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Irrespective of initial treatment for congenital heart disease (CHD) in childhood, CHD is a lifelong condition, leaving patients at risk for complications. To support uninterrupted, age- and development-based care for young persons with CHD, guidelines and consensus papers emphasise the need for formal transition programmes, including transfer to adult CHD (ACHD) clinics. Here, we surveyed existing transfer and transition programmes in European ACHD centres. Our aims were to provide a contemporary view of transitional care for patients with CHD and to evaluate progress over the last decade. We conducted a descriptive, cross-sectional survey in 96 ACHD centres in Europe. A specific survey form was developed that sampled the practices of transfer and/or transition. We used a transfer-transition index to quantify adherence to quality indicators of successful transfer and transition. Of the 96 ACHD centres, 40 (41.7%) offered a formal transition, and 85 (88.5%) had structured transfer from paediatric to ACHD care. Although 31% of the centres performed at a 'good' level on the transfer-transition index, only 4 (4.2%) satisfied all criteria. Most centres with a transition programme offered education and support through a dedicated transition specialist, who was a master's-prepared nurse in most centres. A minority of the ACHD centres offered a flexible transition process, starting at least two years before transfer. Nearly half of the included ACHD centres offered a formal transition programme, and almost 90% offered structured transfer. Despite some improvements since 2009, most of the programmes lacked an age- and development-based approach. • The majority of CHD centres in Europe offer a structured transfer from paediatric to adult care. • Although the number of centres with formal transition increased over time, time, only a minority follows the recommendations to the fullest maximum. • All transition programmes included a dedicated transition specialist; the role is often taken up by nurses. [ABSTRACT FROM AUTHOR]

Background: Quality indicators are crucial in evaluating and comparing the quality of healthcare services. In the case of congenital heart disease, transition programmes for adolescents have been recommended to ensure uninterrupted healthcare and lifelong care. It is necessary to establish quality indicators in order to facilitate the evaluation of programme quality and to allow comparison between different centres. The objective of this study is therefore to develop a set of quality indicators for the transition of adolescents with congenital heart disease. Methods: The RAND/UCLA appropriateness method was employed in a four-step process to develop a set of quality indicators. First, a literature search was conducted on the dimensions of transitional care, based on which a preliminary set of quality indicators was developed. Second, experts were contacted, and an expert panel was established. Third, the panel members were asked to rate the appropriateness of the quality indicators in a two-round process. Finally, in the fourth step, we evaluated the data by measuring the median and Disagreement Index. Results: The expert panel consisted of 16 members, congenital cardiologists, nurses, transition experts, patients and research experts. The preliminary set of quality indicators comprised 16 items, categorized in process and structure criteria. Based on the panel's feedback, the set was refined to 12 quality indicators, which were rated as relevant and feasible. Conclusions: This study represents the first attempt to develop quality indicators for transitional care services for adolescents with congenital heart disease. The set of 12 quality indicators was developed based on existing evidence and expert opinion. Further testing is needed to assess the feasibility of these quality indicators in daily practice. If successfully implemented, these quality indicators could allow comparison and facilitate benchmarking of transitional care services for adolescents with congenital heart disease. [ABSTRACT FROM AUTHOR]

More than 90% of children born with heart defects reach adulthood. They continue to require specialized medical care. In most countries, their care has to be transferred from the pediatric care environment to specialized adult clinics. This transfer of care usually occurs at a time when adolescents become young adults. Supporting adolescents and emerging adults with congenital heart disease through transition has been recognized as an important task of their treating teams in recent years. An environment where adolescents feel welcome and where education and patient participation are fostered is crucial. For an optimal transition process, patients, their families and all health care providers need to be involved. Different models for transition programs have emerged, depending on local policies and resources. The authors offer insight into established transition programs in Bern and Zurich, Switzerland. Advantages and challenges of different models of care and transition programs are presented. [ABSTRACT FROM AUTHOR]

The aim of this study was to test the hypothesis that ear oximetry immediately after the release of a sustained Valsalva maneuver accurately detects patent foramen ovale (PFO). One hundred sixty-five scuba divers underwent transesophageal echocardiography (TEE; reference method) for PFO assessment. Ear oximetry of the right earlobe was performed in a different room within a time frame of 2 hours before or after TEE. The subject and the oximetry operator were unaware of the results of TEE. Oxygen saturation (SO2) measurements were obtained at baseline and during the release phase of 4 Valsalva maneuvers within 10 minutes, and the average SO2 change (SO2 at baseline minus SO2 at Valsalva release) was determined as the primary study end point. One hundred seventeen divers had no PFO, and 48 (29%) had PFO by TEE (mean age 39 ± 8 years). The average SO2 change was 0.79 ± 1.13% (i.e., a slight absolute SO2 decrease in response to the Valsalva maneuver) in the group without PFO and 1.67 ± 1.19% in the PFO group (p <0.0001). Using receiver-operating characteristic curve analysis, a PFO as defined by TEE could be detected at a threshold of a Valsalva-induced decrease in SO2 of ≥0.825 percentage points in comparison to baseline (sensitivity 0.756, specificity 0.706, area under the receiveroperating characteristic curve 0.763, p <0.0001, negative predictive value 0.882). In conclusion, the entirely noninvasive method of ear oximetry in response to repetitive Valsalva maneuvers is accurate and useful as a screening method for the detection of a PFO, as shown in this study of divers. [ABSTRACT FROM AUTHOR]

