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ObjectiveThe clinical and prognostic implications of a hypertensive response to exercise after repair of coarctation of the aorta (CoA) remain controversial. We aimed to determine the prevalence of a hypertensive response to exercise, identify factors associated with peak exercise systolic blood pressure (SBP) and explore the association of peak exercise SBP with resting blood pressure and cardiovascular events during follow-up.MethodsFrom the Dutch national CONgenital CORvitia (CONCOR) registry, adults with repaired CoA who underwent exercise stress testing were included. A hypertensive response to exercise was defined as a peak exercise SBP ≥210 mm Hg in men and ≥190 mm Hg in women. Cardiovascular events consisted of coronary artery disease, stroke, aortic complications and cardiovascular death.ResultsOf the original cohort of 920 adults with repaired CoA, 675 patients (median age 24 years (range 16–72 years)) underwent exercise stress testing. Of these, 299 patients (44%) had a hypertensive response to exercise. Mean follow-up duration was 10.1 years. Male sex, absence of a bicuspid aortic valve and elevated resting SBP were independently associated with increased peak exercise SBP. Peak exercise SBP was positively predictive of office SBP (β=0.11, pConclusionsA hypertensive response to exercise was present in nearly half of the patients in this large, prospective cohort of adults with repaired CoA. Risk factors for increased peak exercise SBP were male sex, absence of a bicuspid aortic valve and elevated resting SBP. Increased peak exercise SBP independently predicted hypertension at follow-up. These results support close follow-up of patients with a hypertensive response to exercise to ensure timely diagnosis and treatment of future hypertension.

Background In the GLOBAL LEADERS trial, ticagrelor monotherapy beyond 1 month compared with standard antiplatelet regimens after coronary stent implantation did not improve outcomes at intention‐to‐treat analysis. Considerable differences in treatment adherence between the experimental and control groups may have affected the intention‐to‐treat results. In this reanalysis of the GLOBAL LEADERS trial, we compared the experimental and control treatment strategies in a per‐protocol analysis of patients who did not deviate from the study protocol. Methods and Results Baseline and postrandomization information were used to classify whether and when patients were deviating from the study protocol. With logistic regressions, we derived time‐varying inverse probabilities of nondeviation from protocol to reconstruct the trial population without protocol deviation. The primary end point was a composite of all‐cause mortality or nonfatal Q‐wave myocardial infarction at 2 years. At 2‐year follow‐up, 1103 (13.8%) of 7980 patients in the experimental group and 785 (9.8%) of 7988 patients in the control group qualified as protocol deviators. At per‐protocol analysis, the rate ratio for the primary end point was 0.88 (95% CI, 0.75–1.03; P =0.10) on the basis of 274 versus 325 events in the experimental versus control group. The rate ratio for the key safety end point of major bleeding was 1.00 (95% CI, 0.79–1.26; P =0.99). The per‐protocol and intention‐to‐treat effect estimates were overall consistent. Conclusions Among patients who complied with the study protocol in the GLOBAL LEADERS trial, ticagrelor plus aspirin for 1 month followed by ticagrelor monotherapy was not superior to 1‐year standard dual antiplatelet therapy followed by aspirin alone at 2 years after coronary stenting. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT01813435.

Background Pulmonary atresia and ventricular septal defect (PA-VSD), with or without systemic pulmonary collateral arteries (SPCAs), represents a complex anatomic and surgical spectrum of congenital heart disease. Currently, there is limited evidence on homograft durability after complete correction, which potentially could be affected by anatomic differences in pulmonary vasculature. Methods This retrospective single-center study included all 69 consecutive PA-VSD patients (46 with SPCAs, 23 without SPCAs) operated on between 1978 and 2018. The primary interest was in homograft durability after complete repair. Longitudinal echocardiographic homograft function and right ventricular systolic pressure were analyzed with linear mixed-effects models. Results The median duration of follow-up was 20 years. Of the 46 patients with SPCAs, 37 (80.4%) underwent biventricular correction at a median age of 2.7 years (interquartile range [IQR], 1.8-6.3 years). Two patients are currently awaiting unifocalization and correction. All 23 patients without SPCAs underwent successful complete correction at a median age of 1.6 years (IQR, 1.1-3.6 years). Freedom from any reintervention after 20 years was 15%. When a homograft was used during correction, freedom from homograft replacement after 20 years was comparable in the 2 groups (P = .925), at 32 ± 11% in the SPCA group and 32 ± 13% in the non-SPCA group. Indications for homograft replacement were isolated stenosis (n = 7; 46.7%), isolated regurgitation (n = 3; 20.0%), and mixed stenosis and regurgitation (n = 5; 33.3%) in the SPCA group and isolated stenosis (n = 8; 88.9%) and stenosis and regurgitation (n = 1; 11.1%) in the non-SPCA group. Peak homograft gradient was significantly (P = .0003) higher in patients without SPCA, with a comparable rate of progression in the 2 groups. However, the prevalence of severe pulmonary regurgitation (PR) was higher in patients with SPCAs, estimated at 35% at 10 years, compared with 15% in patients without SPCAs. Conclusions Homografts used for right ventricular outflow tract reconstruction in patients with PA-VSD, either with or without SPCAs, have similar limited durability. Repeated reintervention is common, and careful follow-up with attention to severe PR is warranted.

Background The long‐term burden of cardiovascular disease after repair of coarctation of the aorta (CoA) has not been elucidated. We aimed to determine the incidence of and risk factors for cardiovascular events in adult patients with repaired CoA. Additionally, mortality rates were compared between adults with repaired CoA and the general population. Methods and Results Using the Dutch Congenital Corvitia (CONCOR) registry, patients aged ≥16 years with previous surgical or transcatheter CoA repair from 5 tertiary referral centers were included. Cardiovascular events were recorded, comprising coronary artery disease, stroke/transient ischemic attack, aortic complications, arrhythmias, heart failure hospitalizations, endocarditis, and cardiovascular death. In total, 920 patients (median age, 24 years [range 16–74 years]) were included. After a mean follow‐up of 9.3±5.1 years, 191 patients (21%) experienced at least 1 cardiovascular event. A total of 270 cardiovascular events occurred, of which aortic complications and arrhythmias were most frequent. Older age at initial CoA repair (hazard ratio [HR], 1.017; 95% CI, 1.000–1.033 [ P =0.048]) and elevated left ventricular mass index (HR, 1.009; 95% CI, 1.005–1.013 [ P P Conclusions This large, prospective cohort of adults with repaired CoA showed a high burden of cardiovascular events, particularly aortic complications and arrhythmias, during long‐term follow‐up. Older age at initial CoA repair and elevated left ventricular mass index were independent risk factors for the occurrence of cardiovascular events. Mortality was 3.3‐fold higher compared with the general population. These results advocate stringent follow‐up after CoA repair and emphasize the need for improved preventive strategies.

