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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: Congenital heart defects (CHD) affect almost 1% of all live born children and the number of adults with CHD is increasing. In families where CHD has occurred previously, estimates of recurrence risk, and the type of recurring malformation are important for counselling and clinical decision-making, but the recurrence patterns in families are poorly understood. We aimed to determine recurrence patterns, by investigating the co-occurrences of CHD in 1163 families with known malformations, comprising 3080 individuals with clinically confirmed diagnosis.Methods and results: We calculated rates of concordance and discordance for 41 specific types of malformations, observing a high variability in the rates of concordance and discordance. By calculating odds ratios for each of 1640 pairs of discordant lesions observed between affected family members, we were able to identify 178 pairs of malformations that co-occurred significantly more or less often than expected in families. The data show that distinct groups of cardiac malformations co-occur in families, suggesting influence from underlying developmental mechanisms. Analysis of human and mouse susceptibility genes showed that they were shared in 19% and 20% of pairs of co-occurring discordant malformations, respectively, but none of malformations that rarely co-occur, suggesting that a significant proportion of co-occurring lesions in families is caused by overlapping susceptibility genes.Conclusion: Familial CHD follow specific patterns of recurrence, suggesting a strong influence from genetically regulated developmental mechanisms. Co-occurrence of malformations in families is caused by shared susceptibility genes.

Item does not contain fulltext AIMS: The aorta in Marfan syndrome (MFS) patients is variably affected. We investigated the assumed genotype-effect on protein production as a risk factor for a severe aortic phenotype in adult MFS patients. METHODS AND RESULTS: We collected clinical and genetic data from all 570 adults with MFS who had been included in the Dutch CONgenital CORvitia registry since the start in 2001. Mean age was 36.5 +/- 13.5 years (51.2% male, 28.9% prior aortic surgery, 8.2% prior aortic dissection). Patients were prospectively followed for a mean duration of 8.2 +/- 3.1 years. Men had more frequently aortic surgery at baseline (38.0 vs. 19.4%, P < 0.001) and during follow-up (24.0 vs. 15.1%, P = 0.008) compared with women. After 10-year follow-up cumulative survival was 93.8% and dissection-free survival was 84.2%. We found a pathogenic FBN1 mutation in 357 patients, of whom 146 patients (40.9%) were positive for a mutation causing haploinsufficiency (reduced fibrillin-1 protein) and 211 (59.1%) for a mutation leading to a DN effect (abnormal fibrillin-1 protein). Corrected for age, sex, and previous aortic complications, patients with a haploinsufficient (HI) mutation had a 2.5-fold increased risk for cardiovascular death (hazard ratio, HR: 2.5, 95% CI: 1.0-6.1, P = 0.049), a 2.4-fold increased risk for the combined endpoint comprising death and dissection (HR: 2.4, 95% CI: 1.4-4.2, P < 0.001) and a 1.6-fold increased risk for any aortic complication compared with patients with a DN mutation (HR: 1.6, 95% CI 1.1-2.3, P = 0.014). CONCLUSION: Marfan syndrome patients with an HI mutation are at increased risk for cardiovascular death and aortic dissection compared with patients with a DN mutation.

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.

Item does not contain fulltext AIMS: The identification of sex differences in the prognosis of adults with a secundum atrial septal defect (ASD2) could help tailor their clinical management, as it has in other cardiovascular diseases. We investigated whether disparity between the sexes exists in long-term outcome of adult ASD2 patients. METHODS AND RESULTS: Patients with ASD2 classified as the primary defect were selected from the Dutch national registry of adult congenital heart disease. Survival stratified by sex was compared with a sex-matched general population. In a total of 2207 adult patients (mean age at inclusion 44.8 years, 33.0% male), 102 deaths occurred during a cumulative follow-up of 13 584 patient-years. Median survival was 79.7 years for men and 85.6 years for women with ASD2. Compared with the age- and sex-matched general population, survival was lower for male, but equal for female patients (P = 0.015 and 0.766, respectively). Logistic regression analyses showed that men had a higher risk of conduction disturbances (OR = 1.63; 95% CI, 1.22-2.17) supraventricular dysrhythmias (OR = 1.41; 1.12-1.77), cerebrovascular thromboembolic events (OR = 1.53; 1.10-2.12), and heart failure (OR = 1.91; 1.06-3.43). CONCLUSION: In contrast to women, adult men with an ASD2 have worse survival than a sex-matched general population. Male patients also have a greater risk of morbidity during adult life. Sex disparity in survival and morbidity suggests the need for a sex-specific clinical approach towards these patients.

