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Data on the evolution of exercise capacity in adults with repaired coarctation of the aorta (CoA) are scarce. We aimed to investigate the evolution and change of measures of exercise capacity obtained by cardiopulmonary exercise testing (CPET) in adults with repaired CoA.Patients 16 years of age and older with CoA, who performed at least two maximal CPETs in our institution, were included in the study. The first and last available tests were used for comparative statistical analysis of common exercise variables.Sixty patients (43 men) performed serial maximal CPET. Mean age at first assessment was 30 ± 10 years (range 17-68). Mean time between first and last assessment was 3.5 years (range 1-7). Mean peak VOIn adults with repaired CoA, we found no significant change in peak VO

Heart failure (HF) is the primary cause of premature death in adult patients with congenital heart disease (ACHD). This study aims to describe the impact of a HF diagnosis on short-term prognosis and to investigate the added prognostic value of a HF diagnosis to the ACHD Anatomic and Physiological classification (ACHD-AP).This study included 3995 patients followed in a tertiary care center (last follow-upJanuary 1 2010). Survival curves were plotted and predictors of the primary endpoint (death, heart transplantation, or ventricular assist device [VAD]) were identified using Cox proportional hazard models and compared using Harrell's C-statistic.Mean age at baseline was 35.7±13.3 years. The prevalence of ACHD-HF was 6.4%. During a median follow up of 3.1 (IQR 2.1-3.6) years, 27.3% of ACHD-HF patients reached the primary endpoint, compared to 1.4% of ACHD patients without HF. Event-free survival was 78.3%, 61.9%, and 57.5% at 1, 3, and 5 years in ACHD-HF patients, compared to 99.3%, 98.3%, and 98.0% in ACHD patients without HF (p0.001). A HF diagnosis (HR 6.9; 95% CI 4.3-11.2) and the physiological classification (HR 2.6; 95% CI 1.9-3.7) were independently associated with the primary endpoint. The addition of HF to the ACHD-AP classification yielded a Harrell's C index of 0.8631, providing a significant improvement over the ACHD-AP classification alone (p=0.0003).The risk of mortality, and need for transplantation or VAD is increased in ACHD-HF patients. A HF diagnosis appears a valuable prognostic marker in addition to the ACHD-AP classification.

INTRODUCTION: Despite right ventricular (RV) dysfunction being a major concern in Senning patients, long-term follow-up data is lacking. This study aimed (1) at evaluating regional (base-mid-apex) RV and left ventricular (LV) function using Colour-Doppler myocardial imaging over a 15-year follow-up period and (2) at comparing results with matched controls. METHODS: For the longitudinal analysis (2004-2019), we compared systolic and diastolic function in 10 Senning patients. For the cross-sectional analysis, we compared the subaortic RV (sRV) of Senning patients with the RV and LV of matched controls and the subpulmonary LV (spLV) of Senning patients with the LV of matched controls. RESULTS: The longitudinal analysis of sRV function showed a significant decrease in apical peak systolic strain (-17 ± 7% vs -12 ± 4%; p = 0.025) and apical peak systolic strain rate (-1.1 ± 0.3s-1 vs -0.8 ± 0.4s-1; p = 0.012). spLV function showed a significant decrease in peak systolic velocity (mid; p = 0.013 and apex; p = 0.011) and peak systolic strain rate (mid; p = 0.048). The cross-sectional analysis revealed significant lower values for basal, mid and apical peak systolic velocity, peak systolic strain rate, peak systolic strain of the sRV of Senning patients when compared to both LV and RV of matched controls (all p

