Homograft durability after correction of pulmonary atresia and ventricular septal defect with or without systemic pulmonary collateral arteries.

Background: Pulmonary atresia and ventricular septal defect (PA-VSD), with or without systemic pulmonary collateral arteries (SPCAs), represents a complex anatomic and surgical spectrum of congenital heart disease. Currently, there is limited evidence on homograft durability after complete correction, which potentially could be affected by anatomic differences in pulmonary vasculature.

Methods: This retrospective single-center study included all 69 consecutive PA-VSD patients (46 with SPCAs, 23 without SPCAs) operated on between 1978 and 2018.

Hypertensive response to exercise in adult patients with repaired aortic coarctation.

Objective: The clinical and prognostic implications of a hypertensive response to exercise after repair of coarctation of the aorta (CoA) remain controversial. We aimed to determine the prevalence of a hypertensive response to exercise, identify factors associated with peak exercise systolic blood pressure (SBP) and explore the association of peak exercise SBP with resting blood pressure and cardiovascular events during follow-up.

Methods: From the Dutch national CONgenital CORvitia (CONCOR) registry, adults with repaired CoA who underwent exercise stress testing were included.

Cardiovascular Morbidity and Mortality in Adult Patients With Repaired Aortic Coarctation.

Background The long-term burden of cardiovascular disease after repair of coarctation of the aorta (CoA) has not been elucidated. We aimed to determine the incidence of and risk factors for cardiovascular events in adult patients with repaired CoA. Additionally, mortality rates were compared between adults with repaired CoA and the general population.

Surgical repair of aortic coarctation in adults: half a century of a single centre clinical experience.

Objectives: Coarctation of the aorta (CoA) is rarely diagnosed and treated in adults and nowadays mostly treated with percutaneous techniques. The objective of this study is to report the long-term outcomes and health-related quality of life in a unique cohort of patients treated with an open surgical repair for their primary adult CoA.

Methods: Ninety adult patients underwent primary surgical repair of CoA at our tertiary referral centre between 1961 and 2008 when the treatment strategy for adult CoA was exclusively surgical.

The Prognostic Value of Myocardial Deformation in Adult Patients With Corrected Tetralogy of Fallot.

Background: Adult patients with repaired tetralogy of Fallot (ToF) are at risk for complications such as heart failure and sudden cardiac death, and identifying high-risk patients is important. Reduced left ventricular (LV) and right ventricular (RV) function has been identified as a predictor of outcomes. However, LV ejection fraction is often preserved, and RV function is difficult to assess.

Acute and midterm outcomes of the post-approval MELODY Registry: a multicentre registry of transcatheter pulmonary valve implantation.

Aims: The post-approval MELODY Registry aimed to obtain multicentre registry data after transcatheter pulmonary valve implantation (TPVI) with the Melody™ valve (Medtronic plc.) in a large-scale cohort of patients with congenital heart disease (CHD).

Methods And Results: Retrospective analysis of multicentre registry data after TPVI with the Melody™ valve.

Echocardiographic parameters of severe pulmonary regurgitation after surgical repair of tetralogy of Fallot.

Aims: Reliable evaluation of the severity and consequences of pulmonary regurgitation (PR) in patients with repaired tetralogy of Fallot (TOF) is crucial to timely identify the need for pulmonary valve intervention. We aimed to identify the accuracy of echocardiographic parameters to differentiate between moderate and severe PR, using phase contrast cardiac magnetic resonance imaging (CMR) as gold standard.

Methods And Results: In this cross-sectional study, 45 TOF patients with both echocardiographic and CMR measurements of PR were enrolled.

Risk Factors for Pulmonary Hypertension in Adults After Atrial Septal Defect Closure.

Atrial septal defect (ASD) closure is performed to prevent pulmonary hypertension (PH), which is associated with poor outcome. This study investigated the prevalence of PH in adults before and after ASD closure and explored associations between patient characteristics and PH after ASD closure. Consecutive adult patients who underwent surgical or percutaneous ASD closure in the Erasmus MC, the Netherlands, were included (2000 to 2014).

Prognostic value of soluble ST2 in adults with congenital heart disease.

Objective: Soluble suppression of tumourigenicity-2 (sST2) is upregulated as response to myocardial stress and may be a potential biomarker for risk stratification in patients with adult congenital heart disease (ACHD). This study aimed to investigate the release of sST2 and its association with cardiovascular events in ACHD.

Methods: In this prospective cohort study, 602 consecutive patients with ACHD visiting the outpatient clinic were included (2011-2013).

Cardiac CT to assess the risk of coronary compression in patients evaluated for percutaneous pulmonary valve implantation.

Background: Coronary compression (CC) is a life threatening complication that can occur during percutaneous pulmonary valve implantation (PPVI). We describe our experience using cardiac CT prior to PPVI to identify patients at high CC-risk due to a close relationship between the coronary arteries and pulmonary trunk (PT).

Methods: A retrospective evaluation of candidates for PPVI who underwent CT prior to the procedure was done.

Multicenter experience with endovascular treatment of aortic coarctation in adults.

Objective: The objective of this study was to evaluate outcomes of endovascular treatment of aortic coarctation in adults.