OBJECTIVES Coarctation of the aorta (CoA) is rarely diagnosed and treated in adults and nowadays mostly treated with percutaneous techniques. The objective of this study is to report the long-term outcomes and health-related quality of life in a unique cohort of patients treated with an open surgical repair for their primary adult CoA. METHODS Ninety adult patients underwent primary surgical repair of CoA at our tertiary referral centre between 1961 and 2008 when the treatment strategy for adult CoA was exclusively surgical. RESULTS The median age at surgery was 24 years (interquartile range 20–36 years), and 39 patients (43%) were asymptomatic at presentation. CoA was located paraductally in most patients (64%), and bicuspid aortic valve was present in 39 (43%) patients. Surgical reconstruction of CoA with an end-to-end anastomosis was performed in majority of the patients (57%). Overall, in-hospital mortality occurred in 1 patient (1%). There was no in-hospital stroke, spinal cord ischaemia, renal replacement therapy or respiratory failure. The cumulative survival was 97.7%, 89.5%, 82.6%, 70.9% and 61.4% at 10, 20, 30, 40 and 50 years, respectively. Thirty-one patients (34%) required an additional cardiac surgery during follow-up. The majority of patients (77%) suffered from refractory hypertension even after decades of surgery. Compared with the matched Dutch population, patients reported a lower social functioning, mental health, vitality and general health with a higher body pain. CONCLUSIONS Patients with native adult CoA have low in-hospital morbidity and mortality when treated with an open surgical reconstruction. However, refractory hypertension and impaired quality of life remain important challenges during follow-up.

Background Coronary compression (CC) is a life threatening complication that can occur during percutaneous pulmonary valve implantation (PPVI). We describe our experience using cardiac CT prior to PPVI to identify patients at high CC-risk due to a close relationship between the coronary arteries and pulmonary trunk (PT). Methods A retrospective evaluation of candidates for PPVI who underwent CT prior to the procedure was done. Measurements of PT were performed using double oblique reconstructed images, with special attention to the stenotic tract of the PT. The analysis of coronary arteries included detection of anomalies of origin and course and assessment of their relationship with the PT, measuring the minimum distance between the coronary artery and the intended site of the future percutaneous valve implantation. Results CT analysis was performed for 52 patients. Thirty patients underwent PPVI after CT and 22 didn’t. In 6/22 cases the reason not to receive a PPVI was high CC-risk detected at CT. In 6 other patients CT detected an intermediate CC-risk but the test balloon performed during angiography prior to valve placement was safe and the patients successfully underwent the procedure. None of the patients deemed as no CC-risk at CT had CC during PPVI. Conclusion CT can detect patients with high and intermediate CC-risk and therefore may identify which patients are unlikely to undergo successful PPVI and those who need a careful analysis with balloon testing. CT can also rule out CC-risk identifying those patients in which balloon inflation testing could be omitted.

Prognosis in children with pulmonary vascular disease (PVD) is closely linked to right ventricular (RV) failure due to increased RV-afterload. Pulmonary arterial (PA) stiffening is known to occur early in the course of PVD and constitutes a main component of RV-afterload. This study aimed to evaluate the clinical value of PA-stiffness in children with PVD by determining its association with advanced pulmonary arterial hypertension (PAH) and mortality at long-term follow-up.Forty-one children with various stages of arterial PVD, defined as mean PA-pressure ≥20 mmHg and/or pulmonary-to-systemic flow-ratio ≥1.2, and mean pulmonary capillary wedge pressure 15 mmHg, underwent cardiac catheterization with intravascular ultrasound (IVUS) imaging between 1994 and 1997 with follow-up until 2015. PA-stiffness indices evaluated were PA-area-compliance (PA-compliance) and PA-area-distensibility (PA-distensibility). During follow-up, advanced PAH was determined by echocardiography and cardiac catheterization. During a median follow-up of 19 years, in 31 (76%) patients PVD had reversed and 10 (24%) had advanced PAH. Six (15%) died due to PVD. In addition to conventional haemodynamics, PA-compliance and PA-distensibility were associated with advanced PAH at long-term follow-up (adjusted OR [95% CI] 0.56 [0.37-0.85] and 0.52 [0.31-0.86]), and mortality (adjusted HR [95% CI] 0.60 [0.41-0.87] and 0.67 [0.49-0.90]). Also in a subgroup of patients with favourable haemodynamics, baseline PA-compliance and PA-distensibility were lower in patients with advanced PAH at follow-up (P = 0.002 /P = 0.030).In children with PVD, PA-stiffness indices assessed by IVUS predict advanced PAH and mortality at long term follow-up. Especially in patients with favourable haemodynamics, assessment of intrinsic PA-stiffness may enhance the prognostication of disease progression and survival.