Guidelines summarize and evaluate all currently available evidence on a particular issue with the aim of assisting physicians in selecting the best management strategies for an individual patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines are no substitutes for textbooks, and their legal implications have been discussed previously. Guidelines and recommendations should help physicians to make decisions in their daily practice. However, the ultimate judgement regarding the care of an individual patient must be made by his/her responsible physician(s). A large number of Guidelines have been issued in recent years by the European Society of Cardiology (ESC) as well as by other societies and organizations. Because of the impact on clinical practice, quality criteria for the development of guidelines have been established in order to make all decisions transparent to the user. The recommendations for formulating and issuing ESC Guidelines can be found on the ESC Web Site (http://www.escardio.org/guidelines/rules). Members of this Task Force were selected by the ESC to represent all physicians involved with the medical care of patients in this pathology. In brief, experts in the field are selected and undertake a comprehensive review of the published evidence for management and/or prevention of a given condition. A critical evaluation of diagnostic and therapeutic procedures is performed, including assessment of the risk–benefit ratio. Estimates of expected health outcomes for larger populations are included, where data exist. The level of evidence and the strength of recommendation of particular treatment options are weighed and graded according to pre-defined scales, as outlined in Tables 1 and 2 . View this table: Table 1 Classes of recommendations View this table: Table 2 Levels of evidence The experts of the writing and reviewing panels have provided disclosure statements of all relationships they may have which …

This editorial refers to ‘Efficacy of losartan vs. atenolol for the prevention of aortic dilation in Marfan syndrome: a randomized clinical trial’[†][1], by A. Forteza et al ., on page 978. The only validated therapy for Marfan patients with aortic aneurysms has always been invasive surgery of the dilated aorta and awaiting progression of the disease to affect the remainder.1,2 For >20 years, pharmacological treatment consisted of beta-blockade as a supportive therapy in order to slow down the rate of aortic dilatation, supposedly by lowering blood pressure and d P /d t in the aorta. Although recommended in North American and European guidelines, beta-blockade has been prescribed based on studies with conflicting results, and only one small, prospective, randomized study of 70 patients.3 Recently, after evidence of the effectiveness of losartan in a Marfan mouse model, expectations were sky high, as it was speculated that this drug might become a more causative treatment, by reducing transforming growth factor-β (TGF-β) signalling in the diseased aortic wall, proposed to be at the origin of the disease.4 The first results in humans were very encouraging. Losartan seemed effective in … [1]: #fn-2

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

Aims To investigate magnitude and determinants of risks during pregnancy in women with Mustard or Senning repair for complete transposition of the great arteries (TGA).Methods and results Using a nationwide registry (CONCOR), 70 women with Senning (23%) or Mustard (77%) repair for TGA were enrolled. A total of 28 patients had 69 pregnancies (two twins), including 17 spontaneous miscarriages and three elective abortions. During 39 of the 49 completed pregnancies, complications were observed. The most important cardiac complication was clinically significant arrhythmia (n=11, 22%), especially occurring in patients with a prior history of arrhythmia. Important general pregnancy complications were preeclampsia (n=5, 10.2%) and pregnancy-induced hypertension (n=4, 8.2%). Obstetric complications included premature rupture of membranes (n=7, 14.3%), premature labour (n=12, 24.4%), premature delivery (n=16, 31.4%), and thrombo-embolic complications (n=2, 4.1%). Mean (singleton) pregnancy duration was 36 +/- 5 weeks. Eleven of the 51 children (21.6%) were small for gestational age. Foetal and neonatal mortality combined was 11.8% (n=6). No recurrence of congenital heart disease in the offspring was documented.Conclusion In this largest report on pregnancy in women with atrial-corrected TGA to date, a high incidence of obstetric complications and mortality in the offspring was observed.

