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Background Patients after atrial switch surgery for transposition of the great arteries (TGA-AtrSO) experience serious clinical events during adulthood, mainly heart failure and arrhythmias, but data on the emerging risks remain scarce. Purpose To assess the risk for events during the clinical course in adulthood of TGA-AtrSO patients and provide a novel risk score for event-free survival. Methods We reviewed medical records of TGA-AtrSO patients from five hospitals. Endpoints were all-cause mortality, heart failure (HF), defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and symptomatic ventricular arrhythmias (VA). Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques. Results We followed 169 TGA-AtrSO patients (60% Mustard, age 28 [IQR 24–36] years) for 13 [IQR 9–16] years, during which 17 (10%) died, 34 (20%) had HF events, and 15 (9%) had VA events. Five-year risk of mortality, first HF event, and first VA increased from 1% each at age 25, to 7% (95% CI 4–10%), 17% (95% CI 10–25%), and 4% (95% CI 2–8%), respectively, at age 50. A prediction model combining age >30, prior VA, age >1 year at repair surgery, QRS duration >120ms, ≥mild LV dysfunction, and severe tricuspid regurgitation discriminated well between patients at low (20%) 5-year risk (optimism corrected C-statistic=0.84). Observed 5- and 10-year survival in low-risk patients were 100% and 99%, compared to only 45% and 19% in high-risk patients. Conclusion The clinical course of atrial switch patients increasingly consists of serious clinical events, especially heart failure. A novel risk score stratifying patients as low, medium, and high risk for event-free survival is presented, providing information on absolute individual risks which may support decisions for pharmacological and interventional management. Figure 1. Observed event-free survival of patients with predicted low risk (20% in 5 years). Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Dutch Heart Foundation; Amsterdam University Fund

Background Anxiety, perceived in patients undergoing percutaneous cardiac intervention is a known problem, which is negatively correlated with quality of life, adherence to recommended clinical practice and medication, health promoting behavior, hospitalization, and patient experience and satisfaction. Hence, preprocedural interventions to alleviate anxiety are interesting. While preoperative education interventions were proven to be effective as anxiety reduction modality, especially those modalities that provide immersive experience appeared to be even more effective within this context. Given the non-invasive nature of these modalities and the introduction of innovative techniques such as Virtual Reality (VR), the question arises whether VR can be successfully introduced as an effective tool to alleviate anxiety in cardiology patients undergoing PFO or ASD closure. Purpose The purpose of the study was to alleviate pre-procedural anxiety in patients undergoing percutaneous PFO or ASD closure using a VR film as an innovative and immersive patient education modality. Methods Patients were randomized to either the VR-group or the control group. The control group received traditional patient information by their cardiologist. The intervention group additionally viewed a 4-minute VR-film, that was designed to create a representative patient-experience of the day of the procedure. The primary outcome was patient anxiety in the VR group compared to the control group, as measured with anxiety scores derived from the State Trait Anxiety Inventory (STAI) questionnaire. Measurements were performed directly after the informed consent visit and one week prior to the procedure. Secondary outcome measure was the duration of anesthesia during the procedure. Results In total 40 patients have been included (mean age = 45 yrs ± 11, male = 21), mean FU duration = 37 days ± 23). So far, 27 patients (VR-group n=14; Control group n=13) completed follow-up. At baseline the control and intervention group were equally anxious (37±9 vs 38±14). During follow-up we found a significant increase in anxiety in the control group (37±9 vs 46±12; p=0.034; figure 1), whereas anxiety in the VR-group remained unchanged (38±14 vs 36±9; figure 1). One week prior to the procedure, the control group was significantly more anxious compared to the VR group (+ 11±4; p=0.012; figure 2). The mean duration of the anesthesia was significantly shorter within the VR-group in comparison with the control group (57±10 min vs 71±18 min; p=0,029). This may be an indication that decreased anxiety could have beneficial effects on the complete duration of anesthesia. Conclusion Our results demonstrate that immersive VR patient education is both innovative and effective in alleviating anxiety prior to a cardiological procedure and may have beneficial effects on patient experience and anesthesia duration. Funding Acknowledgement Type of funding source: None

