Werner Budts

• MD PhD
• Head of Department at Universitair Ziekenhuis Leuven

Background and Aims Pregnancy in women with a prosthetic heart valve is considered high risk, primarily due to the need for effective anticoagulation. However, data on the relationship between anticoagulation practices and pregnancy outcomes are very limited. Methods The Registry of Pregnancy and Cardiac disease is a global registry that prospecti...

Background: The Melody™ TPV has been used as an alternative to surgical pulmonary valve replacement; limited medium-term follow-up data are available. Aims: To report the follow-up data of all Melody™ TPVs implanted locally over a 15-year period (2006-2021). Methods: Single-centre non-randomised prospective observational study of all implanted Melo...

Aims Atrial septal defect (ASD) and partial abnormal pulmonary venous connection (PAPVC) are noncyanotic congenital heart defects (CHD) that produce a left-to-right shunt. This single-center retrospective study aimed to assess the hemodynamic impact of isolated ASD, isolated PAPVC, and ASD-associated PAPVC using cardiovascular magnetic resonance (C...

Patients with a Fontan circulation are at risk for chronic kidney disease (CKD), which is defined as persistently reduced glomerular filtration rate (GFR) <60ml/min/1.73² or elevated marker of kidney injury such as urinary albumin-to-creatinine ratio (UACR) >30mg/g. We determined the prevalence of albuminuria in patients with a Fontan circulation....

Background The Mustard and Senning operations for dextro-transposition of the great arteries (D-TGA) establish a biventricular physiology with a subaortic right ventricle (sRV). While prolonged QRS has been associated with worse prognosis in these patients, current echocardiographic tools fall short in adequately assessing the (mal)performance and...

Background Atrial switch repair was the first surgical intervention to result in long-term survival in patients with ventriculo-arterial discordance or transposition of the great arteries. However, the natural history after atrial switch is not uneventful with frequent atrial arrhythmia, development of baffle stenosis and eventually heart failure....

OBJECTIVES This study aims to identify causes of failure in Fontan patients with a total cavopulmonary connection. METHODS We conducted a comprehensive review of all patients who underwent a total cavopulmonary connection procedure at our centre between 1988 and 2023, aiming to identify and analyze the factors contributing to Fontan failure (defin...

Background Advances in care have significantly improved survival in patients with adult congenital heart disease (ACHD). This growing population is now confronted with new challenges due to their structural malformations, surgical scars, cyanosis and/or longstanding abnormal pressure and volume load that lead to progressive atrial fibrosis. Atrial...

Purpose Ventricular-arterial coupling (VAC) reflects global cardiovascular efficiency. We may assess VAC state non-invasively by combining the non-invasive gold standards for arterial stiffness (carotid-femoral pulse wave velocity or PWV) and left ventricular systolic function (LV global longitudinal strain or GLS). To gain insight in the clinical...

As patients with congenital heart disease (CHD) continue to enjoy longer lives, non-congenital cardiologists and other healthcare professionals are increasingly encountering them in their practice and are challenged by their specific needs. Most under- and post-graduate medical training tends to overlook this common pathology, resulting in insuffic...

Background and aims The objective of this study was to assess the associations of birth weight with cardiac structure and function in adults with dextro-transposition of the great arteries (D-TGA) who underwent the arterial switch operation (ASO). Methods and results Thirty-nine ASO patients (age 24.4 ± 3.3 years) were included during routine clin...

Background Deep learning is the state-of-the-art approach for automated segmentation of the left ventricle (LV) and right ventricle (RV) in cardiovascular magnetic resonance (CMR) images. However, these models have been mostly trained and validated using CMR datasets of structurally normal hearts or cases with acquired cardiac disease, and are ther...

Nowadays cardiorespiratory fitness (CRF) is assessed using summary indexes of cardiopulmonary exercise tests (CPETs). Yet, raw time-series CPET recordings may hold additional information with clinical relevance. Therefore, we investigated whether analysis of raw CPET data using dynamic time warping combined with k-medoids could identify distinct CR...

