Subpulmonary ventricular function and inflammation are related to clinical heart failure in patients with a systemic right ventricle.

Background: Timely diagnosis of heart failure (HF) in patients with a systemic right ventricle (sRV) is difficult but important since clinical deterioration is fast once HF develops. We aimed to compare echocardiography and biomarker profile between sRV patients with and without HF and patients with a systemic left ventricle diagnosed with HF (sLV-HF).

Methods And Results: Eighty-seven sRV patients and 30 sLV-HF patients underwent echocardiographic evaluation and blood sampling.

Atrial arrhythmia and heart failure in adult patients with congenital heart disease: a retrospective cohort study.

Background: Atrial arrhythmias (AA) and heart failure (HF) are major causes of hospitalisation in adult congenital heart disease (ACHD). This study aimed to evaluate the temporal relationship between AA and HF onset, the association between HF and the success of radiofrequency ablation (RFA), and how HF influences outcomes in patients with AA.

Methods: In this single-centre retrospective cohort study, data from 3995 patients with ACHD were analysed.

A retrospective study: Long term prognosis in adults with PA-VSD-MAPCAs.

Background: Pulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment.

Long-term outcome after the Ross procedure in 173 adults with up to 25 years of follow-up.

Objectives: The potential risk of autograft dilatation and homograft stenosis after the Ross procedure mandates lifelong follow-up. This retrospective cohort study aimed to determine long-term outcome of the Ross procedure, investigating autograft and homograft failure patterns leading to reintervention.

Methods: All adults who underwent the Ross procedure between 1991 and 2018 at the University Hospitals Leuven were included, with follow-up data collected retrospectively.

Aorta pathology and pregnancy-related risks in adult congenital cardiac disease: does the aorta dilate during pregnancy?

Background: Aortic dilatation and pregnancy are major concerns in women with aortopathy (AOP). This single-centre retrospective analysis focuses on the evolution of aortic diameters during and after pregnancy in women with Marfan syndrome (MS), Turner syndrome (TS) and bicuspid aortic valve (BAV) aortopathy.

Methods And Results: Thirty-eight women who had one or more single pregnancies were included.

Left atrial strain in patients after arterial switch operation for transposition of the great arteries.

Background: Left atrial (LA) strain, comprising LA reservoir, conduit and contractile function could add mechanistic information of patients after arterial switch operation (ASO) for transposition of the great arteries (TGA). ASO patients might have abnormal ventriculoarterial coupling, which makes them vulnerable to left ventricle (LV) dysfunction and results in reduced exercise capacity. This explorative study aimed to evaluate the relation between LA strain, atrial size, ventricular function, and exercise data obtained by cardiopulmonary exercise testing (CPET).

Biomarkers in transposition of the great arteries after arterial switch operation: A pilot trial with deep phenotyping.

Introduction: Transposition of the great arteries (TGA) is a cyanotic congenital heart defect for which the arterial switch operation (ASO) is the preferred surgical repair. This study wanted to investigate whether a panel of biomarkers could identify morphologic as well as hemodynamic changes obtained by cardiac magnetic resonance (CMR).

Methods: Forty-four adult patients were included.

Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect.

Background And Aims: Patients with Down Syndrome (DS) are frequently born with an atrioventricular septal defect (AVSD). Surgical repair of the defect aims to minimize mortality and morbidity. However, a surgical intervention, specifically in DS patients, is not without risk and a subgroup of patients underwent only conservative non-surgical treatment.

Short-term results of serial cardiopulmonary exercise testing in adults with repaired coarctation of the aorta.

Background: Data on the evolution of exercise capacity in adults with repaired coarctation of the aorta (CoA) are scarce. We aimed to investigate the evolution and change of measures of exercise capacity obtained by cardiopulmonary exercise testing (CPET) in adults with repaired CoA.

Methods: Patients 16 years of age and older with CoA, who performed at least two maximal CPETs in our institution, were included in the study.

Heart failure related to adult congenital heart disease: prevalence, outcome and risk factors.

Aims: Information on the prevalence, outcome and factors associated with heart failure in patients with adult congenital heart disease (CHD) (ACHD-HF) is lacking. We aimed at assessing the prevalence and outcome of ACHD-HF, the variables associated with ACHD-HF, and the differences between major anatomical/pathophysiological ACHD subgroups.

Methods And Results: We included 3905 patients (age 35.

Covered stent placement for treatment of coarctation of the aorta: immediate and long-term results.

Objectives: This study aimed to describe the safety and efficacy of covered stents in patients with coarctation of aorta (CoA) for immediate and long-term follow-up.

Background: Covered stents are increasingly being used in (re)CoA, mainly to reduce the risk of aortic wall injuries (AWI). However, limited data are available on intermediate and long-term outcome.