Background Bicuspid aortic valve (BAV) may frequently lead to aortic dilatation with risk of aortic dissection. In patients with BAV both familial clustering and aortic dilatation in first-degree relatives (FDR) without BAV has been demonstrated. Based on these findings the ESC Aortic Guidelines recommend to consider screening of FDR, while the ACC/AHA Guidelines on Valvular Heart Diseases consider screening of FDR only if the index patient has associated aortopathy. Currently, no data about the effectiveness of screening is available. Purpose To investigate the yield of screening FDR of patients with isolated BAV and to explore subgroups with FDR of patients who had needed surgery or of patients with aortic dilatation. We hypothesized that aortic dilatation (>40mm) in the index patient is not a risk factor for BAV in FDR. Methods From 2012, patients with BAV visiting the outpatient clinic of a teaching hospital, received information advising cardiac screening of FDR. FDR of patients with isolated BAV who were referred, were included. From the 10 index patients from other hospitals, information was retrieved. [Fig.1] Results Referred were FDR from 118 index patients (mean age 60 years, standard deviation [SD] 14, range 15–90 years, 82 males [70%]). Of all index patients 63 (53%) had undergone aortic valve replacement, including concomitant ascending aorta replacement in 25 (21%). In the non-operated index patients, 31 (26%) had dilatation (>40mm) of sinus of Valsalva and/or tubular ascending aorta. Screened were 257 FDR (median 2 per index patient) comprising 20 parents (8%), 103 siblings (40%) and 134 offspring (52%). Mean age of FDR was 48 years (SD16, range 4–83 years) and 89 subjects (42%) were male. The diagnostic imaging modality was echocardiography in 240 cases (93%) and MRI in 17 cases. Ten FDR had an already known BAV and were not included in the screening. Among the 257 FDR, we diagnosed 12 new BAV (4.7%, 95% confidence interval [CI]2.9–8.0%) (mean age 44 years, 50% male). Additionally, we diagnosed 23 new isolated aorta dilatations (8.9%; 95% CI 6.0–13%) at level of sinus of Valsalva and/or tubular ascending aorta (mean age 57 years, 18 [78%] were male) [Fig. 1]. Among them, 11 had hypertension. FDR (n=147) of index patients with BAV and previous aortic valve surgery (n=63), had a risk ratio (RR) of 2.25 (95% CI 0.62–8.10) of having a BAV. FDR (n=126) of index patients with BAV and repaired or unrepaired aortic dilatation (n=56) had RR 0.35 (95% CI 0.10–1.25) of having a BAV. Conclusions Screening FDR of patients with isolated BAV resulted in a reasonable yield of 14% new cases with BAV or isolated aortic dilatation. The RR of the subgroup with aorta dilatation did not justify the limitation of the FDR as suggested in the ACC/AHA Guidelines. Figure 1. Flowchart of screening and result Funding Acknowledgement Type of funding source: None

Aims Sudden cardiac death is a major cause of mortality in adult congenital heart disease (ACHD) patients. The indications for implantable cardioverter-defibrillator (ICD) implantation in ACHD patients are still not well established. We aim to systematically review the literature on indications and outcome of ICD implantation in ACHD patients. Methods and results We performed a comprehensive search in EMBASE, MEDLINE, and Google Scholar to identify all studies on ICD implantation in ACHD patients. We used random effects models to calculate proportions and 95% confidence intervals. Of 1356 articles, 24 studies with 2162 patients were included, with a mean follow-up of 3.6 ± 0.9 years. Half of patients had tetralogy of Fallot. Mean age at implantation was 36.5 ± 5.5 years old and 66% was male. Implantable cardioverter-defibrillators were implanted for primary prevention in 53% (43.5–62.7). Overall, 24% (18.6–31.3) of patients received one or more appropriate ICD interventions (anti-tachycardia pacing or shocks) during 3.7 ± 0.9 years: 22% (16.9–28.8) of patients with primary prevention in 3.3 ± 0.3 years and 35% (26.6–45.2) of patients with secondary prevention in 4.3 ± 1.2 years. Inappropriate shocks occurred in 25% (20.1–31.0) in 3.7 ± 0.8 years and other, particularly lead-related complications in 26% (18.9–33.6) of patients in 3.8 ± 0.8 years. All-cause mortality was 10% during 3.7 ± 0.9 years. Conclusions In ACHD, remarkably high rates of appropriate ICD therapy were reported, both in primary and secondary prevention. Because of the young age and lower death rates, the cumulative beneficial effects are likely greater in ACHD patients than in acquired heart disease patients. However, considering the high rates of inappropriate shocks and complications, case-by-case weighing of costs and benefits, remains essential.