Abstract: The steadily growing number of adults with congenital heart disease has led with some delay to an increase of specialized programs dedicated to the care of these patients. Still, in many countries the prevalence of congenital heart disease in adults is not known, nor the number of adults requiring specialized care. By extrapolating the results of epidemiological studies on the population in Europe and North America, only half or even less of all adults with moderate or complex congenital heart disease is currently under follow-up in specialized centers. Lapse of appropriate medical care in adults with congenital heart disease is associated with unnecessary morbidity and an increased likelihood of the need for urgent interventions. In order to improve medical care for adults with moderate or complex congenital heart disease, the number of patients with specialized follow-up in congenital cardiac clinics has to increase. One approach is to avoid lapses of care in patients already followed by a pediatric or adult congenital heart disease center. Therefore, congenital heart disease clinics should try to identify among their patients the ones at risk for lost to follow-up, and focus some of their efforts on measures to increase the awareness for life-long medical care among these patients. Characteristics of the adult congenital cardiac patient at high risk of inappropriate medical follow-up include having few interventions in early life despite a complex cardiac defect, being born in an earlier decade, receiving no specific follow-up recommendations, and having no education about defect- or surgery-related sequelae or residual lesions. Implementation of a structured transition program, teaching patients about their heart defect and the consequences of repair or palliation in earlier life, establishing a continuity of care within the congenital cardiac clinic, optimizing organization and communication between different care-givers, and encouraging patients to play an active role regarding their personal health care, are some of the recommendations on how we should try to avoid another generation of adult congenital heart disease patients from being lost to follow-up. [Copyright &y& Elsevier]

Objectives: Recurrent embolic events after device closure of patent foramen ovale (PFO) have been related to incomplete closure. Another cause could be atrial fibrillation (AF). The aim of this study was to determine the incidence of AF in stroke patients after PFO closure. Methods: Consecutive patients with device closure of a PFO after a stroke or transient ischemic attack and control patients with stroke underwent 7-day event loop recordings 3 and 6 months after PFO closure or stroke, respectively. Results: Forty patients treated by PFO device closure 96 ± 68 days after cryptogenic ischemic stroke and 70 control patients with ischemic stroke of other etiologies (known AF excluded) were compared. AF was identified in 6 patients (15%) of the treated group and in 12 control patients (17%, p = 0.77). In multivariate analysis, the presence of an occluder device was not an independent risk factor for AF. Conclusions: The incidence of AF is high after device closure of a PFO in stroke patients and similar to that in patients with stroke of non-PFO etiology and, hence, with no device. Further studies are required to determine the risk of thromboembolism and the optimal treatment in patients developing AF after device closure of a PFO. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Adults with repaired tetralogy of Fallot and significant chronic pulmonary regurgitation are at risk for progressive right ventricular (RV) dilatation and dysfunction. The assessment of RV function is important in the management in these patients. There is still a lack of an adequate geometric model to quantify RV function by echocardiography. The myocardial performance index (MPI) is a nonvolumetric method to quantify global ventricular function. In this study, the accuracy of MPI obtained by echocardiography to quantify RV function was assessed in 57 adults with repaired tetralogy of Fallot. The MPI measurement was compared with the RV ejection fraction (EF) derived by cardiac magnetic resonance imaging. There was a negative linear correlation between the MPI and the RVEF (r = 0.73, p <0.001). A MPI cutoff of ≥0.40 had a sensitivity of 81% and a specificity of 85% to diagnose a RVEF <35%. A MPI cutoff of <0.25 had a sensitivity of 70% and a specificity of 89% to identify patients with RVEFs ≥0.50. In a multivariate regression model, the MPI was not affected by the degree of pulmonary regurgitation, the presence of tricuspid regurgitation, or the QRS duration. In conclusion, the Doppler-derived MPI is a simple and reliable method for the evaluation of RV systolic function in adults with repaired tetralogy of Fallot. [Copyright &y& Elsevier]

The article discusses the patent foramen ovale (PFO) in patients with stroke. The PFO is said to be a classic example for presumption of guilt in the world of medicine. Such interatrial communication is first indicted by J. Cohnheim in 1877 to be responsible for a paradoxical embolism (PaE) caused by a right-to-left shunt in a young adult with stroke. The PFO is found to have higher prevalence in patients with cryptogenic stroke compared with healthy controls.

Abstract: The presence of irreversible pulmonary hypertension in patients with atrial septal defect (ASD) is thought to preclude shunt closure. We report the case of a woman with plexiform pulmonary arteriopathy secondary to an ostium secundum ASD who was able to successfully undergo percutaneous shunt closure following therapy with chronic intravenous prostacyclin (Flolan). One year after closure, the patient was weaned off Flolan over a period of 7 months following the institution of oral Bosentan therapy. Our case illustrates how aggressive vasodilator therapy with prostaglandins may be capable of reducing pulmonary artery pressure and permitting shunt closure in a patient once considered to have “inoperable” pulmonary arteriopathy. [Copyright &y& Elsevier]

Assesses the prevalence of symptoms of decompression illness and ischemic brain lesions in Swedish divers with regard to the presence of a patent foramen ovale. Risk for decompression illness in divers with patent foramen ovale versus divers without patent foramen ovale; Frequency of ischemic brain lesions per asymptomatic divers versus nondivers.