ObjectiveGalectin-3 is an emerging biomarker for risk stratification in patients with heart failure. This study aims to investigate the release of galectin-3 and its association with cardiovascular events in patients with adult congenital heart disease (ACHD).MethodsIn this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013. Galectin-3 was measured in thaw serum by batch analysis. The association between galectin-3 and a primary endpoint of all-cause mortality, heart failure, hospitalisation, arrhythmia, thromboembolic events and cardiac interventions was investigated using multivariable Cox models. Reference values and reproducibility were established by duplicate galectin-3 measurements in 143 healthy controls.ResultsGalectin-3 was measured in 591 (98%) patients (median age 33 (25–41) years, 58% male, 90% New York Heart Association (NYHA) class I). Median galectin-3 was 12.7 (range 4.2–45.7) ng/mL and was elevated in 7% of patients. Galectin-3 positively correlated with age, cardiac medication use, NYHA class, loss of sinus rhythm, cardiac dysfunction and N-terminal pro-B-type natriuretic peptide (NT-proBNP). During a median follow-up of 4.4 (IQR 3.9–4.8) years, the primary endpoint occurred in 195 patients (33%). Galectin-3 was significantly associated with the primary endpoint in the univariable analysis (HR per twofold higher value 2.05; 95% CI 1.44 to 2.93, pConclusionsGalectin-3 is significantly associated with functional capacity, cardiac function and adverse cardiovascular events in patients with ACHD. Nevertheless, the additive value of galectin-3 to a more conventional risk marker such as NT-proBNP seems to be limited.

Contains fulltext : 190533.pdf (Publisher’s version ) (Open Access) BACKGROUND: Red cell distribution width (RDW) is a standard component of the automated blood count, and is of prognostic value in heart failure and coronary heart disease. We investigated the association between RDW and cardiovascular events in patients with adult congenital heart disease (ACHD). METHODS AND RESULTS: In this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013. RDW was measured in fresh venous blood samples at inclusion in 592 patients (median age 33 [IQR 25-41] years, 58% male, 90% NYHA I) and at four annual follow-up visits. During 4.3 [IQR 3.8-4.7] years of follow-up, the primary endpoint (death, heart failure, hospitalization, arrhythmia, thromboembolic events, cardiac intervention) occurred in 196 patients (33%). Median RDW was 13.4 (12.8-14.1)% versus 12.9 (12.5-13.4)% in patients with and without the primary endpoint (P

Background We hypothesised that ticagrelor, in combination with aspirin for 1 month, followed by ticagrelor alone, improves outcomes after percutaneous coronary intervention compared with standard antiplatelet regimens. Methods GLOBAL LEADERS was a randomised, open-label superiority trial at 130 sites in 18 countries. Patients undergoing percutaneous coronary intervention with a biolimus A9-eluting stent for stable coronary artery disease or acute coronary syndromes were randomly assigned (1:1) to 75-100 mg aspirin daily plus 90 mg ticagrelor twice daily for 1 month, followed by 23 months of ticagrelor monotherapy, or standard dual antiplatelet therapy with 75-100 mg aspirin daily plus either 75 mg clopidogrel daily (for patients with stable coronary artery disease) or 90 mg ticagrelor twice daily (for patients with acute coronary syndromes) for 12 months, followed by aspirin monotherapy for 12 months. Randomisation was concealed, stratified by centre and clinical presentation (stable coronary artery disease vs acute coronary syndrome), and blocked, with randomly varied block sizes of two and four. The primary endpoint at 2 years was a composite of all-cause mortality or non-fatal centrally adjudicated new Q-wave myocardial infarction as assessed by a core lab in a blinded manner. The key secondary safety endpoint was site-reported bleeding assessed according to the Bleeding Academic Research Consortium criteria (grade 3 or 5). Analysis was by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT01813435, and is closed to new participants, with followup completed. Findings Between July 1, 2013, and Nov 9, 2015, 15 968 participants were randomly assigned, 7980 to the experimental group and 7988 to the control group. At 2 years, 304 (3.81%) participants in the experimental group had died or had a non-fatal centrally adjudicated new Q-wave myocardial infarction, compared with 349 (4.37%) participants in the control group (rate ratio 0.87 [95% CI 0. 75-1. 01]; p=0.073]). There was no evidence for a difference in treatment effects for the primary endpoint across prespecified subgroups of acute coronary syndromes and stable coronary artery disease (p=0.93). Grade 3 or 5 bleeding occurred in 163 participants in the experimental group and 169 in the control group (2.04% vs 2.12%; rate ratio 0.97 [95% CI 0. 78-1. 20]; p=0.77). Interpretation Ticagrelor in combination with aspirin for 1 month followed by ticagrelor alone for 23 months was not superior to 12 months of standard dual antiplatelet therapy followed by 12 months of aspirin alone in the prevention of all-cause mortality or new Q-wave myocardial infarction 2 years after percutaneous coronary intervention. Copright (C) 2018 Elsevier Ltd. All rights reserved. European Clinical Research Institute; Biosensors International; AstraZeneca; Medicines Company; Canada Research Chairs Programme

Background: The number of patients with adult congenital heart disease (ACHD) is rapidly increasing. To optimize patient management, there is a great need to accurately identify high-risk patients. Still, no biomarker has been firmly established as a clinically useful prognostic tool in this group. We studied the association of N-terminal pro-B-type natriuretic peptide (NT-proBNP), high-sensitive troponin-T, and growth-differentiation factor 15 with cardiovascular events in ACHD. Methods: Clinically stable patients with ACHD who routinely visited the outpatient clinic between April 2011 and April 2013 underwent clinical assessment, electrocardiography, echocardiography, and biomarker measurement (NT-proBNP, high-sensitive troponin-T, and growth-differentiation factor 15) at the time of study inclusion. Patients were prospectively followed for the occurrence of cardiovascular events (death, heart failure, hospitalization, arrhythmia, thromboembolic events, and reintervention). Survival curves were derived by the Kaplan-Meier method, and Cox regression was performed to investigate the relation between biomarkers and events with adjustment for multiple clinical and echocardiographic variables. Results: In total, 595 patients were included (median age, 33 years; interquartile range, 25–41 years; 58% male; 90% New York Heart Association class I). Patients were followed during a median of 42 (interquartile range, 37–46) months. Of the 3 evaluated biomarkers, NT-proBNP in the upper quartile (>33.3 pmol/L) was most strongly associated with cardiovascular events (n=165, adjusted hazard ratio, 9.05 [3.24–25.3], P P 14 pmol/L), elevated high-sensitive troponin-T (>14 ng/L), and elevated growth-differentiation factor 15 (>1109 ng/L) identified those patients at highest risk of cardiovascular events (log-rank P Conclusions: NT-proBNP provides prognostic information beyond a conventional risk marker model in patients with ACHD and can reliably exclude the risk of death and heart failure. Elevated levels of NT-proBNP, high-sensitive troponin-T, and growth-differentiation factor 15 identify patients at highest risk of cardiovascular events. These biomarkers therefore may play an important role in the monitoring and management of patients with ACHD.