AIMS: To investigate the role of guidelines in structuring the clinical care for adult patients with congenital heart disease (CHD), and to assess adherence to the guidelines in Europe. METHODS AND RESULTS: A selected number of current guidelines were chosen pertaining to operative procedures, investigations, and the use of medication ('interventions'). The source for this analysis was the database of the Euro Heart Survey on adult CHD, which contains retrospectively collected data on 4110 patients followed-up for a median of 5.1 years. For each guideline investigated, patients were selected from the database for whom the particular guideline was relevant. The selected cases were classified according to two criteria: was there an indication for the particular intervention and did the intervention take place? In this manner, cases of 'undue treatment' and 'insufficient treatment' were identified. Adherence to guidelines was found to be good in the case of operative procedures and prophylactic drug treatment. However, regarding diagnostic procedures there had been adherence to guidelines in only slightly more than half of the cases. CONCLUSION: Guidelines have an important role in the actual clinical care of adults with CHD. However, large outcome studies are needed to develop more precise guidelines

Aim Patients with Marfan syndrome may develop dissection due to progressive dilatation in the entire aorta, which is not always predictable by mere anatomic assessment of the aortic diameter, especially of the descending aorta. The aim of this study was to identify the predictive value of aortic stiffness on the occurrence of dissection and progressive aortic dilatation. Methods and results In 78 non-operated patients with Marfan syndrome (mean age 31±8 years, mean aortic root diameter 43±6 mm, range 31–55 mm) aortic stiffness and diameters were assessed by magnetic resonance imaging (MRI) at multiple levels. After a median follow-up of 71 months (25–75%: 68–72 months) a second MRI was performed and the incidence of aortic dissection and progressive aortic dilatation, defined as mean aortic diameter increase \batchmode \documentclass[fleqn,10pt,legalpaper]{article} \usepackage{amssymb} \usepackage{amsfonts} \usepackage{amsmath} \pagestyle{empty} \begin{document} \(>\) \end{document}1 mm/year was determined. During follow-up, 4 (5%) of 78 patients developed an aortic dissection (1 type A, 2 type B, and 1 infra-renal dissection). Twenty (26%) of the 78 patients had progressive aortic root dilatation. There were 5 (6%) patients with progressive descending thoracic aortic dilatation and 6 (7%) with progressive abdominal aortic dilatation. Multivariate logistic regression analysis revealed that local distensibility was an independent predictor of progressive thoracic descending aortic dilatation (OR=4.14, 95% CI, 1.02–16.7). For progressive aortic root and abdominal aortic dilatation local initial diameter appeared to be the major predictor (OR=1.37, 95% CI, 1.16–1.62; OR=1.36, 95% CI, 1.09–1.69, respectively). Conclusion In patients with Marfan syndrome both aortic diameter and aortic distensibility are independent predictors of progressive aortic dilatation. For optimal risk assessment and monitoring of patients with Marfan syndrome, both aortic stiffness and diameter should be assessed at least annually.

This editorial refers to ‘Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance’† by E.R. Valsangiacomo Buchel et al., on page 2721 Tetralogy of Fallot is the most common form of cyanotic congenital heart disease, with a prevalence of 0.26–0.8 per 1000 live births.1 Total repair for tetralogy of Fallot has been available for 50 years with a favourable outcome in most patients. Today, one is faced with an increasing number of patients with residual pulmonary regurgitation. It was previously thought that pulmonary regurgitation in Fallot patients was rather harmless. However, accurate measurements of right ventricular volumes using cardiovascular magnetic resonance (CMR) imaging have visualized important enlargement of the right ventricle in patients with severe pulmonary regurgitation.2 Moreover, it has been recently demonstrated that pulmonary regurgitation leads to progressive right ventricular dilatation and, with time, to right ventricular dysfunction, exercise intolerance, ventricular arrhythmias, and sudden death.3 Pulmonary valve replacement can be performed electively with little risk and may improve symptoms of right ventricular failure and provides excellent mid-term survival. The surgical procedure has a peri-operative mortality of 1–4% and a 10-year survival of 86–95%.4 Previous echocardiographic evaluation of right ventricular dimensions in children and adolescents showed a decrease in end-diastolic volume and end-systolic volume after pulmonary valve replacement. However, in adults, radionuclide angiography measurements showed no effects of pulmonary valve replacement on right ventricular volumes and ejection fraction. … *Corresponding author. Tel: +31 71 5262020, fax: +31 71 5248116. E-mail address : e.e.van\_der\_wall{at}lumc.nl