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 pr...

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-H...

Objective To investigate the incidence of cardiovascular disease (CVD) overall and by age, sex, and socioeconomic status, and its variation over time, in the UK during 2000-19. Design Population based study. Setting UK. Participants 1 650 052 individuals registered with a general practice contributing to Clinical Practice Research Datalink and n...

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...

Background Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great arteries. Objectives The authors aimed to estimate the cumulative incidence of NeoAR, assess whether larger neoaortic root dimensions were associated with NeoAR, and eval...

Background: A first quality of life (QOL) study among Maltese adults with congenital heart disease (ACHD) in 2016 found no significant differences when compared to the general population. The aims of the present study were i) to compare QOL between Maltese and other European ACHD patients and ii) investigate medical predictors (i.e. number of surgi...

Background Childhood cancer survivors are at increased risk of developing cardiovascular diseases, presenting as the main causes of morbidity and mortality within this group. Besides the usual primary and secondary prevention in combination with screening during follow-up, the modifiable lifestyle factors of physical activity, nutrition, and body w...

Objectives This study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe. Methods A questionnaire was sent to ACHD cardiologists from 34 European countries. Results Representatives from 31 of 34 countries (91%) responded. ACHD cardiology was recognised by the respective ministry of Health in...

Background In Fontan patients, the slope of invasively measured pulmonary artery pressures plotted against cardiac output (CO) during exercise has prognostic significance. We propose a methodology using exercise echocardiography combined with cardiopulmonary exercise testing and simultaneous peripheral venous pressure measurements (CPET-echo-PVP) t...

Background and objectives Cardiopulmonary exercise testing (CPET) remains underutilized for cardiovascular (CV) risk assessment. Integrative interpretation of CPET results may improve characterization of cardiorespiratory fitness and assessment of CV risk. Therefore, we explored the clinical value of CPET-based phenomapping for CV risk stratificati...

Background Hereditary haemorrhagic telangiectasia (HHT), or Rendu–Osler–Weber syndrome, is a rare genetic disorder characterized by the development of telangiectasias and arteriovenous malformations (AVMs) throughout the body. We present a case of percutaneous embolization of pulmonary AVMs in an adult patient. Case summary A 26-year-old male pati...

In addition to arrhythmia, heart failure (HF) is one of the major late complications in adult congenital heart disease (ACHD). Residual lesions and sequelae of previous interventions might cause circulatory failure, with or without progressive ventricular dysfunction, leading to reduced functional capacity, signs and symptoms of HF, and finally pre...

In Part 3 of this 3-part series, we focus on the importance of transitioning young adult patients to adult care to ensure their optimal health and well-being into adulthood. The articles in this section bring to light the unique needs of these adolescents and how to better help them and their families navigate this time of transition. Available to...

Background: Exercise capacity is impaired in patients after arterial switch operation (ASO) for complete transposition of the great arteries. Maximal oxygen consumption is related with outcome. Objectives: This study assessed ventricular function by advanced echocardiography and cardiac magnetic resonance(CMR) imaging at rest and during exercise...

Funding Acknowledgements Type of funding sources: None. Background Ventricular compliance is known to have a direct impact on ventricular filling and therefore on diastolic function. Chamber compliance, in turn, is directly related to myocardial stiffness. The operational stiffness of the myocardium can be non-invasively assessed using shear wave...

Integrative interpretation of cardiopulmonary exercise tests (CPETs) may improve assessment of cardiovascular (CV) risk. Here, we identified patient phenogroups based on CPET summary metrics and evaluated their predictive value for CV events. We included 2280 patients with diverse CV risk who underwent maximal CPET by cycle ergometry. Key CPET indi...

The number of adult patients with congenital heart disease (CHD) is steadily increasing and exceeds nowadays the number of children with CHD. This is due to significant advances in therapeutic possibilities that became available over the last four decades. As such, this aging population survives the CHD complications and is exposed to the tradition...