Background: Left atrial (LA) size predicts cardiovascular outcome in chronic heart failure. Its prognostic value in adults with repaired tetralogy of Fallot (ToF) is unknown. This study therefore investigated the association of LA size and function with cardiovascular events in adults with ToF. Methods: Clinically stable adults with ToF who visited the outpatient clinic between 2011 and 2013 underwent echocardiography and were prospectively followed for the occurrence of death, heart failure, hospitalizations, arrhythmia, thromboembolic events, and re-interventions. LA maximal, minimal and pre-A wave volume, area and length were measured on the apical four-chamber view. Total, passive and active emptying fractions were calculated. Results: In total, 134 patients were included (median age 35 [IQR 29-45] years, 65% male, 91% NYHA I). Median follow-up was 40 [IQR 32-47] months. Patients with a dilated LA (≥34mL/m2, 43%) were at higher risk of cardiovascular events (n=33, adjusted HR 2.48 [1.09-5.62], P =0.030). Analysis of LA volumes as continuous variables yielded similar conclusions. In addition, LA length (adjusted HR 2.49 [1.51-4.09], P

Item does not contain fulltext PURPOSE: The aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome. METHODS AND RESULTS: All Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described. Partial anomalous pulmonary venous return was diagnosed in 24 (25%) out of 96 Turner patients included and 14 (58%) of these 24 partial anomalous pulmonary venous return had not been reported previously. Right atrial or ventricular dilatation was present in 11 (46%) of 24 partial anomalous pulmonary venous return patients. CONCLUSION: When studied with advanced imaging modalities and looked for with specific attention, PAPVR is found in 1 out of 4 Turner patients. Half of these patients had right atrial and/or ventricular dilatation. Evaluation of pulmonary venous return should be included in the standard protocol in all Turner patients.

Atrial septal defect (ASD) closure is performed to prevent pulmonary hypertension (PH), which is associated with poor outcome. This study investigated the prevalence of PH in adults before and after ASD closure and explored associations between patient characteristics and PH after ASD closure. Consecutive adult patients who underwent surgical or percutaneous ASD closure in the Erasmus MC, the Netherlands, were included (2000 to 2014). Echocardiograms before and after ASD closure were retrospectively assessed. Patients were categorized into 3 groups (no PH, possible PH, and PH) based on tricuspid regurgitation velocity (2.9, 2.9 to 3.4, and ≥3.4 m/s) or mean pulmonary arterial pressure (20, 20 to 24, and ≥25 mm Hg). Cox regression was performed to identify associations between patient characteristics and PH after ASD closure. Of the 244 eligible patients who underwent ASD closure, 198 (81%) had echocardiograms both before and median 15 (interquartile range 12 to 35) months after ASD closure (median age at closure 45 [interquartile range 30 to 57] years, 75% woman). The prevalence of PH was 13.1% (n = 26) before ASD closure and 5.0% (n = 10) after closure. New York Heart Association III to IV (hazard ratio [HR] 11.07, 95% confidence interval [CI] 3.12 to 39.29, p0.001), pulmonary disease (HR 10.43, 95% CI 2.12 to 51.21, p = 0.004), cardiac medication use (HR 3.96, 95% CI 1.02 to 15.34, p = 0.047), right ventricular fractional area change (HR 0.87, 95% CI 0.81 to 0.93, p0.001), and tricuspid annular plane systolic excursion (HR 0.75, 95% CI 0.59 to 0.95, p = 0.018) were significantly associated with PH. In conclusion, adult patients with low pulmonary pressures before ASD closure are not at risk of PH after closure. Nevertheless, PH remained prevalent in approximately 5% of patients. Especially those patients with high New York Heart Association functional class, presence of pulmonary disease, cardiac medication use and impaired RV function at baseline are at risk.

textabstractAims: Reliable evaluation of the severity and consequences of pulmonary regurgitation (PR) in patients with repaired tetralogy of Fallot (TOF) is crucial to timely identify the need for pulmonary valve intervention. We aimed to identify the accuracy of echocardiographic parameters to differentiate between moderate and severe PR, using phase contrast cardiac magnetic resonance imaging (CMR) as gold standard. Methods and results: In this cross-sectional study, 45 TOF patients with both echocardiographic and CMR measurements of PR were enrolled. All quantitative and semiquantitative echocardiographic measurements such as pressure half time (PHT), Color flow jet width (CFJW), ratio CFJW/right ventricle outflow tract (RVOT) diameter, PR index and the presence of early termination of the PR jet, end-diastolic antegrade flow and diastolic backflow in main pulmonary artery (MPA), and PA branches correlated significantly with PR fraction on CMR. Qualitative assessment with color flow on echocardiography overestimated PR Multivariate linear regression analysis identified the ratio of CFJW/RVOT diameter and PHT as independent predictors of PR fraction. Accuracy of echo parameters was tested to differentiate between mild-to-moderate and severe PR Combining different echocardiographic parameters increased sensitivity and specificity. The addition of diastolic flow reversal in the PA branches to PHT below 167 milliseconds increased the NPV from 87% to 89% and PPV from 62% to 76%. Conclusions: Comparison with CMR confirms that echocardiographic parameters are reliable in predicting PR severity. Combined measurement of diastolic flow reversal in the pulmonary artery branches and PHT is reliable in the detection of severe PR in the follow-up of TOF patients.

