Aortic coarctation and long-term morbidity after arterial switch operation: a multicentre international cohort study.

Background: In patients with dextro-transposition of the great arteries, cardiovascular interventions and complications are common after the arterial switch operation (ASO). While complex anatomy-typically defined by ventricular septal defects (VSDs)-is often linked to these outcomes, the independent role of aortic coarctation (CoA) remains unclear.

Methods: We analysed 502 adults from the EPOCH (European collaboration for Prospective Outcome research in Congenital Heart disease)-ASO multicentre registry (median age 25.

Stable Longitudinal Quality of Life in the SERVE Trial Among Adults With Transposition of the Great Arteries and a Systemic Right Ventricle.

Background: Adults with a transposition anatomy and a systemic right ventricle (RV) face long-term complications that may impact their quality of life (QoL). Few data are available regarding the QoL in this patient group and its evolution over time.

Methods: This study was performed in the SERVE trial's (identifier: NCT03049540) prospective cohort of patients (n = 100) with congenitally corrected transposition of the great arteries (TGA) or dextro-TGA after the atrial switch procedure and a longitudinal follow-up of 3 years.

Effect of phosphodiesterase-5 inhibition on SystEmic Right VEntricular size and function. A multicentre, double-blind, randomized, placebo-controlled trial: SERVE.

Aims: In adults with congenital heart disease and systemic right ventricles, progressive right ventricular systolic dysfunction is common and is associated with adverse outcomes. Our aim was to assess the impact of the phosphodiesterase-5-inhibitor tadalafil on right ventricular systolic function.

Methods And Results: This was a double-blind, randomized, placebo-controlled, multicentre superiority trial (NCT03049540) involving 100 adults with systemic right ventricles (33 women, mean age: 40.

Intracardiac bone cement embolism resulting in ventricular perforation: an unusual cause of sudden chest pain.

Leakage of bone cement is a known complication after percutaneous kyphoplasty. In rare cases, bone cement can reach the venous system and cause life-threatening embolism. We present the case of a 73-year-old male, who was admitted to our hospital with new-onset chest pain and dyspnoea.

Thromboprophylaxis in Patients With Fontan Circulation.

Background: The optimal strategy for thromboprophylaxis in patients with a Fontan circulation is unknown.

Objectives: The aim of this study was to compare the efficacy and safety of aspirin, warfarin, and nonvitamin K oral anticoagulants (NOACs) in a network meta-analysis.

Methods: Relevant studies published by February 2022 were included.

Atrial function in the Fontan circulation: comparison with invasively assessed systemic ventricular filling pressure.

Abnormal atrial mechanics in biventricular circulations have been associated with elevated left heart filling pressures. Similar associations in the Fontan circulation are unknown. The aim of this study was to examine the relationship between atrial mechanics and invasively assessed hemodynamic parameters late after the Fontan operation.

Hepatic Steatosis in Patients With Single Ventricle and a Fontan Circulation.

Background Hepatic steatosis, caused by nonalcoholic fatty liver disease, is a leading cause of chronic liver disease. The interplay between hepatic steatosis and the development of liver disease following the Fontan procedure is not well understood. This study examined the prevalence and associations of hepatic steatosis in patients with a Fontan circulation.

Dynamic exercise changes in venous pressure and liver stiffness in Fontan patients: effects of Treprostinil.

Background: Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response.

Ebstein's Anomaly of the Tricuspid Valve: an Overview of Pathology and Management.

Purpose Of Review: Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population.

Recent Findings: Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence.

Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes.

Background: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation.

Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality.

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology.

Risk Factors for Mortality and Ventricular Tachycardia in Patients With Repaired Tetralogy of Fallot: A Systematic Review and Meta-analysis.

Background: Patients with repaired tetralogy of Fallot (rTOF) have increased risk for mortality, sudden cardiac death, and ventricular tachycardia (VT). The aim of this systematic review and meta-analysis is to offer an updated analysis of risk factors following significant changes in surgical and perioperative management.

Methods: A meta-analysis based on the published literature between 2008 and 2018 was conducted.

Lymphopenia in adults after the Fontan operation: prevalence and associations.

Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes.

Abdominal Skeletal Muscle Index as a Potential Novel Biomarker in Adult Fontan Patients.

Background: Fontan palliation results in a chronic multisystem disorder with diminished exercise capacity and increased risk of muscle wasting. The aims of this study were to assess the feasibility of skeletal muscle mass measurements in Fontan patients undergoing magnetic resonance imaging liver surveillance to compare muscle mass with a historic control and to assess its correlation with cardiorespiratory fitness.