Background: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries (D-TGA) repaired by an atrial switch operation. The aim of this study was to externally validate the model. Meth...

The development of collateral circulation is not that rare in patients with congenital heart defects. These collaterals can affect cardiovascular hemodynamics and cause systemic arterial desaturation, which arises the question whether these should be closed. To date, few if any reports have been published on the therapeutic management of collateral...

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 treatme...

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 w...

Purpose of review: Cardiovascular disease is increasingly emerging as a cause of peripartum morbidity and mortality. Peripartum cardiomyopathy (PPCM) is defined as pregnancy-related heart failure with a reduced left ventricular ejection fraction <45%. PPCM develops in the peripartum phase and is not an aggravation of an existing prepregnancy cardi...

Cardiovascular disease is a leading cause of morbidity and mortality in individuals with Down syndrome. Congenital heart disease is the most common cardiovascular condition in this group, present in up to 50% of people with Down syndrome and contributing to poor outcomes. Additional factors contributing to cardiovascular outcomes include pulmonary...

Objective Children and adults with repaired tetralogy of Fallot (rTOF) have an impaired exercise capacity, a less active lifestyle and an increased long-term risk of adverse outcomes compared with healthy peers. This study aimed to summarise the current evidence for the effectiveness and safety of exercise training interventions in patients with rT...

Objective To assess the effects of preterm birth on cardiac structure and function and transplant-free survival in patients with hypoplastic left heart syndrome and associated anomalies throughout the staged palliation process. Study design Data from the Single Ventricle Reconstruction (SVR) trial were used to assess the impact of prematurity on e...

Background: Heart failure (HF) is the primary cause of premature death in adult patients with congenital heart disease (ACHD). This study aims to describe the impact of a HF diagnosis on short-term prognosis and to investigate the added prognostic value of a HF diagnosis to the ACHD Anatomic and Physiological classification (ACHD-AP). Methods: T...

Nowadays most patients with a univentricular heart after Fontan repair survive until adulthood. One of the hallmarks of Fontan circulation is permanently elevated central venous pressure, which leads to congestive hepatopathy. Subsequently, liver fibrosis, cirrhosis, or hepatocellular carcinoma may occur, all of them constituting an entity called F...

Background: Mechanical factors may cause bottlenecks in a Fontan circuit. Extracardiac conduits (ECC) are placed at a young age, but the materials do not allow growth. Restriction in ECC dimensions may deteriorate the function of the circuit. Aims: This study aimed to evaluate the feasibility and safety of stent expansion of an ECC to the nomina...

Background At the beginning of the COVID-19 pandemic, professionals in charge of particularly vulnerable populations, such as adult congenital heart disease (ACHD) patients, were confronted with difficult decision-making. We aimed to assess changes in risk stratification and outcomes of ACHD patients suffering from COVID-19 between March 2020 and A...

Background Although many adults with congenital heart disease (CHD) still die prematurely, end-of-life care for these patients receives limited attention. There are indications that current care provision at the end of life is burdensome, expensive, and not in line with patients' needs and preferences. We sought to analyse end-of-life care in adult...

Background Clinical deterioration is fast once heart failure (HF) develops in patients with a systemic right ventricle (sRV) [1]. Despite its established role in HF patients with a normal heart anatomy [2,3], little is known about the contribution of inflammation in sRV-HF patients. Aims To assess inflammation and echocardiography in sRV patients...

Background Tetralogy of Fallot (ToF) remains associated with significant morbidity and mortality. Artificial intelligence (AI) is a viable tool for identifying markers: deep learning (DL) can be used to automate measurements on the ECG trace and dimensionality reduction (DR) algorithms can be used for grouping patients based on ECG, imaging and gen...

Objective To evaluate the available data on long-term kidney dysfunction, hypertension, and mortality after cardiac surgery-associated acute kidney injury (AKI) in the pediatric population. Study design PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for eligible studies published from inception through March...

