Percutaneous pulmonary valve implantation guided by three-dimensional rotational angiography.

Objectives: To describe the workflow and value of three-dimensional rotational angiography (3DRA) in percutaneous pulmonary valve implantation (PPVI).

Background: 3DRA offers visualization of the entire topography in the chest and may enhance safety and reduce the risk for complications in PPVI through improved pre-procedural planning and per-procedural guidance.

Methods: All PPVI procedures with the use of 3DRA performed between August 2011 and December 2022 were reviewed.

Prognostic significance of echocardiographic deformation imaging in adult congenital heart disease.

Background: Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes.

Fifth decennium after the arterial switch operation for transposition of the great arteries.

Background: From 1977 onwards, patients with both simple and complex transposition of the great arteries (TGA) have been treated with the arterial switch operation (ASO) in the Wilhelmina Children's Hospital/University Medical Center Utrecht the Netherlands. In this study, we compared mortality and morbidity between two patient groups: A. operated before and B.

Strategies for low-molecular-weight heparin management in pregnant women with mechanical prosthetic heart valves: A nationwide survey of Dutch practice.

Background: In this study we investigated current Dutch practice of low molecular weight heparin (LMWH) treatment in pregnant women with mechanical prosthetic heart valves (MPHV) in order to evaluate how management can be optimized.

Methods: Between December 2020 and February 2021, we conducted a survey among Dutch congenital cardiologists of tertiary centers in the Netherlands. We collected and analyzed written, unstructured, open questionnaires that were send to all 8 specialized pregnancy heart teams.

Right atrial and ventricular strain detects subclinical changes in right ventricular function in precapillary pulmonary hypertension.

Right ventricular (RV) ejection fraction (EF) by cardiac magnetic resonance (CMR) correlates to outcome in precapillary pulmonary hypertension (pPH) patients, but is insensitive to early changes. Strain might provide incremental information. In this study, we compare right atrial (RA) and RV strain in pPH patients to healthy controls, and evaluate the prognostic value of strain in pPH.

Common Genetic Variants Contribute to Risk of Transposition of the Great Arteries.

Rationale: Dextro-transposition of the great arteries (D-TGA) is a severe congenital heart defect which affects approximately 1 in 4,000 live births. While there are several reports of D-TGA patients with rare variants in individual genes, the majority of D-TGA cases remain genetically elusive. Familial recurrence patterns and the observation that most cases with D-TGA are sporadic suggest a polygenic inheritance for the disorder, yet this remains unexplored.

Baffle Complications in Adults After Atrial Switch for Transposition of the Great Arteries.

Background: Baffle complications, ie, leakage or stenosis, after an atrial switch operation (AtrSO) for transposition of the great arteries (TGA) are difficult to detect with the use of routine transthoracic echocardiography (TTE). We examined baffle interventions and the prevalence of baffle complications.

Methods: This dual-centre study followed TGA-AtrSO patients for the occurrence of baffle interventions.

Two decades of aortic coarctation treatment in children; evaluating techniques.

Objective: This study focuses on the evolution of treatment techniques for aortic coarctation in children and assesses long-term morbidity.

Methods: This retrospective cohort study evaluates patients treated for native aortic coarctation, with at least 7 years of follow-up. To assess time-related changes, three time periods were distinguished according to year of primary intervention (era 1, 2 and 3).

Medication in adults after atrial switch for transposition of the great arteries: clinical practice and recommendations.

Aims: Heart failure is the main threat to long-term health in adults with transposition of the great arteries (TGA) corrected by an atrial switch operation (AtrSO). Current guidelines refrain from recommending heart failure medication in TGA-AtrSO, as there is insufficient data to support the hypothesis that it is beneficial. Medication is therefore prescribed based on personal judgements.

Type D Personality Associated With Increased Risk for Mortality in Adults With Congenital Heart Disease.

Background: Type D personality has been previously shown to increase the risk for mortality in patients with acquired heart disease.

Objective: We aimed to compare mortality in adult patients with congenital heart disease (CHD) with and without type D.

Methods: Survival was assessed using prospective data from the Dutch national Congenital Corvitia registry for adults with CHD.

New developments in adult congenital heart disease.

Congenital heart disease (CHD) affects 0.8% of live births and over the past decades technical improvements and large-scale repair has led to increased survival into adulthood of over 95% of the new-born. A new group of patients, those who survived their congenital heart defect, has emerged but late complications including heart failure, pulmonary hypertension (PH), arrhythmias, aneurysms and endocarditis appeared numerous, with a huge impact on mortality and morbidity.