Thank you for the opportunity to respond to the comment from Hager and co-workers. It is an immense tragedy when a young woman dies during pregnancy. We agree with Hager and co-workers that there is no definite safe aortic root diameter for women with Marfan syndrome …

Purpose: Obstetric and neonatal complications are common during pregnancy in women with Congenital Heart Disease (CHD). We assessed mode of delivery and the prevalence of these complications. Methods: ZAHARA II is a national, prospective multi-center cohort study. We included 213 consecutive pregnant women with CHD and 70 healthy pregnant women (controls). We compared prevalence of obstetric and neonatal complications between women with CHD and healthy women. Results: Vaginal delivery rates were lower in women with CHD compared to healthy women (76.5% vs. 87.0% p=0.087), but did not reach statistical significance. Induction of labour was more common in women with CHD than in controls (37.1% vs. 21.4%, p=0.019) and more assisted deliveries were observed (47.9% vs. 25.7% p=0.001). Elective caesarean sections were more often performed in women with CHD (14.1% vs. 1.4% p=0.002), 36.6% for cardiac reasons. No differences were observed in hypertensive complications of pregnancy or post partum hemorrhage. Offspring death occurred in 2.8% of women with CHD versus 0% in controls. Mean pregnancy duration was 38 weeks in women with CHD compared to 39 weeks in healthy women (p

Purpose: Anatomic measurements and blood pressure differences in rest are currently used to determine whether patients after aortic repair for coarctation have a recoarctation or not. Whether flow patterns in the abdominal aorta at rest and during exercise are of additional value in determining functional restriction of the former coarctation side is unknown. Therefore, we investigated Doppler flow patterns in the abdominal aorta compared these with normal volunteers. Methods: We included random patients after aortic coarctation repair without significant re-coarctation on MRI, or uncontrolled hypertension. All patients underwent a rest echocardiography and a bicycle exercise test with echocardiographic examination limited to 70% of the predicted heart rate. Echocardiography was performed according to international recommendations. The systolic diastolic ratio (S/D) was measured in the abdominal aorta. The pulse delay was the difference of the time measured from R-wave until peak velocity in the left ventricular outflow tract and the abdominal aorta. Ten random healthy volunteered controls underwent the similar protocol. Results: Twenty adult patients were included (10 males, mean age 38 years±5) and 10 healthy controls (4 males, mean age 41 years± 10). The maximum flow velocity in the descending aorta was significantly higher than in healthy volunteers. The S/D ratio at rest was similar for both patient groups, but differed significantly at exercise. The pulse delay was significantly larger in coarctation patients than in controls. All parameters indicate a abnormal arterial systemic flow pattern below the coarctation site. View this table: Conclusion: Patients after coarctation without clinical recoarctation have very abnormal flow patterns in the post coarctation aorta, especially during exercise. Further research is needed to determine whether this is clinical meaningful and whether other definitions of recoarctation should be formulized.

Purpose: Patients with congenital heart disease (CHD) often undergo right-sided surgery, and contribution of the right ventricle (RV) to their cardiac pump function is essential. We aimed to identify determinants of adverse outcome after cardiac surgery. Methods: 503 consecutive adults (56% male, median age 36 years) with CHD operated between January 2001 and January 2011 in the Academic Medical Center in Amsterdam were studied. RV function was considered normal for levels of TAPSE and TDI S' above 15mm and 11 cm/s respectively. RV failure was defined as combination of (1) elevated jugular venous pressure, (2) impaired RV function on transthoracic echocardiography and (3) diagnosis of RV failure was documented in the medical charts. Intensive care stay was prolonged if it exceeded four days. The composite end point of an adverse in-hospital outcome was operative mortality, RV failure, inotropes on intensive care, a prolonged intensive care stay, or all. Determinants of adverse outcome were evaluated by logistic regression analysis. Results: Nine patients (1,8%) died peri-operatively, 19 had RV failure, 83 needed inotropic therapy on intensive care and 21 had prolonged intensive care stay. The composite end point was reached in 91 patients (18,1%). Significant pre-operative determinants are shown in the Table. View this table: Table 1. Pre operative determinants of adverse outcome after congenital cardiac surgery Conclusion: Supraventricular arrhythmia, renal function and ventricular function were determinants of adverse in-hospital outcome after congenital cardiac surgery.