Background and aims Although life expectancy in adults with congenital heart diseases (CHD) has increased dramatically over the past five decades, still a substantial number of patients dies prematurely. To gain understanding in the trajectories of dying in adults with CHD, the last year of life warrants further investigation. Therefore, our study...

The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40–59 years and 18–39 years. Adjusted for demogr...

Patient: Female, 67-year-old Final Diagnosis: Absence of right pulmonary artery • lung cancer • pulmonary stenosis Symptoms: Abdomen distension • dyspnea • fatigue • right heart failure Medication: — Clinical Procedure: Percutaneous pulmonary valve implantation • radiation therapy • right heart catherization Specialty: Cardiology • Pulmonology Obj...

Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Research Foundation Flanders (to PM, EG, and LVB) European Society of Cardiology (Nursing Training Grant to LVB) Background Heart failure (HF) is a common cause of morbidity and mortality in patients with congenital heart disease (CHD). Although limited in scope,...

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....

Purpose: Virtual reality (VR) can provide an added value for diagnosis and/or intervention planning. Several VR software implementations have been proposed but they are often application dependent. Previous attempts for a more generic solution incorporating VR in medical prototyping software (MeVisLab) were still lacking functionality precluding e...

Given the increasing use of transcatheter (para)valvular interventions and mechanical circulatory support, haemolysis is becoming a more frequently encountered complication. In this setting, patients should be actively monitored for signs of haemolysis, given the potential major adverse consequences. Although not commonly used, plasmapheresis is a...

Introduction Acute unilateral lung edema immediately following reperfusion of newly transplanted lung is rare. We present a case of a hemodynamic collapse with acute lung edema during a sequential single lung transplantation (LTx) for emphysema in a patient with post-operatively diagnosed double-chambered right ventricle (DCRV). Case Report A 61-y...

Background: Pulmonary arterial end‐diastolic forward flow (EDFF) following repaired tetralogy of Fallot has been thought to represent right ventricular (RV) restrictive physiology, but is not fully understood. This systematic review and meta‐analysis sought to clarify its physiological and clinical correlates, and to define a framework for understa...

Background: Acute kidney injury (AKI) after pediatric cardiac surgery is common. Longer‐term outcomes and the incidence of chronic kidney disease after AKI are not well‐known. Methods and Results: All eligible children (aged <16 years) who had developed AKI following cardiac surgery at our tertiary referral hospital were prospectively invited for...

Background: The benefits of pulmonary valve replacement (PVR) for pulmonary insufficiency in patients with repaired tetralogy of Fallot (TOF) are still incompletely understood, and optimal timing remains challenging. Methods: We systematically reviewed databases (PubMed/MEDLINE, EMBASE, CENTRAL/CCTR, ClinicalTrials.gov, SciELO, LILACS, Google Scho...

Background: Limited data exist on training of European paediatric and adult congenital cardiologists. Methods: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. Results: Delegates from 30 countries (91%) responded. Paediatric card...

Patients with a systemic right ventricle (sRV) have a high risk of developing heart failure (HF), with heart transplantation as the only long-term treatment option. Unfortunately, more than half of sRV patients have pulmonary hypertension (PH) which may impact the decision to opt for heart-only transplantation. Moreover, an increased scarcity of do...

Document Reviewers: Rudolf A. de Boer (CPG Review Coordinator) (Netherlands), P. Christian Schulze (CPG Review Coordinator) (Germany), Magdy Abdelhamid (Egypt), Victor Aboyans (France), Stamatis Adamopoulos (Greece), Stefan D. Anker (Germany), Elena Arbelo (Spain), Riccardo Asteggiano (Italy), Johann Bauersachs (Germany), Antoni Bayes‐Genis (Spain)...

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 performe...

BACKGROUND: Tricuspid regurgitation (TR) is a common finding in adults with congenital heart disease referred for pulmonary valve replacement (PVR). However, indications for combined valve surgery remain controversial. This study aimed to evaluate early results of concomitant tricuspid valve intervention (TVI) at the time of PVR. METHODS AND RESULT...