Objective: Self-efficacy is a known predictor of patient-reported outcomes in individuals with acquired diseases. With an overall objective of better understanding patient-reported outcomes in adults with congenital heart disease, this study aimed to: (i) assess self-efficacy in adults with congenital heart disease, (ii) explore potential demographic and medical correlates of self-efficacy and (iii) determine whether self-efficacy explains additional variance in patient-reported outcomes above and beyond known predictors. Methods: As part of a large cross-sectional international multi-site study (APPROACH-IS), we enrolled 454 adults (median age 32 years, range: 18–81) with congenital heart disease in two tertiary care centres in Canada and Switzerland. Self-efficacy was measured using the General Self-Efficacy (GSE) scale, which produces a total score ranging from 10 to 40. Variance in the following patient-reported outcomes was assessed: perceived health status, psychological functioning, health behaviours and quality of life. Hierarchical multivariable linear regression analysis was performed. Results: Patients’ mean GSE score was 30.1 ± 3.3 (range: 10–40). Lower GSE was associated with female sex (p = 0.025), not having a job (p = 0.001) and poorer functional class (p = 0.048). GSE positively predicted health status and quality of life, and negatively predicted symptoms of anxiety and depression, with an additional explained variance up to 13.6%. No associations between self-efficacy and health behaviours were found. Conclusions: GSE adds considerably to our understanding of patient-reported outcomes in adults with congenital heart disease. Given that self-efficacy is a modifiable psychosocial factor, it may be an important focus for interventions targeting congenital heart disease patients’ well-being. [ABSTRACT FROM AUTHOR]

In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions. We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention. TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention. The following outcome parameters were included: predicted (%) peak oxygen uptake (peak VO2), tricuspid annular plane systolic excursion (TAPSE), tricuspid Lateral Annular Systolic Velocity (TV S′), right ventricle (RV)-arterial coupling (defined as TAPSE/RV systolic pressure ratio), and N-terminal proBNP (NT-proBNP). 447 TGA patients with a mean age of 25.0 (interquartile range (IQR) 21–29) years were included. Patients without previous RVOT intervention (n = 338, 76%) had a significantly higher predicted peak VO2 (78.0 ± 17.4%) compared to patients with single approach catheter-based RVOT intervention (73.7 ± 12.7%), single approach surgical RVOT intervention (73.8 ± 28.1%), and patients with multiple approach RVOT intervention (66.2 ± 14.0%, p = 0.021). RV-arterial coupling was found to be significantly lower in patients with prior catheter-based and/or surgical RVOT intervention compared to patients without any RVOT intervention (p = 0.029). TGA patients after a successful arterial switch repair have a decreased exercise capacity. A considerable amount of TGA patients with either catheter or surgical RVOT intervention perform significantly worse compared to patients without RVOT interventions. • TGA patients after a successful arterial switch repair have a decreased exercise capacity. • Lower excercise capacity was seen in ASO patients after RVOT intervention compared to patients without RVOT intervention. • RV-arterial coupling emerged as the most sensitive hemodynamic parameter for RV performance after RVOT intervention. [ABSTRACT FROM AUTHOR]

Abstract: Altered myocardial perfusion is frequent in patients with congenital heart disease. Measurement of myocardial blood flow per gram of myocardium at rest and during hyperemia is the most robust method to evaluate myocardial perfusion in absolute terms. So far, most studies in congenital heart disease patients used positron emission tomography to evaluate absolute myocardial blood flow. With the recent advances in contrast echocardiography, we have now a more easily applicable but similarly solid method at hand to assess myocardial perfusion in different clinical scenarios. Myocardial contrast echocardiography has proven its clinical and scientific value in coronary artery disease patients. In this review, we discuss the merits of myocardial perfusion assessment in patients with a systemic right ventricle, a univentricular heart physiology, and tetralogy of Fallot. We outline how myocardial contrast echocardiography can improve our understanding of ventricular function. We have to admit that its clinical value remains to be elucidated. For most congenital defects, the impact of impaired myocardial perfusion on ventricular function and clinical outcome is still poorly investigated. Myocardial contrast echocardiography can contribute to fill this gap. [Copyright &y& Elsevier]

Abstract: Background: Systolic right ventricular (RV) function is an important predictor in the course of various congenital and acquired heart diseases. Its practical determination by echocardiography remains challenging. We compared routine assessment of lateral tricuspid annular systolic motion velocity (TVlat, cm/s) using pulsed-wave tissue Doppler imaging from the apical 4-chamber view with cardiac magnetic resonance (CMR) as reference method. Methods and results: 254 individuals (43±18years) underwent both CMR (contiguous short axis slices; retrogated cine steady state free precession technique; manual contour tracing) and echocardiography within 2±2months. Seventy-five had coronary artery disease, 87 congenital heart disease, 17 dilated cardiomyopathy, 15 pulmonary artery hypertension, and 47 normal findings. RV ejection fraction (EF) by CMR was 51±12% (range 17–78%). There was a linear correlation between RVEF and TVlat (r =0.60; p <0.0001). A TVlat cut-off of 12cm/s identified patients with normal EF (≥50%) with 81% sensitivity and 68% specificity, and a threshold of TVlat <9cm/s identified patients with severely reduced RVEF (<30%) with 82% sensitivity and 86% specificity. Conclusions: Systolic long-axis velocity measurements of the lateral tricuspid annulus allow a reliable assessment of RVEF in clinical routine. A threshold of TVlat <9cm/s identifies patients with severely reduced RVEF (<30%) with high sensitivity and specificity. [Copyright &y& Elsevier]

Objective A consequence in patients with d-transposition of the great arteries (d-TGA) and tetralogy of Fallot (TOF) is right ventricular hypertrophy (RVH) and right ventricular failure. Myocardial contrast echocardiography (MCE) permits the determination of the myocardial microvascular density reflected by the relative myocardial blood volume (rBV; ml/ml). This study was conducted to elucidate the relationship between RVH and myocardial microvascular changes by quantitative MCE in patients with d-TGA and TOF. Methods Three groups of individuals were included in the study: 22 patients with d-TGA, 18 patients with TOF and 22 healthy individuals (controls). MCE was performed at rest and during adenosine-induced hyperaemia. rBV and myocardial blood flow (MBF; ml/min per gram) were derived from steady state and refill sequences of ultrasound contrast agent. Results Hyperaemic septal rBV differed significantly between the groups and was highest in controls: d-TGA 0.141±0.060 ml/ml, TOF 0.134±0.080 ml/ml, controls 0.189±0.074 ml/ml, p=0.005. Myocardial blood flow reserve (MBFR), that is the ratio of hyperaemic to baseline MBF, differed significantly between the groups and was lowest in d-TGA (2.68±1.13) versus TOF (3.37±1.57) and controls (4.22±1.17, p=0.001). Hyperaemic septal rBV, MBF and MBFR showed a significant correlation with right ventricular systolic function as determined by tricuspid annular plane systolic excursion. Conclusions Right ventricular myocardial microvascular density of the septal wall in d-TGA and TOF patients with RVH due to pressure and/or volume overload is reduced. This appears to be related to a reduced myocardial perfusion reserve and impaired right ventricular systolic function. [ABSTRACT FROM AUTHOR]