Aims Early recognition of adverse remodelling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to short-term clinical evolution in this population. Methods and results Thirty-three patients (76% male) with sRV (atrial switch repair for D-transposition of the great arteries and congenitally corrected transposition of the great arteries) underwent detailed phenotyping including exercise cardiac magnetic resonance and were followed over mean follow-up time of 3 years. Mean age was 40 ± 8 (range 26–57) years at latest follow-up. Adverse outcome was a composite of heart failure (HF) and tachyarrhythmia. Descriptive statistics and univariate cox regression analyses were performed. When compared with baseline: (i) most patients remained in New York Heart Association functional class I (76%), (ii) the degree of severity of the systemic atrioventricular valve regurgitation rose, and (iii) more electrical instability was documented at latest follow-up. Six (18%) of a total of 9 events were counted as first cardiovascular events (9% HF and 9% arrhythmia). NT-proBNP, oxygen pulse, left ventricle end-diastolic volume index (LVEDVi), and stroke volume index (SVi) of the subpulmonary left ventricle (LV) both in rest and at peak exercise were significantly associated with the first cardiovascular event. Conclusion NT-proBNP was by far the best prognostic marker for clinical outcome. Adverse remodelling with increase of LVEDVi and SVi of the subpulmonary LV at rest and during exercise were associated with worse clinical outcome. We theorize that remodelling of the subpulmonary ventricle might be an early sign of a failing sRV circulation.

Background Aortic dilatation and pregnancy are major concerns in women with aortopathy (AOP). This single-centre retrospective analysis focuses on the evolution of aortic diameters during and after pregnancy in women with Marfan syndrome (MS), Turner syndrome (TS) and bicuspid aortic valve (BAV) aortopathy. Methods and results Thirty-eight women who had one or more single pregnancies were included. The ascending aorta was measured during pregnancy and postpartum. During pregnancy, a significant increase of diameters of the sinus aortae (median 1.4 mm; [−1.3; 3.8]) and ascending aorta (median 2.1 mm; [0.0; 4.0]) was noted. Systemic hypertension gives dilation of the aorta, but it did not influence the overall trajectory during pregnancy. Conclusion Significant aortic dilatation is noted during pregnancy in women with underlying AOP, even persisting in the long term. Pre-existing systemic hypertension is associated with larger aortic diameters prior to pregnancy. More research on a larger study population however is needed.

Background Heart failure hospitalizations in adult patients with congenital heart disease (ACHD) are increasing and are associated with higher healthcare-related costs. We aimed to evaluate factors that are associated with repeated heart failure hospitalizations and whether heart failure hospitalizations are related to adverse outcome in ACHD patients with heart failure (ACHD-HF). Methods Out of 3995 patients under active follow-up in our institution (last visit >2010), 256 patients (mean age 49.5±16.7 years) had ACHD-HF and were included in the study. Medical records were reviewed, including heart failure hospitalization prior and after study inclusion. A combined endpoint of death, ventricular assist device and transplantation was defined. Results Overall, 136 ACHD-HF patients (53%) had a prior heart failure hospitalization. Over a mean follow-up of 2.5±2.3 years, 44 patients (17%) had repeated heart failure hospitalizations. Of these, 31 patients (12%) had 1; 9 patients (4%) 2 and 4 patients (2%) 3 repeated heart failure hospitalizations. Patients with repeated heart failure hospitalizations had higher NYHA class (p=0.031), were more likely to have end-organ dysfunction (p=0.025) and more likely to have a prior heart failure hospitalization (p Conclusions Heart failure hospitalizations are frequent in ACHD-HF. End-organ dysfunction appears to be a strong determinant of repeated heart failure hospitalizations. Event-rate is high in patients with ACHD-HF, but repeated heart failure hospitalizations were not related to outcome in this short-term follow-up study. Funding Acknowledgement Type of funding sources: None. Table 1.1Table 1.2