Built on the foundation of the randomized controlled trial (RCT), Evidence Based Medicine (EBM) is at its best when optimizing outcomes for homogeneous cohorts of patients like those participating in an RCT. Its weakness is a failure to resolve a clinical quandary: patients appear for care individually, each may differ in important ways from an RCT...

2020 ESC Guidelines for themanagement of adult congenital heart disease

Background Heart failure hospitalizations in adult patients with congenital heart disease (ACHD) are increasing and are associated with higher healthcare-related costs. We aimed to evaluate factors that are associated with repeated heart failure hospitalizations and whether heart failure hospitalizations are related to adverse outcome in ACHD patie...

Background Heart failure (HF) is one of the primary causes of premature death in patients with adult congenital heart disease (ACHD), yet population-specific data remains scarce. This study aims at assessing the short-term value of prognostic value of a heart failure diagnosis in patients with adult congenital heart disease, and its relation to ACH...

Background: Tricuspid regurgitation (TR) is a common finding in adults with congenital heart disease referred for pulmonary valve replacement (PVR). However, indications for combined valve surgery remain controversial. This study aimed to evaluate early results of concomitant tricuspid valve intervention (TVI) at the time of PVR. Material and metho...

Introduction A left to right (LR) shunt in atrial septal defect (ASD) may cause right heart and pulmonary overfilling, at the expense of the systemic circulation. Purpose The study objective was to evaluate the impact of LR shunt on left (LV) and right ventricular (RV) filling, function, and myocardial strain by using cardiovascular magnetic reson...

Background and objectives AKI is a common complication after pediatric cardiac surgery and has been associated with higher morbidity and mortality. We aimed to compare the efficacy of available pharmacologic and nonpharmacologic strategies to prevent AKI after pediatric cardiac surgery. Design, setting, participants, & measurements PubMed/MEDLINE,...

The growing population of women with heart disease of reproductive age has been associated with an increasing number of high-risk pregnancies. Pregnant women with heart disease are a very heterogeneous population, with different risks for maternal cardiovascular, obstetric, and foetal complications. Adverse cardiovascular events during pregnancy po...

AimTo scrutinize the economic impact of different care levels, such as shared care, in the follow-up of adult congenital heart disease (ACHD) patients.Methods The BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC) was analyzed. Patients (N = 6579) were categorized into five care levels based on their car...

Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): This work was supported by Research Foundation Flanders [grant numbers G097516N to PM, 12E9816N to EG and 1154719N to LVB]; the King Baudouin Foundation (Fund Joseph Oscar Waldmann-Berteau & Fund Walckiers Van Dessel); the National Foun...

Funding Acknowledgements Type of funding sources: Other. Main funding source(s): This research received project funding by KU Leuven Background – Early recognition of adverse remodeling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to sh...

Background Patients with adult congenital heart disease (ACHD) have many risk factors for chronic pain such as prior cardiac interventions and adult comorbidities. However, the prevalence of chronic pain has not been well described in this population. We sought to determine the prevalence of pain in a large international cohort of patients with ACH...

Aims: The Fontan operation has resulted in improved survival in patients with single-ventricle congenital heart disease. As a result, there is a growing population of teenagers and adults with a Fontan circulation. Many co-morbidities have been increasingly recognized in this population due to the unique features of the Fontan circulation. Standar...

Aims Early recognition of adverse remodelling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to short-term clinical evolution in this population. Methods and results Thirty-three patients (76% male) with sRV (atrial switch repair for D-t...

Background: Congenital heart disease (CHD) is the most common type of major congenital malformations. CHD outcomes have improved in the past 20 years, and research efforts in the field have increased exponentially. The purpose of this investigation is to identify the most influential recent publications related to CHD, and reveal their shared featu...

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. Metho...

Background: Influenza vaccination is the most commonly recommended immune prevention strategy. However, data on influenza vaccination in patients with congenital heart disease (CHD) is scarce. In this study, our goals were to (i) measure vaccination coverage rates (VCR) for influenza in a large cohort of children, adolescents and adults with CHD,...