Aims In most pregnancy reports, atrioventricular septal defects (AVSD) are not differentiated from more simple septal defects, thus underestimating the risks of pregnancy. To investigate the magnitude and determinants of risk during pregnancy in female patients with balanced AVSD.Methods and results Using a nation-wide registry (CONCOR), 79 female patients with balanced/isolated AVSD were identified. A total of 29 patients had 62 pregnancies, including 12 miscarriages (19%) and two elective abortions. Detailed recordings of each completed (greater-than 20 weeks gestation) pregnancy (n=48, 26 women) were obtained. Cardiovascular events complicated almost 40% of the completed pregnancies. In particular, post-partum persistence of pregnancy-related New York Heart Association (NYHA) class deterioration [23% mainly patients with residual atrial septal defects (ASD)] and deterioration of pre-existing left AV-valvular regurgitation (17%) were frequently recorded. Additional cardiac complications were arrhythmias (19%) and symptomatic heart failure (2%). Congenital heart disease (CHD) recurred in six children (12%): AVSD (n=4, three with left-sided hypoplasia), patent ductus arteriosus (n=1), and ASD (n=1). Three children died including two children with left-sided hypoplasia.Conclusion Pregnancy is not always well tolerated in women with AVSD, predominantly due to NYHA class deterioration and worsening of pre-existing AV-valvular regurgitation. Offspring mortality is high (6.3%), primarily due to recurrence of complex CHD.

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.

Aims The risk of infective endocarditis (IE) in adults with congenital heart disease is known to be increased, yet empirical risk estimates are lacking. We sought to predict the occurrence of IE in patients with congenital heart disease at the transition from childhood into adulthood. Methods and results We identified patients from the CONCOR national registry for adults with congenital heart disease. Potential predictors included patient characteristics, and complications and interventions in childhood. The outcome measure was the occurrence of IE up to the age of 40 and 60. A prediction model was derived using the Cox proportional hazards model and bootstrapping techniques. The model was transformed into a clinically applicable risk score. Of 10 210 patients, 233 (2.3%) developed adult-onset IE during 220 688 patient-years. Predictors of IE were gender, main congenital heart defect, multiple heart defects, and three types of complications in childhood. Up to the age of 40, patients with a low predicted risk (

Aims The significance of mild residual. descending aortic narrowing in post-coarctectomy patients is not known. The aim of our study was to investigate the influence of mild residual descending aortic narrowing on blood pressure and vascular damage in patients after repair of aortic coarctation. Methods and results In 107 consecutive post-coarctectomy patients, magnetic resonance imaging, ambulatory blood pressure monitoring, and B-mode ultrasound of the carotid arteries were performed. A significant residual aortic narrowing was defined as: (i) a resting blood pressure gradient &GE; 30 mmHg with hypertension or exercise-induced hypertension (European Society of Cardiology guidelines); and/or (ii) a site of repair/diaphragmatic aorta ratio &LT; 0.7. Thirty-four patients (32%) had a significant residual aortic narrowing and were excluded from the analysis. Of the remaining 73 patients (43 mate) with no or only mild residual descending aortic narrowing, median age was 29.8 years (range 17.1-52.5 years), mean age at repair 8.1 years (range 0.02-37.3 years), mean arm/leg gradient 2 &PLUSMN; 12 mmHg, and mean common carotid intima-media thickness 0.612 &PLUSMN; 0.118 mm. Thirty-three (45%) of these patients had hypertension. In multivariable regression analysis the site of repair/diaphragmatic aorta ratio was a strong and independent predictor of mean daytime systolic blood pressure (P &LT; 0.001) and common carotid intima-media thickness (P = 0.027). Conclusion Mild residual descending aortic narrowing in post-coarctectomy patients is independently associated with mean daytime blood pressure and carotid intima-media thickness. Our data suggest that a threshold for re-intervention of residual. aortic narrowing tower than posed in current guidelines may be desirable to improve Long-term outcome in these patients. However, further research on such aggressive interventional. approaches is needed

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.