRV failure in adult patients with sRV.

A comparison of ECG-based home monitoring devices in adults with CHD.

Background: Various electrocardiogram (ECG)-based devices are available for home monitoring, but the reliability in adults with CHD is unknown. Therefore, we determined the accuracy of different ECG-based devices compared to the standard 12-lead ECG in adult CHD.

Methods And Results: This is a single-centre, prospective, cross-sectional study in 176 consecutive adults with CHD (54% male, age 40 ± 16.

Pre-procedural virtual reality education reduces anxiety in patients undergoing atrial septal closure - Results from a randomized trial.

Background: Patients undergoing invasive cardiothoracic procedures are prone for pre-procedural anxiety and depression. Patient education is known to reduce anxiety. This study was performed to assess the effect of Virtual Reality (VR) as a means to educate patients to reduce pre-procedural anxiety in adult patients undergoing percutaneous closure of a patent foramen ovale (PFO) or atrial septal defect (ASD).

Patient information portal for congenital aortic and pulmonary valve disease: a stepped-wedge cluster randomised trial.

Background: In response to an increased need for patient information in congenital heart disease, we previously developed an online, evidence-based information portal for patients with congenital aortic and pulmonary valve disease. To assess its effectiveness, a stepped-wedge cluster randomised trial was conducted.

Methods: Adult patients and caregivers of paediatric patients with congenital aortic and/or pulmonary valve disease and/or tetralogy of Fallot who visited the outpatient clinic at any of the four participating centres in the Netherlands between 1 March 2016-1 July 2017 were prospectively included.

Identification of patients at risk of sudden cardiac death in congenital heart disease: The PRospEctiVE study on implaNTable cardIOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD).

Background: Sudden cardiac death (SCD) is the main preventable cause of death in patients with adult congenital heart disease (ACHD). Since robust risk stratification methods are lacking, we developed a risk score model to predict SCD in patients with ACHD: the PRospEctiVE study on implaNTable cardIOverter defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease (PREVENTION-ACHD) risk score model.

Objective: The purpose of this study was to prospectively study predicted SCD risk using the PREVENTION-ACHD risk score model and actual SCD and sustained ventricular tachycardia/ventricular fibrillation (VT/VF) rates in patients with ACHD.

Tricuspid regurgitation after transcatheter mitral valve repair: Clinical course and impact on outcome.

Objectives: The aim of this study was to determine the course of tricuspid regurgitation (TR) after transcatheter mitral valve repair (TMVR), identify predictors for severe TR after TMVR and determine the association of severe TR after TMVR with outcome.

Background: TR is often present in patients with symptomatic mitral regurgitation (MR) and is associated with increased morbidity and mortality. The clinical course of TR after TMVR has not been clearly determined.

Medication in adults after atrial switch for transposition of the great arteries: clinical practice and recommendations.

Aims: Heart failure is the main threat to long-term health in adults with transposition of the great arteries (TGA) corrected by an atrial switch operation (AtrSO). Current guidelines refrain from recommending heart failure medication in TGA-AtrSO, as there is insufficient data to support the hypothesis that it is beneficial. Medication is therefore prescribed based on personal judgements.

Advantages of mobile health in the management of adult patients with congenital heart disease.

Background: Adults with congenital heart disease (ACHD) often suffer from deterioration related to cardiac arrhythmias, hypertension (HT) or heart failure (HF), frequently occurring between planned visits. Mobile health (mHealth) could improve management through remote monitoring by enabling swift therapeutic response and detecting new diagnoses.

Methods: We performed a prospective study employing mHealth in ACHD patients, weekly monitoring heart rhythm, weight and blood pressure.

Complementary role of cardiac computed tomography angiography in the diagnosis of prosthetic aortic valve endocarditis and septic coronary embolism - a case report.

A 73-year old man presented with a posterolateral ST-elevated myocardial infarction 9 months after biological aortic valve replacement for aortic valve stenosis. Invasive coronary angiography showed a filling defect across the left main coronary artery bifurcation extending into the left anterior descending artery and the ramus circumflex. Transthoracic echocardiography revealed a thickened prosthesis leaflet with signs of slight stenosis.

Staged total cavopulmonary connection: serial comparison of intra-atrial lateral tunnel and extracardiac conduit taking account of current surgical adaptations.

Objectives: Our goals were to compare the outcome of the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC) techniques for staged total cavopulmonary connection (TCPC) and to compare the current modifications of the TCPC technique, i.e. the prosthetic ILT technique with the current ECC technique with a ≥18-mm conduit.

Aortic valve stenosis and aortic diameters determine the extent of increased wall shear stress in bicuspid aortic valve disease.

Background: Use of 4-dimensional flow magnetic resonance imaging (4D-flow MRI) derived wall shear stress (WSS) heat maps enables identification of regions in the ascending aorta with increased WSS. These regions are subject to dysregulation of the extracellular matrix and elastic fiber degeneration, which is associated with aortic dilatation and dissection.

Purpose: To evaluate the effect of the presence of aortic valve stenosis and the aortic diameter on the peak WSS and surface area of increased WSS in the ascending aorta.

Knowledge-based reconstruction for measurement of right ventricular volumes on cardiovascular magnetic resonance images in a mixed population.

Objective: Follow-up of right ventricular performance is important for patients with congenital heart disease. Cardiac magnetic resonance imaging is optimal for this purpose. However, observer-dependency of manual analysis of right ventricular volumes limit its use.

Quantification of myocardial deformation in children by cardiovascular magnetic resonance feature tracking: determination of reference values for left ventricular strain and strain rate.

Background: The objective assessment of global and regional cardiac function in children has shown to be clinically relevant but is challenging to conduct. Cardiovascular magnetic resonance (CMR) has emerged as a valuable diagnostic modality especially in patients with cardiomyopathy or congenital heart disease. However, data on the normal cardiac deformation in children assessed by CMR is lacking at present.

Enlarged right ventricular size at 11 years' follow-up after closure of secundum-type atrial septal defect in children.

Background: The fate of right ventricular dimensions after surgical closure of secundum-type atrial septal defects remains unclear. The objectives of this study were to assess ventricular dimensions, exercise capability, and arrhythmias of patients operated for secundum-type atrial septal defect and compare the results with those in healthy references.

Methods: A total of 78 consecutive patients underwent surgical closure for a secundum-type atrial septal defect between 1990 and 1995.