External Validation of a Risk Score Model for Predicting Major Clinical Events in Adults After Atrial Switch.

Background: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model.

Methods And Results: A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied.

Biventricular performance in adults with a systemic right ventricle: new insights from myocardial work analysis.

To evaluate biventricular mechanics by means of echo-derived myocardial work (MW) analysis in patients with a systemic right ventricle (sRV). Comprehensive echo data were collected in all patients with a sRV who underwent transthoracic echocardiography at our tertiary centre between 2020 and 2021 including sRV function indices, global longitudinal strain (GLS) of right and left ventricle (RV/LV), biventricular MW, and atrial strain in those with congenitally corrected transposition of the great arteries (ccTGA). Fifty-six patients (37 [30.

End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

Background And Aims: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear.

Methods: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained.

Fulminant biventricular multivalvular infective endocarditis in complex congenital heart disease: a case report.

Background: Infective endocarditis (IE) is a major issue during follow-up of adults with congenital heart disease (ACHD), leading to significant mortality.

Case Summary: A 37-year-old woman with transposition of great arteries and previous Mustard operation developed a drug-resistant pneumonia shortly after a pacemaker implant procedure performed at a local hospital. After referral to the ACHD centre, the patient was diagnosed with multivalvular IE with biventricular involvement by methicillin-resistant .

Telemedicine in Adult Congenital Heart Disease: Usefulness of Digital Health Technology in the Assistance of Critical Patients.

The number of adults with congenital heart disease (ACHD) has progressively increased in recent years to surpass that of children. This population growth has produced a new demand for health care. Moreover, the 2019 coronavirus pandemic has caused significant changes and has underlined the need for an overhaul of healthcare delivery.

Infective Endocarditis in Adults With Congenital Heart Disease: Never Stop Digging-The Role of Multimodality Imaging.

A challenging case of infective endocarditis in a young woman with repaired tetralogy of Fallot and a diagnosis of ankylosing spondylitis is described. Despite the presence of multiple confounding factors, a multidisciplinary approach with the use of multimodality cardiac imaging allowed a correct diagnosis and effective medical treatment. ().

Mid-term follow-up after COVID-19 vaccination in adults with CHD: a prospective study.

Background: Long-term data on COVID-19 vaccine safety, immunogenicity, and acceptance in adults with CHD are lacking.

Methods: This is a prospective study including adults with CHD patients undergoing COVID-19 vaccination from January 2021 to June 2022. Data on adverse events, antispike IgG titre, previous or subsequent COVID-19 infection, booster doses, and patients' attitude towards vaccination were collected.

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study.

Background: Sacubitril/valsartan was demonstrated to reduce hospitalization rate and mortality in patients with heart failure with reduced ejection fraction. Data on the effects of sacubitril/valsartan in patients with a systemic right ventricle are still lacking.

Methods: Patients with transposition of the great arteries following Senning/Mustard procedure or congenitally corrected transposition of the great arteries with impaired systemic right ventricle systolic function were prospectively included.

Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries.

Background: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes.

Objectives: The authors aimed to determine factors associated with survival in a large cohort of such individuals.

Methods: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center.

Wearable cardioverter-defibrillator (life-vest): A feasible bridging treatment in adult congenital heart disease.

Background: Wearable cardioverter-defibrillators (WCDs) are currently used in patients at temporarily heightened risk for sudden cardiac death (SCD) who are temporarily unable to receive an implantable cardioverter-defibrillator (ICD). WCD can safely record and terminate life-threatening arrhythmias through a non-invasive electrode-based system. The current clinical indications for WCD use are varied and keep evolving as experience with this technology increases.

A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle.

Aims: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV).

Methods And Results: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed.

Prognostic Relevance of Thyroid Disorders in Adults With Congenital Heart Disease.

Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TDs). However, the clinical relevance of TD in ACHD remains unknown. We aimed to describe the prevalence of TD in the ACHD population and to ascertain whether TD are associated with worse outcome.

Chylothorax Due to Superior Vena Cava Obstruction in a Patient With Complex Congenital Heart Disease.

Obstruction of the superior vena cava represents an under-recognized cause of chylothorax in the adult population. Our case report describes the successful conservative management of chylothorax due to bilateral superior vena cava obstruction in an adult patient with complex congenital heart disease. ().

COVID-19 in Adults With Congenital Heart Disease.

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications.

Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes.

Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide.

Pulmonary valve endocarditis in adults with congenital heart disease: the role of echocardiography in a case series.

Background: Pulmonary valve (PV) endocarditis is a frequent complication during follow-up in patients with repaired right ventricular outflow tract (RVOT) obstruction and poses relevant diagnostic and treatment challenges. We aimed to describe in details the possible different clinical presentations of this rare condition and to highlight the role of both transthoracic and transoesophageal echocardiography which, in experienced hands, may provide comprehensive useful information for the clinicians.

Case Summary: We below describe the clinical presentation and the echo findings of three cases of pulmonary valve endocarditis complicating disease course after different repair modalities of congenital right ventricular outflow tract obstruction.

Safety and efficacy of non-vitamin K antagonist oral anticoagulants for prevention of thromboembolism in adults with systemic right ventricle: Results from the NOTE international registry.

Background: Patients with systemic right ventricle (sRV), including transposition of great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of great arteries (ccTGA), may require anticoagulation for thromboembolism (TE) prevention. In the absence of data on non-vitamin K antagonist oral anticoagulants (NOACs), vitamin K antagonists (VKAs) remain the agent of choice. We investigated the safety, efficacy and feasibility of NOACs treatment in adults with sRV in a worldwide study.