Abstract: Aims: The goal of this study was to assess the prevalence of left ventricular (LV) hypertrophy in patients with aortic stenosis late (>6 months) after aortic valve replacement and its impact on cardiac-related morbidity and mortality. Methods and results: In a single tertiary centre, echocardiographic data of LV muscle mass were collected. Detailed information of medical history and angiographic data were gathered. Ninety-nine of 213 patients (46%) had LV hypertrophy late (mean 5.8±5.4 years) after aortic valve replacement. LV hypertrophy was associated with impaired exercise capacity, higher New York Heart Association dyspnoea class, a tendency for more frequent chest pain expressed as higher Canadian Cardiovascular Society class, and more rehospitalizations. 24% of patients with normal LV mass vs. 39% of patients with LV hypertrophy reported cardiac-related morbidity (p =0.04). In a multivariate logistic regression model, LV hypertrophy was an independent predictor of cardiac-related morbidity (odds ratio 2.31, 95% CI 1.08 to 5.41), after correction for gender, baseline ejection fraction, and coronary artery disease and its risk factors. Thirty seven deaths occurred during a total of 1959 patient years of follow-up (mean follow-up 9.6 years). Age at aortic valve replacement (hazard ratio 1.85, 95% CI 1.39 to 2.47, for every 5 years increase in age), coexisting coronary artery disease at the time of surgery (hazard ratio 3.36, 95% CI 1.31 to 8.62), and smoking (hazard ratio 4.82, 95% CI 1.72 to 13.45) were independent predictors of overall mortality late after surgery, but not LV hypertrophy. Conclusions: In patients with aortic valve replacement for isolated aortic stenosis, LV hypertrophy late after surgery is associated with increased morbidity. [Copyright &y& Elsevier]

Objectives: We set out to study the effect of transcatheter closure of atrial septal defect (ASD) on right ventricular (RV) and left ventricular (LV) function assessed by myocardial performance index (MPI), as well as left atrial (LA) volumes. Background: The hemodynamic response to the closure of ASD is well-documented in surgically treated patients. However, few studies have documented echocardiographic evaluation of ventricular function in patients undergoing transcatheter closure of ASDs. Methods: Pre- and post-ASD device closure echocardiograms of 25 consecutive patients were retrospectively reviewed. Measurements of RV and LV MPI and LA volumes were made. Results: Twenty-five patients with an average age of 45.5 ± 16.3 years underwent transcatheter closure of ASD. There was statistically significant improvement in RV MPI (0.35 to 0.28, p = 0.004), LV MPI (0.37 to 0.31, p = 0.04), and LA volume index (25.7 to 21.8 ml/m2, p < 0.001) after closure of ASD. Conclusions: Device closure of ASDs leads to improvement of both RV and LV function as well as reduction in LA volume. These hemodynamic improvements provide insights into the symptomatic benefits gained in closure of ASDs using the transcatheter approach. [Copyright &y& Elsevier]

Pulmonary hypertension (PH), especially pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH/CTEPH), are rare and progressive conditions. Despite recent advances in treatment and prognosis, PH is still associated with impaired quality of life and survival. Long‐term PH‐registry data provide information on the changing PH‐epidemiology and may help to direct resources to patient's needs. This retrospective analysis of the Swiss Pulmonary Hypertension Registry includes patients newly diagnosed with PH (mainly PAH/CTEPH) registered from January 2001 to June 2019 at 13 Swiss hospitals. Patient characteristics (age, body mass index, gender, diagnosis), hemodynamics at baseline, treatment, days of follow‐up, and events (death, transplantation, pulmonary endarterectomy, or loss to follow‐up) at last visit were analyzed. Patients were stratified into four time periods according to their date of diagnosis. Survival was analyzed overall and separately for PAH/CTEPH and time periods. 1427 PH patients were included (thereof 560 PAH, 383 CTEPH). Over the years, age at baseline (mean ± SD) significantly increased from 59 ± 14 years in 2001–2005 to 66 ± 14 years in 2016–2019 (p < 0.001) while the gender distribution tended toward equality. Mean pulmonary artery pressure and pulmonary vascular resistance significantly decreased over time (from 46 ± 15 to 41 ± 11 mmHg, respectively, 9 ± 5 to 7 ± 4 WU, p < 0.001). Three‐year survival substantially increased over consecutive periods from 69% to 91% (for PAH 63%–95%, for CTEPH 86%–93%) and was poorer in PAH than CTEPH independently of time period (p < 0.001). Most patients were treated with mono‐ or combination therapy and an increasing number of CTEPH underwent pulmonary endarterectomy (40% 2016–2019 vs. 15% 2001–2005). This long‐term PH registry reveals that over two decades of observation, newly diagnosed patients are older, less predominantly female, have less impaired hemodynamics and a better survival. [ABSTRACT FROM AUTHOR]