Background Adult patients with repaired tetralogy of Fallot (ToF) are at risk for complications such as heart failure and sudden cardiac death, and identifying high-risk patients is important. Reduced left ventricular (LV) and right ventricular (RV) function has been identified as a predictor of outcomes. However, LV ejection fraction is often preserved, and RV function is difficult to assess. With the introduction of strain analysis, an easy and more sensitive parameter became available. The aim of this study was to investigate the association between strain variables and cardiovascular events in patients with ToF. Methods Stable adult patients with repaired ToF were consecutively included in a prospective observational study between 2011 and 2013 (N = 151; median age, 33.2 years [interquartile range, 25.5–42.0 years]; 61.6% men). For the left ventricle, global longitudinal strain and apical and basal rotation were measured, and longitudinal strain was measured for the right ventricle. The primary endpoint was a composite of death or heart failure. The secondary endpoint was a composite of death, heart failure, arrhythmia, reintervention, or hospitalization for cardiac reasons. Results During a median follow-up period of 71.5 months (interquartile range, 64.0–75.3 months), the primary and secondary endpoints occurred in 14 (9%) and 62 (41%) patients, respectively. After adjusting for LV ejection fraction and LV global longitudinal strain, RV longitudinal strain remained independently associated with the primary endpoint in a ridge regression analysis. LV apical rotation remained independently associated with the secondary end point (adjusted hazard ratio, 0.72; 95% CI, 0.52–0.98; P = .035) after adjusting for age, New York Heart Association functional class, QRS duration, LV ejection fraction, RV longitudinal strain, and LV global longitudinal strain. Conclusions Myocardial deformation variables of both the left and right ventricles were associated with cardiovascular events in patients with ToF. LV and RV longitudinal strain and LV rotation should become part of the routine assessment of patients with ToF.

Objectives This study examines the outcome and procedural outcomes of percutaneous stent angioplasty for aortic coarctation in patients with Turner syndrome (TS). Background TS occurs in 1 in 2,500 live-born females and is associated with aortic coarctation. Methods In this multicenter, retrospective cohort study, all patients with TS and a coarctation of the aorta, treated with percutaneous stent implantation were included. The procedural strategies were dictated by local protocols. Adverse events at short- and long-term follow-up and qualitative parameters concerning the stent implantation were assessed. Results In the largest study to date of TS patients receiving aortic stents, a total of 19 patients from 10 centers were included. Twelve patients were treated for native and 7 for recurrent coarctation. Age at intervention was 16.9 (7–60) years (median; min–max). The coarctation diameter increased significantly from 8.0 mm (2–12) pre-intervention to 15.0 mm (10–19) post-intervention (P

Context Matrix metalloproteinases (MMPs) are associated with diastolic dysfunction and heart failure in acquired heart disease. Objective To investigate the role of MMPs as novel biomarkers in clinically stable adults with congenital heart disease. Methods We measured serum MMP-2, -3, -9 and tissue inhibitor of matrix metalloproteinase-1 in 425 patients and analysed the association with cardiac function and exercise capacity. Results MMP-2 was significantly associated with exercise capacity, ventilatory efficiency and left ventricular deceleration time, independently of age, sex, body surface area and NT-proBNP. Conclusion MMP-2 may provide new information in the clinical evaluation of adults with congenital heart disease.

Contains fulltext : 171611.pdf (Publisher’s version ) (Open Access) The prevalence of patients with congenital heart disease (CHD) has increased over the last century. As a result, the number of CHD patients presenting with late, postoperative tachyarrhythmias has increased as well. The aim of this review is to discuss the present knowledge on the mechanisms underlying both atrial and ventricular tachyarrhythmia in patients with CHD and the advantages and disadvantages of the currently available invasive treatment modalities.

Despite successful aortic coarctation (CoA) repair, systemic hypertension often recurs which may influence left ventricular (LV) function. We aimed to detect early LV dysfunction using LV global longitudinal strain (GLS) in adults with repaired CoA, and to identify associations with patient and echocardiographic characteristics. In this cross-sectional study, patients with repaired CoA and healthy controls were recruited prospectively. All subjects underwent echocardiography, ECG and blood sampling within 1 day. With speckle-tracking echocardiography, we assessed LV GLS on the apical four-, three- and two-chamber views. We included 150 subjects: 75 patients (57 % male, age 33.4 ± 12.8 years, age at repair 2.5 [IQR: 0.1–11.1] years) and 75 healthy controls of similar sex and age. LV GLS was lower in patients than in controls (−17.1 ± 2.3 vs. −20.2 ± 1.6 %, P

ObjectiveSoluble suppression of tumourigenicity-2 (sST2) is upregulated as response to myocardial stress and may be a potential biomarker for risk stratification in patients with adult congenital heart disease (ACHD). This study aimed to investigate the release of sST2 and its association with cardiovascular events in ACHD.MethodsIn this prospective cohort study, 602 consecutive patients with ACHD visiting the outpatient clinic were included (2011–2013). The association between sST2 and a primary composite endpoint of all-cause mortality, heart failure, hospitalisation, arrhythmia, thromboembolic events or cardiac interventions was investigated using multivariable Cox regression.ResultssST2 was measured in 590 (98%) patients (median age 33 [25–41] years, 42% women). After a median follow-up of 5.8 [IQR 5.1–6.2) years, 225 (38.5%) reached the primary endpoint. sST2 was significantly associated with the primary endpoint when adjusted for age, sex, creatinine and N terminal pro-B type brain natriuretic peptide (NT-proBNP) (HR per twofold higher sST2: 1.28, 95% CI 1.03 to 1.58, p=0.025). This association negated when adjusted for clinical variables and NT-proBNP (HR per twofold higher sST2: 1.19, 95% CI 0.96 to 1.48, p=0.106). Stratified analysis in complex ACHD did show a significant association between sST2 and the primary endpoint when adjusted for clinical variables and NT-proBNP (HR per twofold higher sST2: 1.31, 95% CI 1.01 to 1.69, p=0.043). Sex-specific analysis showed an association between sST2 and the primary endpoint in women (HR per twofold higher sST2 1.80, 95% CI 1.30 to 2.49, pConclusionssST2 is a promising novel biomarker in patients with ACHD, specifically in complex ACHD and women. Future research is warranted to elucidate sex-specific and diagnosis-specific differences.