15-Year follow-up of regional right and left ventricular function after the Senning operation: a Colour-Doppler myocardial imaging study.

Introduction: Despite right ventricular (RV) dysfunction being a major concern in Senning patients, long-term follow-up data is lacking. This study aimed (1) at evaluating regional (base-mid-apex) RV and left ventricular (LV) function using Colour-Doppler myocardial imaging over a 15-year follow-up period and (2) at comparing results with matched controls.

Methods: For the longitudinal analysis (2004-2019), we compared systolic and diastolic function in 10 Senning patients.

Perioperative Risk Factors for Mortality in Patients Undergoing Surgery for Ebstein's Anomaly at a Single Institution: A Retrospective Analysis.

Background: Mortality after cardiac surgery for Ebstein's anomaly ranges from 2.5% to 31%. Independent predictors for mortality and morbidity remain poorly defined because of the low incidence of this congenital anomaly.

Outcome of arterial switch operation for transposition of the great arteries. A 35-year follow-up study.

Background: Arterial switch operation (ASO) is today the first-choice surgical treatment for patients with transposition of the great arteries. Long-term outcome data still remain scarce. Moreover, the course of these patients is not uneventful.

Percutaneous closure of an uncommon aortic pseudoaneurysm after arterial switch repair: a case report.

Background: The development of an aortic pseudoaneurysm is a rather rare but potentially fatal complication after cardiac surgery for aortic valve and aorta disease. If a pseudoaneurysm is left untreated, it carries a substantial risk of rupture, thrombosis with subsequently systemic embolization, and compression of the surrounding structures.

Case Summary: We describe a case of a transcatheter repair of a more complex and uncommon pseudoaneurysm following aortic valve replacement in a patient with a history of arterial switch repair for transposition of the great arteries.

Advanced care planning in adult congenital heart disease: Transitioning from repair to palliation and end-of-life care.

As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2].

Advanced Imaging to Phenotype Patients With a Systemic Right Ventricle.

Background Reduced ventricular function and decreased exercise capacity are widespread in adults with complete transposition of the great arteries after atrial switch ( TGA -Mustard/Senning) and congenitally corrected TGA (cc TGA ). Advanced imaging techniques may help to better phenotype these patients and evaluate exercise cardiac response. Methods and Results Thirty-three adults with a systemic right ventricle (70% TGA -Mustard/Senning, 37±9 years of age, 24% female, 94% New York Heart Association class I- II ) underwent echocardiogram, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging at rest and during a 4-stage free-breathing bicycle test.

Up to 11 years of experience with the Melody valved stent in the right ventricular outflow tract.

Aims: The aim of the study was to report up to 11 years of follow-up after Melody valve implantation in the pulmonary position.

Methods And Results: This was a single-institution non-randomised prospective observational study of all Melody valves in the pulmonary position after discharge between 2006 and 2017 (n=188). Mean age was 19.

Right ventricular systolic dysfunction at rest is not related to decreased exercise capacity in patients with a systemic right ventricle.

Background: To evaluate the relationship between right ventricular (RV) systolic dysfunction at rest and reduced exercise capacity in patients with a systemic RV (sRV).

Methods: All patients with congenitally corrected transposition of the great arteries (ccTGA) or complete TGA after atrial switch (TGA-Mustard/Senning) followed in our institution between July 2011 and September 2017 who underwent cardiac imaging within a six-month time period of cardiopulmonary exercise testing (CPET) were analyzed. We assessed sRV systolic function with TAPSE and fractional area change on echocardiogram and, if possible, with ejection fraction, global longitudinal and circumferential strain on cardiac magnetic resonance (CMR) imaging.

Management of acute heart failure in adult patients with congenital heart disease.

Heart failure is an increasing reason for hospitalization and the leading cause of death in patients with adult congenital heart disease (ACHD). Recently, the European Society of Cardiology and the American Heart Association published consensus documents on the management of chronic heart failure in ACHD patients. However, little data and/or guidelines are available for the management of (sub)acute heart failure.

Outcome after cardiopulmonary resuscitation in patients with congenital heart disease.

Background: Outcome after cardiopulmonary resuscitation (CPR) in patients with underlying congenital heart disease is uncertain. This study aimed at evaluating outcome after CPR in patients with underlying congenital heart disease, factors related to worse outcome after CPR and whether survivors of sudden cardiac death (SCD) have a worse outcome when compared to an age, gender and disease-matched control population.

Methods: Between 1984 and 2015, all patients with congenital heart disease who received in or out-of-hospital CPR were identified from the database of congenital heart disease from the University Hospitals Leuven.