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. This is an international multicentre prospective study, using data from the NOTE registry on adults with sRV taking NOACs between 2014 and 2019. The primary endpoints were TE and major bleeding (MB). The secondary endpoint was minor bleeding. A total of 76 patients (42.5 ± 10.0 years, 76% male) with sRV (74% TGA, 26% ccTGA) on NOACs were included in the study. During a median follow-up of 2.5 years (IQR1.5–3.9), TE events occurred in 3 patients (4%), while no MB episodes were reported. Minor bleeding occurred in 9 patients (12%). NOAC treatment cessation rate was 1.4% (95%CI:0.3–4%) during the first year of follow-up. All the patients with TE events had a CHA 2 DS 2 -VASc score ≥ 2 and impaired sRV systolic function at baseline. The total incidence of major events during follow-up was significantly lower compared to historical use of VKAs or aspirin before study inclusion (1.4% (95%CI:0.29–4%) vs 6,9% (95%CI:2.5–15.2%); p =.01). In this prospective study, NOACs appear to be well-tolerated, with excellent efficacy and safety at mid-term in patients with sRV. • NOACs treatment is safe and effective at mid-term in the population with sRV. • Discontinuation rate is low at mid-term. • Events rate on NOACs is lower compared to vitamin K antagonists or aspirin use. • Subjects with impaired sRV function and CHA 2 DS 2 -VASc≥2 may have higher risk of TE. [ABSTRACT FROM AUTHOR]

Clinical guidelines emphasise the need for specialised adult congenital heart disease (ACHD) programmes. In 2014, the working group on Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC) published recommendations on the organisation of specialised care for ACHD. To appraise the extent to which these recommendations were being implemented throughout Europe, we assessed the number of patients in active follow-up and available staff resources in European ACHD programmes. We conducted a descriptive, cross-sectional, paper-based survey of specialised ACHD centres in Europe in late 2017 concerning their centre status in 2016. Data from 96 ACHD centres were analysed. We categorised ACHD programmes into seven different centre types based on their staff resources and composition of interdisciplinary teams. Only four centres fulfilled all medical and non-medical staffing requirements of the ESC recommendations. Although 60% of the centres offered all forms of medical care, they had incomplete non-medical resources (i.e., specialised nurses, social workers, or psychologists). The participating centres had 226,506 ACHD patients in active follow-up, with a median of 1500 patients per centre (IQR: 800-3400). Six per cent of the patients were followed up in a centre that lacked a CHD surgeon or congenital interventional cardiologist. A minority of European ACHD centres have the full recommended staff resources available. This suggests that as of 2016 either ACHD care in Europe was still not optimally organised, or that the latest ESC recommendations were not fully implemented in clinical practice. • Overview of the structure and staffing of ACHD centres in Europe. • Steady increase in the number of centres but not in patient numbers per centre. • High variability within centres between patient load and staff resources. • Poor adherence in staff resources to the current recommendations. [ABSTRACT FROM AUTHOR]

Background In biventricular hearts the filling and contractility of one ventricle affects the performance of the other. In this study, we compared right ventricular systolic function and exercise capacity in patients with a subaortic right ventricle (RV) in relation to the presence of a left ventricular outflow tract obstruction (LVOTO). Methods Retrospective chart review of adults with congenitally corrected transposition of the great arteries (ccTGA) or with a previous atrial switch procedure for complete TGA (D-TGA). A LVOTO was defined by a peak instantaneous systolic gradient > 20 mm Hg. Right and left ventricular ejection fraction (EF) were measured by cardiac magnetic resonance imaging (CMR), and exercise capacity as the predicted peak oxygen consumption (peak VO 2 ) on a cycle ergometer. Results We identified 79 clinically stable adults (age 33 ± 10 years, 70% male). Nine patients (11%) had cc-TGA and 70 patients had (89%) D-TGA. Thirteen patients (16%) had a LVOTO with a mean peak instantaneous systolic gradient of 43 ± 22 mm Hg. Patients with a LVOTO had higher left (68 ± 7% vs. 60 ± 9%, p = 0.01) and right ventricular EF (52 ± 8 vs. 46 ± 9%, p = 0.05) by CMR compared to patients without LVOTO. In a multivariate regression analysis with left ventricular EF and LVOTO as predictors, only left ventricular EF was independently associated with right ventricular EF (correlation coefficient 0.41, p < 0.01). The presence of a LVOTO was not associated with improved exercise capacity. Conclusions In adults with a subaortic RV, a pressure loaded subpulmonary left ventricle has a beneficial effect on systemic right ventricular EF. [ABSTRACT FROM AUTHOR]

Background Patients with a systemic right ventricle (RV) have a compromised late outcome caused by ventricular dysfunction. Standard medical heart failure therapy has not been shown to improve RV function and survival in these patients. Phosphodiesterase (PDE)-5 inhibition increases contractility in experimental models of RV hypertrophy, but not in the normal RV. In clinical practice, the effects of PDE-5 inhibition on systemic RV function and exercise capacity in adults with a systemic RV have not been tested. Methods The SERVE protocol is a double-blind, randomized placebo-controlled multicenter superiority trial to study the effect of PDE-5 inhibition with Tadalafil on RV volumes and function in patients with either D-transposition of the great arteries repaired with an atrial switch procedure or with congenitally corrected transposition of the great arteries. Tadalafil 20 mg or placebo will be given over a study period of 3 years. The primary endpoint is the change in mean end-systolic RV volumes from baseline to study end at 3 years of follow-up (or at the time of permanent discontinuation of the randomized treatment if stopped before 3- years of follow-up), and will be measured by cardiovascular magnetic resonance imaging (CMR) or by cardiac computed tomography in patients with contraindications for CMR. Secondary endpoints are changes in RV ejection fraction, VO2max and NT-proBNP. Conclusion The objective of this study is to assess the effect of PDE-5 inhibition with Tadalafil on RV size and function, exercise capacity and neurohumoral activation in adults with a systemic RV over a 3-year follow-up period. [ABSTRACT FROM AUTHOR]