To develop and validate a clinically useful risk prediction tool for patients with adult congenital heart disease (ACHD).A risk model was developed in a prospective cohort of 602 patients with moderate/complex ACHD who routinely visited the outpatient clinic of a tertiary care centre in the Netherlands (2011-2013). This model was externally validated in a retrospective cohort of 402 ACHD patients (Czech Republic, 2004-2013). The primary endpoint was the 4-year risk of death, heart failure, or arrhythmia, which occurred in 135 of 602 patients (22%). Model development was performed using multivariable logistic regression. Model performance was assessed with C-statistics and calibration plots. Of the 14 variables that were selected by an expert panel, the final prediction model included age (OR 1.02, 95%CI 1.00-1.03, p = 0.031), congenital diagnosis (OR 1.52, 95%CI 1.03-2.23, p = 0.034), NYHA class (OR 1.74, 95%CI 1.07-2.84, p = 0.026), cardiac medication (OR 2.27, 95%CI 1.56-3.31, p 0.001), re-intervention (OR 1.41, 95%CI 0.99-2.01, p = 0.060), BMI (OR 1.03, 95%CI 0.99-1.07, p = 0.123), and NT-proBNP (OR 1.63, 95%CI 1.45-1.84, p 0.001). Calibration-in-the-large was suboptimal, reflected by a lower observed event rate in the validation cohort (17%) than predicted (36%), likely explained by heterogeneity and different treatment strategies. The externally validated C-statistic was 0.78 (95%CI 0.72-0.83), indicating good discriminative ability.The proposed ACHD risk score combines six readily available clinical characteristics and NT-proBNP. This tool is easy to use and can aid in distinguishing high- and low-risk patients, which could further streamline counselling, location of care, and treatment in ACHD.

Background A single NT ‐pro BNP (N‐terminal pro‐B‐type natriuretic peptide) measurement is a strong prognostic factor in adult congenital heart disease. This study investigates NT ‐pro BNP profiles within patients with adult congenital heart disease and relates these to cardiovascular events. Methods and Results In this prospective cohort, 602 patients with adult congenital heart disease were enrolled at the outpatient clinic (years 2011–2013). NT ‐pro BNP was measured at study inclusion in 595 patients (median age 33 [ IQR 25–41] years, 58% male, 90% NYHA I) and at subsequent annual visits. The primary end point was defined as death, heart failure, hospitalization, arrhythmia, thromboembolic event, or cardiac intervention; the secondary end point as death or heart failure. Repeated measurements were analyzed using linear mixed models and joint models. During a median follow‐up of 4.4 [ IQR 3.8–4.8] years, a total of 2424 repeated measurements were collected. Average NT ‐pro BNP increase was 2.9 pmol/L the year before the primary end point (n=199, 34%) and 18.2 pmol/L before the secondary end point (n=58, 10%), compared with 0.3 pmol/L in patients who remained end point‐free ( P ‐value for difference in slope 0.006 and NT ‐pro BNP (>14 pmol/L, n=315, 53%), repeated measurements were associated with the primary end point ( HR per 2‐fold higher value 2.08; 95% CI 1.31–3.87; P HR 2.47; 95% CI 1.13–5.70; P =0.017), when adjusted for the baseline measurement. Conclusions NT ‐pro BNP increased before the occurrence of events, especially in patients who died or developed heart failure. Serial NT ‐pro BNP measurements could be of additional prognostic value in the annual follow‐up of patients with adult congenitive heart disease with an elevated NT ‐pro BNP .

Aims Stent placement as treatment for coarctation of the aorta (CoA) has become a more common choice in the last 20 years. Clinical results of CoA stenting are usually reported in small retrospective case series. This systematic review provides an overview of clinical experience with stenting for CoA. Methods and results A systematic review of the reports published between January 1990 and December 2014 after stenting a CoA was performed with a focus on relief of obstruction and lowering of blood pressure. Study and patient characteristics were extracted, as well as pre- and post-stenting aortic diameter in mm, systolic pressure gradient (SPG) and pre- and post-stenting systolic blood pressure (mmHg), periprocedural and follow-up complications. Forty-five articles met the inclusion criteria. Three outcomes were extracted from the articles - aortic diameter, systolic pressure gradient and blood pressure. Diameter increased from 6.4 mm (5.6, 7.3) to 15.1 mm (14.5, 15.7), pressure gradients decreased from 40 mmHg (35, 42) to 4 mmHg (3, 5) and systolic blood pressure decreased from 153 mmHg (148, 158) to 132 mmHg (127, 136). Stent migration was the most common periprocedural complication (2.4%), and mortality was low (0.4%). Conclusions Stenting is an effective treatment with regard to immediate relief of obstruction and direct lowering effect on blood pressure. However, there is a lack of evidence regarding late effectiveness concerning durable blood pressure lowering, and limited information on periprocedural and late complications. This observation calls for a systematic and longer prospective follow-up of patients after CoA stenting.

ObjectiveTo determine longitudinal changes in psychopathology in a cohort of patients 30–43 years after their first cardiac surgery for Congenital Heart Disease (CHD) in childhood, to compare outcomes of the 30- to 43-year follow-up with normative data, and to identify medical predictors for psychopathology.MethodsThis study is the third follow-up of this cohort. The first and second follow-ups of this same cohort were conducted in 1990 and 2001, respectively. At all three follow-ups, psychopathology was assessed with standardised, parallel questionnaires. In 2011, subjective health status was assessed by the Short Form-36. Medical predictor variables were derived from medical examinations and medical records.ResultsIn this third follow-up, a total of 252 patients participated. Of these, 152 patients participated in all three follow-ups. Over a 30-year period, proportions of patients showing psychopathology decreased significantly.At the 30- to 43-year follow-up, overall outcomes on psychopathology for the CHD sample were similar or even better compared with normative groups. Subjective health status was also better compared with normative data.No differences were found between cardiac diagnostic groups. Medical variables that predicted the course of psychopathology over time were as follows: the scar, as judged by the patient, results of the first cardiac surgery, and the number of hospitalisations.ConclusionsOver a 30-year period, psychopathology decreased in patients with CHD. Levels of psychopathology in these patients, who are now aged between 30 and 54 years, were comparable or even better than normative data.