Methods: Skeletal muscle area (SMA) and skeletal muscle index (SMI) were measured at T12 and L3.

Thromboembolic Events Are Independently Associated with Liver Stiffness in Patients with Fontan Circulation.

Thromboembolism (TE) and Fontan-associated liver disease (FALD) are common and lead to significant morbidity in Fontan circulations. Risk factors for TE and the potential link between TE and FALD are not well understood. The objective of this study was to evaluate the association between TE and the severity of FALD based on radiologic liver stiffness.

Timing of Repair in Tetralogy of Fallot: Effects on Outcomes and Myocardial Health.

Early complete repair of tetralogy of Fallot (ToF) prior to 1 year of age has been demonstrated to be safe and has survival benefits over late repair. The age at repair of ToF affects long-term outcomes. This may largely be related to preserved, or comparatively better, myocardial health.

Relation of Magnetic Resonance Elastography to Fontan Failure and Portal Hypertension.

Fontan associated liver disease is associated with morbidity and mortality in palliated single-ventricle congenital heart disease patients. Magnetic resonance elastography (MRE) provides a quantitative assessment of liver stiffness in Fontan patients. We hypothesized that MRE liver stiffness correlates with liver enzymes, hemodynamics, portal hypertension, and Fontan failure (FF).

Myocardial blood flow and cardiac sympathetic innervation in young adults late after arterial switch operation for transposition of the great arteries.

Background: The arterial switch operation (ASO) for repair of transposition of the great arteries (TGA) requires transection of the great arterial trunks and re-implantation of the coronary arteries into the neoaortic root resulting in cardiac sympathetic denervation which may affect myocardial blood flow (MBF) regulation. The aims of the present study were to evaluate sympathetic (re-)innervation in young adults after ASO and its impact on MBF.

Methods: Twelve patients (age 22.

Quantification of intrathoracic fat adds prognostic value in women undergoing myocardial perfusion imaging.

Aim: Amongst patients with coronary artery disease (CAD), women experience relatively worse outcomes as compared to men. Evidence to date has failed to explore unique female imaging targets as major determinants of cardiovascular risk. We sought to assess the prognostic value of epicardial (EFV) and intrathoracic fat volume (IFV) quantification in women and men with suspected and known CAD.

Corrected coronary opacification decrease from coronary computed tomography angiography: Validation with quantitative 13N-ammonia positron emission tomography.

Background: To assess the functional relevance of a coronary artery stenosis, corrected coronary opacification (CCO) decrease derived from coronary computed tomography angiography (CCTA) has been proposed. The present study aims at validating CCO decrease with quantitative 13N-ammonia positron emission tomography (PET) myocardial perfusion imaging (MPI).

Methods And Results: This retrospective study consists of 39 patients who underwent hybrid CCTA/PET-MPI.

Long-term outcome prediction by functional parameters derived from coronary computed tomography angiography.

Background: Estimation of hemodynamic relevance of a coronary stenosis from coronary computed angiography (CCTA) has raised substantial interest. Recently, the corrected coronary opacification (CCO) decrease and the transluminal attenuation gradient (TAG) have been suggested as faster alternatives to the FFR. The aim of the study was to evaluate whether the diagnostic accuracy of CCO decrease and TAG translates into an added prognostic value in patients evaluated for coronary artery disease (CAD).

Long-term prognostic performance of low-dose coronary computed tomography angiography with prospective electrocardiogram triggering.

Objectives: To assess long-term prognosis after low-dose 64-slice coronary computed tomography angiography (CCTA) using prospective electrocardiogram-triggering.

Methods: We included 434 consecutive patients with suspected or known coronary artery disease referred for low-dose CCTA. Patients were classified as normal, with non-obstructive or obstructive lesions, or previously revascularized.

A low-dose and an ultra-low-dose contrast agent protocol for coronary CT angiography in a clinical setting: quantitative and qualitative comparison to a standard dose protocol.

Objective: To evaluate the impact of a low-dose (LD) and an ultra-LD (ULD) contrast protocol for coronary CT angiography on qualitative and quantitative image parameters in a clinical setting.

Methods: We scanned 120 consecutive patients with a 256-slice CT scanner applying a LD (60 patients, 35-55 ml) or ULD (60 patients, 25-45 ml) contrast protocol adapted to the body mass index. Visually assessed image quality and attenuation measured in each coronary segment were retrospectively compared in 20 consecutive patients scanned with a normal-dose (ND, 40-105 ml) contrast protocol.