Purpose Patients with Fontan circulation have reduced heart rate variability (HRV) in supine position. However, neither cardiac autonomic nervous activity (CANA) in response to orthostatic challenge nor vagal reactivation by means of heart rate (HR) recovery after cessation of exercise have previously been investigated in Fontan patients. The aim of this study was to compare HRV in supine and standing position, as well as HR recovery between Fontan patients and healthy controls. Methods Eight Fontan patients (4 male/4 female) without pacemakers and 12 healthy volunteers (5 m/7 f) with minimum age of 18 years were recruited. HR was measured by Holter-electrocardiogram. HRV was measured in supine position and after orthostatic challenge. The power of the high frequency (HF: 0.15 Hz–0.4 Hz) and low frequency (LF: 0.04 Hz–0.15 Hz) bands was analysed by fast-Fourier transformation. HR recovery was determined at 30 s and 60 s after termination of a maximal exercise test. Results In both supine and standing position, total power, HF and LF power were reduced in Fontan patients compared to controls (by approximately a factor of 10) while there was no differences in LF/HF power ratio. Response to orthostatic challenge was blunted in absolute power but normal in relative power. HR recovery was not different between groups. Conclusion Fontan patients have greatly reduced HRV, a blood-pressure dependent marker of CANA, but normal HR recovery, a blood pressure independent marker of vagal reactivation, suggesting that vagal activity may be normal, and only vascular capacitance reduced. [ABSTRACT FROM AUTHOR]

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD. [ABSTRACT FROM AUTHOR]

Background: Registries are important for real-life epidemiology on different pulmonary hypertension (PH) groups. Objective: To provide long-term data of the Swiss PH registry of 1998-2012. Methods: PH patients have been classified into 5 groups and registered upon written informed consent at 5 university and 8 associated hospitals since 1998. New York Heart Association (NYHA) class, 6-min walk distance, hemodynamics and therapy were registered at baseline. Patients were regularly followed, and therapy and events (death, transplantation, endarterectomy or loss to follow-up) registered. The data were stratified according to the time of diagnosis into prevalent before 2000 and incident during 2000-2004, 2005-2008 and 2009-2012. Results: From 996 (53% female) PH patients, 549 had pulmonary arterial hypertension (PAH), 36 PH due to left heart disease, 127 due to lung disease, 249 to chronic thromboembolic PH (CTEPH) and 35 to miscellaneous PH. Age and BMI significantly increased over time, whereas hemodynamic severity decreased. Overall, event-free survival was 84, 72, 64 and 58% for the years 1-4 and similar for time periods since 2000, but better during the more recent periods for PAH and CTEPH. Of all PAH cases, 89% had target medical therapy and 43% combination therapy. Of CTEPH patients, 14 and 2% underwent pulmonary endarterectomy or transplantation, respectively; 87% were treated with PAH target therapy. Conclusion: Since 2000, the incident Swiss PH patients registered were older, hemodynamically better and mostly treated with PAH target therapies. Survival has been better for PAH and CTEPH diagnosed since 2008 compared with earlier diagnosis or other classifications. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background Data on pharmacological management during pregnancy are scarce. The aim of this study was to describe the type and frequency of cardiac medication used in pregnancy in patients with cardiovascular disease and to assess the relationship between medication use and fetal outcome. Methods and results Between 2007 and 2011 sixty hospitals in 28 countries enrolled 1321 pregnant women. All patients had structural heart disease (congenital 66%, valvular 25% or cardiomyopathy 7% or ischemic 2%). Medication was used by 424 patients (32%) at some time during pregnancy: 22% used beta-blockers, 8% antiplatelet agents, 7% diuretics, 2.8% ACE inhibitors and 0.5% statins. Compared to those who did not take medication, patients taking medication were older, more likely to be parous, have valvular heart disease and were less often in sinus rhythm. The odds ratio of fetal adverse events in users versus non-users of medication was 2.6 (95% CI 2.0-3.4) and after adjustment for cardiac and obstetric parameter was 2.0 (95% CI 1.4-2.7). Babies of patients treated with beta-blockers had a significantly lower adjusted birth weight (3140 versus 3240 g, p = 0.002). The highest rate of fetal malformation was found in patients taking ACE inhibitors (8%). Conclusion One third of pregnant women with heart disease used cardiac medication during their pregnancy, which was associated with an increased rate of adverse fetal events. Birth weight was significantly lower in children of patients taking beta-blockers. A randomized trial is needed to distinguish the effects of the medication from the effects of the underlying maternal cardiac condition. [ABSTRACT FROM AUTHOR]

In adults with congenital heart disease and a systemic right ventricle, subaortic ventricular systolic dysfunction is common. Echocardiographic assessment of systolic right ventricular (RV) function in these patients is important but challenging. The aim of the present study was to assess the reliability of conventional echocardiographic RV functional parameters to quantify the systolic performance of a subaortic right ventricle. We compared 56 contemporary echocardiograms and cardiac magnetic resonance studies in 37 adults, aged 26.9 - 7.4 years, with complete transposition and a subaortic right ventricle. The fractional area change (FAC), lateral tricuspid annular plane systolic excursion, lateral RV systolic motion velocities by tissue Doppler, RV myocardial performance index, and the rate of systolic RV pressure increase (dp/dt) measured across the tricuspid regurgitant jet were assessed by echocardiography and correlated with the cardiac magnetic resonance-derived RV ejection fraction (EF). The mean RVEF was 48.0 - 7.8%. FAC (r2 [ 0.206, p [ 0.001) and dp/dt (r2 [ 0.173, p [ 0.009) significantly correlated with RVEF, and the other nongeometric echocardiographic parameters failed to show a significant correlation with RVEF by linear regression analysis. FAC <33% and dp/dt <1,000 mm Hg/s identified a RVEF of <50% with a sensitivity of 77% and 69% and a specificity of 58% and 87%, respectively. In conclusion, in patients with a systemic right ventricle, routine nongeometric echocardiographic parameters of RV function correlated weakly with cardiac magnetic resonance-derived EF. RV FAC and the measurement of the rate of systolic RV pressure increase (dp/dt) should be preferentially used to assess systemic systolic function in adult patients with a subaortic right ventricle. [ABSTRACT FROM AUTHOR]