Background: Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD).Objectives: The objective of this study was to invest...

Background: Adult congenital heart disease (ACHD) patients are at risk of late complications including arrhythmias, heart failure and sudden death. High-sensitive troponin-T (hs-TnT) is the standard for diagnosing acute coronary syndrome, but is also associated with cardiac function and prognosis in other cardiac diseases. We aimed to describe hs-TnT level in ACHD patients, and determine its relationship with cardiac function and other biomarkers. Methods: Consecutive ACHD patients, visiting the outpatient clinic, underwent echocardiography, exercise testing and venipuncture on the same day. Results: In total 587 patients were included (median age 33 [IQR 25-41] years, 58% male, 90% NYHA class I). hs-TnT was above the detection limit of 5 ng/L in 241 patients (41%), of whom 47 (8%) had hs-TnT levels above the 99th percentile of normal of 14 ng/L. hs-TnT levels were highest in patients with a systemic RV or pulmonary hypertension. Patients with normal or non-detectable hs-TnT were younger (32 [IQR 24-40] years) than patient with elevated hs-TnT (42 [IQR 36-60] years, p < 0.001). The prevalence of hs-TnT >= 14 ng/L was higher in patients with NYHA >= II (36%, p < 0.001), systemic systolic dysfunction (38%, p < 0.001), non-sinus rhythm (43%, p < 0.001) and elevated pulmonary pressures (39%, p < 0.001). hs-TnT was correlated with NT-proBNP (r = 0.400, p < 0.001). Conclusions: hs-TnT above the 99th percentile of normal is observed in a non-trivial portion of stable ACHD patients, especially in those with a systemic RV or elevated pulmonary pressures. Since this biomarker of myocardial damage is related to NT-proBNP and ventricular function, its potential predictive value in ACHD patients seems promising and further investigation of underlying mechanisms is warranted. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

We present a rare case of a 6-day old female neonate with a particularly exceptional coronary anatomy encompassing a fistula between the left main coronary artery and the coronary sinus with steal phenomenon, mandating surgical correction. The condition is extremely rare with an estimated incidence of about 0.1-0.2%. We operated this girl early on to prevent ischaemic events and pulmonary overflow with future heart failure. Further, we discuss possible aetiology and pathophysiological mechanisms of coronary artery fistulae and steal phenomena. Depending on specific anatomic characteristics, an interventional approach may be an alternative for surgical closure; however, this was not possible in our case.

Late complications of an atrial septal occluder device (ASO) are rare but may be serious. We report a case with extensive hemopericardium five years after ASO implantation most likely triggered by anticoagulant therapy. Although not surgically confirmed, indirect clues for erosion of the atrial wall by the device were the exclusion of other etiologies, lack of recurrence after pericardial drainage and withdrawal of anticoagulants. In addition, multimodality imaging using echocardiography, computed tomography, and cardiac magnetic resonance imaging were helpful to elucidate this unusual cause. Initiation of anticoagulant treatment in patients with an ASO should be carefully balanced and may warrant more frequent echocardiographic follow-up.

Item does not contain fulltext Fragmented QRS complexes (fQRS) on 12-lead electrocardiogram are known predictors of ventricular tachyarrhythmia (VTA) in patients with coronary artery disease. There is limited knowledge of the clinical implications of fQRS in patients with congenital heart defects (CHD). Aims of this study were to examine (1) the occurrence of fQRS in patients with various types of CHD and (2) whether fQRS is associated with development of VTA. This study was designed as retrospective case-control study. Patients with CHD with VTA were included and matched with control patients of the same age, gender, and CHD type. Clinical data and fQRS were analyzed and compared. The initial VTA episode developed in 139 patients with CHD at a mean age of 39 +/- 14 years. Compared with controls (n = 219, age 38 +/- 13 years), QRS duration was longer in patients with VTA (110 vs 100 ms; p

Aims: The Xeltis bioabsorbable pulmonary valved conduit (XPV), designed to guide functional restoration of patients' own tissue, is potentially more durable than current pulmonary bioprosthetic valves/valved conduits. The aim of this study was to assess the haemodynamic performance of the novel XPV implanted in an ovine model. Methods and results: The XPV was surgically implanted in adult sheep under general anaesthesia and cardiopulmonary bypass (XPV group, n= 20). Sheep that received a Hancock bioprosthetic pulmonary valved conduit served as a control group (HPV group, n = 3). Transthoracic echocardiograms from VARC-2 recommended time points at 3, 6, 9, 12, 18 and 24 months (XPV group) and at 3 and 6 months (HPV group) after the procedure were analysed in an independent core laboratory. The primary endpoint was favourable valved conduit performance, defined as peak systolic pressure gradient

Stent placement is commonly used to treat aortic coarctation. Although invasive angiography remains the gold standard, follow-up is often performed using computed tomography, which allows rapid, noninvasive assessment of the aorta and surrounding tissues. The goal of this pictorial essay is to provide a guide to the interpretation of these examinations. Normal and abnormal computed tomographic appearance of different stent types is shown along with reconstructions that can help assess stent integrity and the stent position in relation to the aortic wall and branches. Furthermore, imaging findings of complications including aortic wall injuries, restenosis, and intimal hyperplasia are depicted.

Aims Percutaneous pulmonary valve implantation (PPVI) is used to treat patients with dysfunctional pulmonary valve conduits. Short- and longer-term results from multiple trials have outlined haemodynamic improvements. Our aim was to report the long-term results, including quality of life, from a multicentre trial in Europe and Canada. Methods and results From October 2007 to April 2009, 71 patients (24 female; median age 19.0 [IQR: 14.0 to 25.0] years) were enrolled in a prospective cohort study. PPVI was performed successfully in 63 patients. At five-year follow-up four patients had died. Moderate and severe pulmonary regurgitation were completely resolved in all except one patient, who needed re-PPVI. Outflow tract obstruction improved significantly from a mean pressure gradient of 37.7±12.1 mmHg before PPVI to 17.3±9.7 mmHg at five-year follow-up; however, 11 patients needed treatment for restenosis. The EQ-5D quality of life utility index and visual analogue scale scores were both significantly improved six months post PPVI and remained so at five years. Conclusions Five-year results following PPVI demonstrate resolved moderate or severe pulmonary regurgitation, improved right ventricular outflow tract obstruction, and improved quality of life.