Reduced right ventricular function on cardiovascular magnetic resonance imaging is associated with uteroplacental impairment in tetralogy of Fallot.

Background: Maternal right ventricular (RV) dysfunction (measured by echocardiography) is associated with impaired uteroplacental circulation, however echocardiography has important limitations in the assessment of RV function. We therefore aimed to investigate the association of pre-pregnancy RV and left ventricular (LV) function measured by cardiovascular magnetic resonance with uteroplacental Doppler flow parameters in pregnant women with repaired Tetralogy of Fallot (ToF).

Methods: Women with repaired ToF were examined, who had been enrolled in a prospective multicenter study of pregnant women with congenital heart disease.

Infectious stentitis after treatment of coarctation of the aorta: a case report.

Background: Aortitis is a rare condition that can be caused by inflammatory or infectious aetiologies. The clinical presentation of aortitis includes a heterogeneous range of symptoms and clinical signs.

Case Summary: We present a 53-year-old man whose medical history included presence of a ventricular septal defect, a bicuspid aortic valve, and coarctation of the aorta.

Echocardiography and MRI parameters associated with exercise capacity in patients after the arterial switch operation.

Background: The arterial switch operation (ASO) for transposition of the great arteries has excellent survival, but a substantial number of patients suffer from a reduced exercise capacity. The goal of this study was to identify imaging parameters associated with a reduced exercise capacity in patients after ASO.

Methods: A retrospective analysis was performed of ASO patients who underwent cardiopulmonary exercise testing (CPET) between 2007 and 2017.

Doppler gradients, valve area and ventricular function in pregnant women with aortic or pulmonary valve disease: Left versus right.

Objective: Little is known about the course of echocardiographic parameters used for the evaluation of valvular heart disease (VHD) during pregnancy, hampering interpretation of possible changes (physiological vs. pathophysiological). Therefore we studied the course of these parameters and ventricular function in pregnant women with aortic and pulmonary VHD.

Safety and efficacy of stenting for aortic arch hypoplasia in patients with coarctation of the aorta.

Background: Despite a successful repair procedure for coarctation of the aorta (CoA), up to two-thirds of patients remain hypertensive. CoA is often seen in combination with abnormal aortic arch anatomy and morphology. This might be a substrate for persistent hypertension.

Risk of coronary artery disease in adults with congenital heart disease: A comparison with the general population.

Background: Coronary artery disease (CAD) will increasingly determine outcome in the aging adult congenital heart disease (CHD) population. We aimed to determine sex-specific incidence of CAD in adult CHD patients throughout adulthood, compared to the general population.

Methods And Results: We followed 11,723 adult CHD patients (median age 33 years; 49% male; 57% mild, 34% moderate, 9% severe CHD) from the Dutch CONCOR registry, and two age-sex-matched persons per patient from the general population for first CAD event in national registers (period 2002-2012).

Exercise Capacity in Asymptomatic Adult Patients Treated for Coarctation of the Aorta.

A reduced exercise capacity is a common finding in adult congenital heart disease and is associated with cardiovascular morbidity and mortality. However, data on exercise capacity in patients after repair of coarctation of the aorta (CoA) are scarce. Furthermore, a high rate of exercise-induced hypertension has been described in CoA patients.

Medium-term systemic blood pressure after stenting of aortic coarctation: a systematic review and meta-analysis.

Objective: Long-term prognosis of patients with coarctation of the aorta (CoA) is impaired due to the high prevalence of hypertension and consequent cardiovascular complications. Although stent implantation results in acute anatomical and haemodynamic benefit, limited evidence exists regarding the late clinical outcome. In this meta-analysis, we aimed to evaluate the medium-term effect of stent placement for CoA on systemic blood pressure (BP).

Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study.

Background: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD).

Methods: An international multicenter prospective study of NOACs in ACHD was established.

The Natural and Unnatural History of Congenital Aortic Arch Abnormalities Evaluated in an Adult Survival Cohort.

Background: This study describes the different types of congenital vascular rings according to their anatomy, symptoms, and age at clinical onset and reports the surgical outcomes.

Methods: A retrospective observational database study was conducted, reviewing the medical charts of 69 adult survivors with a history of a vascular ring, identified from the Dutch Congenital Cor vitia database.

Results: Median age at presentation was 8.

Quality of Life Among Patients With Congenital Heart Disease After Valve Replacement.

Most studies concerning valve replacement in congenital heart disease (CHD) focus on surgical morbidity and mortality. However, with the increased life expectancy of these patients, the focus shifts to quality of life (QOL). The aim of this study was to report and compare the QOL of CHD patients after valve replacement with the general population and to find factors associated with QOL.