Structural abnormalities of the medial aorta have been described for conotruncal defects (e.g., tetralogy of Fallot [TOF] and complete transposition of the great arteries (dextrotransposition [d]-TGA). In TOF, progressive aortic dilation is a frequent finding. In patients with d-TGA with an atrial switch, this problem is less often described. The aim of the present study was to compare the extent of dilative aortopathy and aortic distensibility in adults with an atrial switch procedure (n = 39) to that in adults with repaired TOF (n = 39) and controls (n = 39), using cardiac magnetic resonance imaging. The groups were matched for age and gender. Diameters of the aorta indexed to the body surface area were significantly increased in the patients with d-TGA and TOF compared to that of the controls at the aortic sinus up to the level of the right pulmonary artery. On multivariate testing, the diagnosis of a conotruncal defect (β = 0.260; p = 0.003) and aortic regurgitant fraction (β = 0.405; p <0.001) were independent predictors of an increased aortic sinus diameter. Ascending aorta distensibility was significantly reduced in those with d-TGA and TOF compared to controls: 3.6 (interquartile range 1.5 to 4.4) versus 2.8 (interquartile range 2.0 to 3.7) versus 5.5 (interquartile range 4.8 to 6.9) ×10−3 mm Hg−1 (p <0.001). The independent predictors of ascending aorta distensibility were the diagnosis of a conotruncal defect (p <0.001) and age (p = 0.028). In conclusion, intrinsic aortopathy, manifested as increased ascending aortic diameters and reduced ascending aortic distensibility, is not only evident in adults with TOF, but also in adults with d-TGA and an atrial switch procedure. Long-term follow-up is needed to monitor the aortic size in both patient groups. [Copyright &y& Elsevier]

Objective: To test the effect of patent foramen ovale (PFO) closure on neurological events in divers. Design: Prospective, non-randomised, longitudinal threearm study. Setting: Tertiary referral centre. Population: 104 scuba divers with a history of major decompression illness (DCI). Intervention: Transcutaneous PFO closure. Main outcome measures: Baseline and three follow-up examinations with a questionnaire about health status and diving habits/accidents, transoesophageal echocardiography at baseline for PFO grading, cerebral MRI at all examinations. Results: 39 divers had no PFO, 26 had a PFO and chose to undergo percutaneous closure and 39 had a PFO, but decided not to undergo closure. The total number of dives, including those performed before baseline and those during long-term follow-up, was 81 654; 18 394 dives during the follow-up period of 5.3±0.3 years, during which there were a total of five major neurological DCI eventsenamely 0 in the no PFO group, 0.5±2.5/104 dives in the PFO closure group and 35.8±102.5/104 dives in the PFO no closure group (four events; p=0.045 between the PFO groups). In the groups, no PFO, PFO closure and PFO no closure, there were 1.162.6, 0.861.4, 3.3±6.9 ischaemic brain lesions, respectively, at follow-up (p=0.039 between the PFO groups)dthat is, 16±42/104 dives in the no PFO group, 6613/104 dives in the PFO closure group and 1046246/104 dives in the PFO no closure group (overall p=0.042; p=0.024 between the PFO groups). Conclusion: PFO closure in continuing divers appears to prevent symptomatic (major DCI) and asymptomatic (ischaemic brain lesions) neurological events during longterm follow-up. [ABSTRACT FROM AUTHOR]

Objectives Patent foramen ovale (PFO) has been linked to migraine, and an improvement in migraine prevalence or frequency has been reported after PFO closure for other reasons. We sought to identify whether there is a specific patient population of migraineurs which may be more susceptible to benefiting from PFO closure. Design Retrospective cohort study. Setting Tertiary care centre. Participants 603 consecutive patients undergoing percutaneous PFO closure for secondary prevention of paradoxical embolism. Interventions PFO closure using the Amplatzer PFO occluder. Main outcome measures Improvement of migraine symptoms. Results 150 patients (25%; aged 51±11 years) suffered from migraine, including 96 patients with migraine with aura. All implantation procedures were successful, without procedural complications. Contrast transoesophageal echocardiography at 6 months showed complete PFO closure in 136 patients (91%), whereas a minimal, moderate or large residual shunt persisted in 11 (7%), 2 (1%) or 1 (1%) patients, respectively. During 5.0±1.9 years of follow-up, one TIA and one ischaemic stroke occurred. Migraine headaches disappeared in 51 patients (34%) and improved in 72 additional patients (48%). Mean subjective improvement was 69±35%. Overall, mean headache frequency (from 2∧3í/month to 1í/month; p<0.001), duration (from 4-72 to <4 h; p<0.001) and intensity (from 7±2 to 3±3; p<0.001) improved significantly. The prevalence of any migraine headaches (from 100% to 66%; p<0.001), migraine with aura (from 64% to 19%; p<0.001) and the number of patients taking any migraine medication (from 90% to 50%; p<0.001) decreased significantly. Both the presence of aura (OR 3.2; 95%CI 1.3 to 8.2; p=0.014) and high pain intensity at baseline (pain scale >5; OR 3.3; 95%CI 1.3 to 8.4; p=0.013) were independent predictors of response to PFO closure. A residual shunt had no influence on migraine improvement (OR 0.6; 95%CI 0.1 to 2.3; p=0.42). Conclusions These results suggest that percutaneous PFO closure durably alters the spontaneous course of shunt-associated migraine, especially but not exclusively in case of migraine with aura. [ABSTRACT FROM AUTHOR]