BACKGROUND: A 33-year-old adult with complex congenital heart disease and severe cyanosis presented to us with increasing symptoms of hyperviscosity, decreasing exercise tolerance and a thromboembolic complication. INVESTIGATION: Physical examination, laboratory investigations, ECG, transthoracic echocardiogram, cardiopulmonary exercise testing, angiography. DIAGNOSIS: Subvalvular and valvular pulmonary stenosis due to a calcified pulmonary valve. TREATMENT: Amiodarone, angiotensin-converting enzyme inhibitor, beta-blockers, percutaneous pulmonary balloon valvuloplasty, warfarin.

Aims To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair. Methods and results This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age

Aims The aim of this study was to assess the value of two-dimensional (2D) transthoracic simultaneous multiplane imaging (SMPI) in the evaluation of suitability for percutaneous atrial septal secundum defect (ASD) closure compared with the golden standard 2D transoesophageal echocardiography (TEE). Methods and results Twenty-nine patients with an ASD underwent both SMPI and TEE. Ten patients (34%) were male (age 41 +/- 18 years, range 20-74). SMPI assessment of ASD size and rims included xPlane and I-rotate modes. Rims were defined as suitable for ASD percutaneous closure using a cut-off value of 5 mm. There were no significant differences between SMPI in xPlane mode and TEE regarding the sizes of the anterior-posterior dimension (13.7 +/- 4.5 vs. 14.5 +/- 5.2 mm) and superior-inferior dimension (13.5 +/- 3.9 vs. 14.1 +/- 5.0 mm, respectively). Agreement for the aortic, atrioventricular, inferior, right upper pulmonary vein, and superior rims was 100, 100, 100, 96, and 96%, respectively. Conclusion The SMPI technique can reliably assess the dimensions and rim size of a secundum ASD for pre-interventional selection when compared with TEE and has thus the potential to replace TEE.

The objective of this study was to evaluate outcomes of endovascular treatment of aortic coarctation in adults.Clinical data and imaging studies of 93 consecutive patients treated at nine institutions from 1999 to 2015 were reviewed. We included newly diagnosed aortic coarctation (NCO), recurrent coarctation, and aneurysmal/pseudoaneurysmal degeneration (ANE) after prior open surgical repair (OSR) of coarctation. Primary end points were morbidity and mortality. Secondary end points were stent patency and freedom from reintervention.There were 54 (58%) male and 39 (42%) female patients with a mean age of 44 ± 17 years. Thirty-two patients had NCO (mean age, 48 ± 16 years) and 61 had endovascular reinterventions after prior OSR during childhood (mean, 30 ± 17 years after initial repair), including 50 patients (54%) with recurrent coarctation and 11 (12%) with ANE. Clinical presentation included asymptomatic in 31 patients (33%), difficult to control hypertension in 42 (45%), and lower extremity claudication in 20 (22%). Endovascular treatment was performed using balloon-expandable covered stents in 47 (51%) patients, stent grafts in 36 (39%) patients, balloon-expandable uncovered stents in 9 (10%) patients, and primary angioplasty in 1 (1%) patient. Mean lesion length and diameter were 64.5 ± 50.6 mm and 19.5 ± 6.7 mm, respectively. Mean systolic pressure gradient decreased from 24.0 ± 17.5 mm Hg to 4.4 ± 7.4 mm Hg after treatment (P .001). Complications occurred in nine (10%) patients, including aortic dissections in three (3%) patients and intraoperative ruptures in two patients; type IA endoleak, renal embolus, spinal headache, and access site hemorrhage occurred in one patient each. The aortic dissections and ruptures were treated successfully by deploying an additional covered stent proximal to the site of dissection or rupture. Two patients died within 30 days of the index procedure. After a mean follow-up of 3.2 ± 3.1 years, nearly all patients (98%) were clinically improved and all stents were patent. Reintervention was needed in 10 (11%) patients. Freedom from reintervention at 5 years was 85%. Two additional patients died during follow-up of coarctation-related causes, including rupture of an infected graft and visceral ischemia. Patient survival at 5 years was 89%.Endovascular repair is effective with an acceptable safety profile in the treatment of NCO and postsurgical complications of coarctation after initial OSR. Aortic rupture is an infrequent (2%) but devastating complication with high mortality. Balloon-expandable covered stents are preferred for NCO, whereas stent grafts are used for ANE. The rate of reinterventions is acceptable, with high procedural and long-term clinical success.

Background To investigate the overall sexual functioning and disease specific sexual problems in congenital heart disease (ConHD) patients, for both genders and different cardiac diagnostic groups, and compare these with Dutch normative data. Also disease specific sexual problems were investigated. Methods From a longitudinal cohort of patients, operated for ConHD between 1968 and 1980, 254 patients (median age: 40, 53.4% male) were included in this study: atrial septal defect (n=72), ventricular septal defect (n=71), pulmonary stenosis (n=30), tetralogy of Fallot (n=53) and transposition of the great arteries (n=28). Patients completed internationally validated, generic questionnaires and also disease specific instruments on sexual functioning. Results Patients showed a delay in starting sexual activities compared with peers. Females with ConHD scored significantly worse compared with normative data on all scales of sexual functioning, indicating a broad range of sexual problems and 15% showed clinical levels of sexual dysfunction. Of the males, 14% suffered from erectile dysfunction. Males with ConHD scored worse on erectile function, orgasmic function and satisfaction regarding their sexual life compared with normative data. No differences were found between the different cardiac diagnoses. The majority of patients reported disease specific worries and fears about the use of contraceptives, heredity, pregnancy and delivery. Patients indicated to have been suboptimally informed about sexuality in early adolescence. Conclusions This study shows that sexual functioning is impaired in adults with ConHD. Providing information to patients about sexuality, pregnancy, delivery and heredity should be improved, and given at young age.