Methods: Clinical data and imaging studies of 93 consecutive patients treated at nine institutions from 1999 to 2015 were reviewed. We included newly diagnosed aortic coarctation (NCO), recurrent coarctation, and aneurysmal/pseudoaneurysmal degeneration (ANE) after prior open surgical repair (OSR) of coarctation.

Late complications of an atrial septal occluder provoked by anticoagulant therapy.

Late complications of an atrial septal occluder device (ASO) are rare but may be serious. We report a case with extensive hemopericardium five years after ASO implantation most likely triggered by anticoagulant therapy. Although not surgically confirmed, indirect clues for erosion of the atrial wall by the device were the exclusion of other etiologies, lack of recurrence after pericardial drainage and withdrawal of anticoagulants.

Development and validation of a risk prediction model in patients with adult congenital heart disease.

Aims: To develop and validate a clinically useful risk prediction tool for patients with adult congenital heart disease (ACHD).

Methods And Results: A risk model was developed in a prospective cohort of 602 patients with moderate/complex ACHD who routinely visited the outpatient clinic of a tertiary care centre in the Netherlands (2011-2013). This model was externally validated in a retrospective cohort of 402 ACHD patients (Czech Republic, 2004-2013).

Computed tomography image quality of aortic stents in patients with aortic coarctation: a multicentre evaluation.

Background: Stents are commonly used to treat aortic coarctation. The objective of this study was to evaluate the post-implantation computed tomography (CT) image quality of different stent types used to treat aortic coarctation.

Methods: Adult and paediatric patients with stent-treated aortic coarctation who underwent contrast-enhanced CT were retrospectively included from three tertiary care centres.

Prognostic Value of Serial N-Terminal Pro-B-Type Natriuretic Peptide Measurements in Adults With Congenital Heart Disease.

Background: A single NT-proBNP (N-terminal pro-B-type natriuretic peptide) measurement is a strong prognostic factor in adult congenital heart disease. This study investigates NT-proBNP profiles within patients with adult congenital heart disease and relates these to cardiovascular events.

Methods And Results: In this prospective cohort, 602 patients with adult congenital heart disease were enrolled at the outpatient clinic (years 2011-2013).

Red cell distribution width in adults with congenital heart disease: A worldwide available and low-cost predictor of cardiovascular events.

Background: Red cell distribution width (RDW) is a standard component of the automated blood count, and is of prognostic value in heart failure and coronary heart disease. We investigated the association between RDW and cardiovascular events in patients with adult congenital heart disease (ACHD).

Methods And Results: In this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013.

Complications After Stent Placement for Aortic Coarctation: A Pictorial Essay of Computed Tomographic Angiography.

Stent placement is commonly used to treat aortic coarctation. Although invasive angiography remains the gold standard, follow-up is often performed using computed tomography, which allows rapid, noninvasive assessment of the aorta and surrounding tissues. The goal of this pictorial essay is to provide a guide to the interpretation of these examinations.

Partial anomalous pulmonary venous return in Turner syndrome.

Purpose: The aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome.

Methods And Results: All Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described.

Prognostic value of galectin-3 in adults with congenital heart disease.

Objective: Galectin-3 is an emerging biomarker for risk stratification in patients with heart failure. This study aims to investigate the release of galectin-3 and its association with cardiovascular events in patients with adult congenital heart disease (ACHD).

Methods: In this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013.

Midterm performance of a novel restorative pulmonary valved conduit: preclinical results.

Aims: The Xeltis bioabsorbable pulmonary valved conduit (XPV), designed to guide functional restoration of patients' own tissue, is potentially more durable than current pulmonary bioprosthetic valves/valved conduits. The aim of this study was to assess the haemodynamic performance of the novel XPV implanted in an ovine model.

Methods And Results: The XPV was surgically implanted in adult sheep under general anaesthesia and cardiopulmonary bypass (XPV group, n=20).

Pulmonary arterial stiffness indices assessed by intravascular ultrasound in children with early pulmonary vascular disease: prediction of advanced disease and mortality during 20-year follow-up.

Aims: Prognosis in children with pulmonary vascular disease (PVD) is closely linked to right ventricular (RV) failure due to increased RV-afterload. Pulmonary arterial (PA) stiffening is known to occur early in the course of PVD and constitutes a main component of RV-afterload. This study aimed to evaluate the clinical value of PA-stiffness in children with PVD by determining its association with advanced pulmonary arterial hypertension (PAH) and mortality at long-term follow-up.

Prognostic value of left atrial size and function in adults with tetralogy of Fallot.

Background: Left atrial (LA) size predicts cardiovascular outcome in chronic heart failure. Its prognostic value in adults with repaired tetralogy of Fallot (ToF) is unknown. This study therefore investigated the association of LA size and function with cardiovascular events in adults with ToF.

Five-year results from a prospective multicentre study of percutaneous pulmonary valve implantation demonstrate sustained removal of significant pulmonary regurgitation, improved right ventricular outflow tract obstruction and improved quality of life.

Aims: Percutaneous pulmonary valve implantation (PPVI) is used to treat patients with dysfunctional pulmonary valve conduits. Short- and longer-term results from multiple trials have outlined haemodynamic improvements. Our aim was to report the long-term results, including quality of life, from a multicentre trial in Europe and Canada.