Abstract: Background: Due to better early and long-term outcome, the increasing population of grown-ups with congenital heart disease (GUCH) brings up unexpected quality of life (QoL) issues. The cardiac lesion by itself is not always the major problem for these patients, since issues pertaining to QoL and psychosocial aspects often predominate. This study analyses the QoL of GUCH patients after cardiac surgery and the possible impact of medical and psychosocial complications. Patients and methods: A questionnaire package containing the SF-36 health survey (health related QoL), the HADS test (anxiety/depression aspects) and an additional disease specific questionnaire was sent to 345 patients (mean 26±11 years) operated for isolated transposition of the great arteries (TGA), tetralogy of Fallot (TOF), and ventricular septal defect (VSD). The scores were compared with age- and gender-matched standard population data and in relation to the underlying congenital heart disease (CHD). Results: In all SF-36 and HADS health dimensions the GUCH patients showed excellent scores (116±20), which are comparable to the standard population (100±15), regardless of the initial CHD (p =0.12). Eighty-two percent of the patients were found to be in NYHA class I and 83% patients declared that they do not consider their QoL to be limited by their malformation. Complications like reoperations (p =0.21) and arrhythmias (p =0.10) do not show significant impact on the QoL. The additional questionnaire revealed that 76% of adult patients have a fulltime job, 18% receive a full or partial disability pension, 21% reported problems with insurances, most of them regarding health insurances (67%), and 4.4% of adult patients declared to have renounced the idea of having children due to their cardiac malformation. Conclusion: QoL in GUCH patients following surgical repair of isolated TOF, TGA and VSD is excellent and comparable to standard population, this without significant difference between the diagnosis groups. However, these patients are exposed to a high rate of complications and special psychosocial problems, which are not assessed by standardized questionnaires, such as the SF-36 and HADS. These findings highlight the great importance for a multidisciplinary and specialized follow-up for an adequate management of these complex patients. [Copyright &y& Elsevier]

Objectives: This study sought to investigate the safety and efficacy of transcatheter treatment of atrial septal aneurysm (ASA) associated with patent foramen ovale (PFO). Background: Patients with both ASA and PFO are at high risk for recurrent paradoxical embolism. Methods: The procedural, echocardiographic, and clinical outcomes of 141 patients with ASA + PFO and ≥1 paradoxical embolic event undergoing transcatheter treatment were compared with 220 patients with PFO alone. Results: Device success (ASA + PFO, 99.3%; PFO alone, 99.5%; p = 0.75) and procedural complications (ASA + PFO, 0.7%; PFO alone, 3.2%; p = 0.12) were similar in both groups. Maximal atrial septal excursion in patients with ASA + PFO decreased from 16 ± 4 mm before to 4 ± 3 mm after the intervention (p < 0.0001). At 6 months follow-up, right-to-left shunt was abolished in 120 (86%) patients with ASA + PFO, compared to 187 (85%) patients with PFO alone (p = 0.80). Freedom from recurrent transient ischemic attack, stroke, and peripheral embolism at 4 years was 95% (ASA + PFO) and 94% (PFO alone, p = 0.70), respectively. A residual right-to-left shunt after the intervention was the only predictor for recurrence (hazard ratio [HR] 6.9; 95% confidence interval [CI] 1.3 to 36.9, p < 0.03) in patients with ASA + PFO. Conclusions: Transcatheter treatment of ASA + PFO is safe and effective in patients with paradoxical embolism. The procedure effectively abolishes right-to-left shunt and decreases atrial septal mobility. Long-term prevention of recurrent events appears favorable when compared to patients with PFO alone. [Copyright &y& Elsevier]

The purpose of this study was to compare the efficacy of medical treatment with percutaneous closure of patent foramen ovale (PFO).Patients with cryptogenic stroke and PFO are at risk for recurrent cerebrovascular events.We compared the risk of recurrence in 308 patients with cryptogenic stroke and PFO, who were treated either medically (158 patients) or underwent percutaneous PFO closure (150 patients) between 1994 and 2000.Patients undergoing percutaneous PFO closure had a larger right-to-left shunt (p < 0.001; 95% confidence interval [CI] 1.38 to 3.07) and were more likely to have suffered more than one cerebrovascular event (p = 0.03; 95% CI 1.04 to 2.71). At four years of follow-up, percutaneous PFO closure resulted in a non-significant trend toward risk reduction of death, stroke, or transient ischemic attack (TIA) combined (8.5% vs. 24.3%; p = 0.05; 95% CI 0.23 to 1.01), and of recurrent stroke or TIA (7.8% vs. 22.2%; p = 0.08; 95% CI 0.23 to 1.11) compared with medical treatment. Patients with more than one cerebrovascular event at baseline and those with complete occlusion of PFO were at lower risk for recurrent stroke or TIA after percutaneous PFO closure compared with medically treated patients (7.3% vs. 33.2%; p = 0.01; 95% CI 0.08 to 0.81, and 6.5% vs. 22.2%; p = 0.04; 95% CI 0.14 to 0.99, respectively).Percutaneous PFO closure appears at least as effective as medical treatment for prevention of recurrent cerebrovascular events in cryptogenic stroke patients with PFO. It might be more effective than medical treatment in patients with complete closure and more than one cerebrovascular event. [Copyright &y& Elsevier]