ispartof: CONGENITAL HEART DISEASE vol:15 issue:2 pages:69-78 status: published

Funding Acknowledgements Type of funding sources: Other. Main funding source(s): This research received project funding by KU Leuven Background – Early recognition of adverse remodeling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to short-term clinical evolution in this population. Purpose - We aimed assessing short-term clinical evolution and early prognostic markers of cardiac complications in adults with sRV (atrial switch repair for D-transposition of the great arteries (D-TGA) and congenitally corrected transposition of the great arteries (ccTGA)) based on detailed phenotyping. Methods– Thirty-three patients with sRV underwent detailed phenotyping including exercise CMR. Adverse outcome was a composite of heart failure episode and tachyarrhythmia. Descriptive statistics and univariate cox regression analyses were performed. Results - Thirty-three patients (76% male) with sRV were followed over mean follow-up time of 3 years. Mean age was 40 ± 8 (range 26-57) years at latest follow-up. When compared to baseline, (I) most patients remained in NYHA functional class I (76%), (II) the degree of severity of the SAVV regurgitation rose and (III) more electrical instability was documented at latest follow-up. Six (18%) of a total of nine events were counted as first cardiovascular events (9% heart failure, 9% arrhythmia). NTproBNP (HR 11.02 (95%CI 1.296-93.662), p= 0.028), oxygen pulse (HR 1.202 (95% CI 1.012-1.428), p = 0.037), left ventricle end diastolic volume index (LVEDVi) in rest (HR 1.046 (95% CI 1.002-1.092), p = 0.041) and during exercise (HR 1.035 (95% CI 1.002-1.069), p = 0.038), stroke volume index (SVi) of the subpulmonary left ventricle (LV) in rest (HR 1.154 (95% CI 1.005-1.322), p = 0.038) and at peak exercise (HR 1.065 (95% CI 1.007-1.125), p = 0.026) were significantly associated with the first cardiovascular event (Figure 1A and B). Conclusion – NTproBNP was by far the best prognostic marker for clinical outcome. Adverse remodelling with increase of LVEDVi and SVi of the subpulmonary LV at rest and during exercise were associated with worse clinical outcome. We theorize that remodeling of the subpulmonary ventricle might be an early sign of a failing sRV circulation (Figure 2).

AIMS: Information on the prevalence, outcome and factors associated with heart failure in patients with adult congenital heart disease (CHD) (ACHD-HF) is lacking. We aimed at assessing the prevalence and outcome of ACHD-HF, the variables associated with ACHD-HF, and the differences between major anatomical/pathophysiological ACHD subgroups. METHODS AND RESULTS: We included 3905 patients (age 35.4 ± 13.2 years) under active follow-up in our institution (last visit >2010). Outcome of ACHD-HF cases was compared with sex- and age-matched cases. Univariable and multivariable binary logistic regression with ACHD-HF diagnosis as a dependent variable was performed. Overall prevalence of ACHD-HF was 6.4% (mean age 49.5 ± 16.7 years), but was higher in patients with cyanotic CHD (41%), Fontan circulation (30%), and a systemic right ventricle (25%). All-cause mortality was higher in ACHD-HF cases when compared with controls (mortality rate ratio 4.67 (2.36-9.27); P = 0.0001). In multivariable logistic regression analysis, age at latest follow-up [per 10 years; odds ratio (OR) 1.52; 95% confidence interval (CI) 1.31-1.77], infective endocarditis (OR 4.11; 95%CI 1.80-9.38), history of atrial arrhythmia (OR 3.52; 95%CI 2.17-5.74), pacemaker implantation (OR 2.66; 95% CI 1.50-4.72), end-organ dysfunction (OR 2.41; 95% CI 1.03-5.63), New York Heart Association class (OR 9.28; 95% CI 6.04-14.25), heart rate (per 10 bpm; OR 1.27; 95% CI 1.08-1.50), ventricular dysfunction (OR 3.62; 95% CI 2.54-5.17), and pulmonary hypertension severity (OR 1.66; 95% CI 1.21-2.30) were independently related to the presence of ACHD-HF. Some variables (age, atrial arrhythmia, pacemaker, New York Heart Association, and ventricular dysfunction) were related to ACHD-HF in all anatomical/physiological subgroups, whereas others were not. CONCLUSIONS: ACHD-HF is prevalent especially in complex CHD and is associated with poor prognosis. Our data provide insight in the factors related to ACHD-HF including differences between specific anatomical and physiological subgroups. ispartof: ESC HEART FAILURE vol:8 issue:4 pages:2940-2950 ispartof: location:England status: published

Background Left-to-right shunt in patients with sinus venosus septal defect (SVSD) may affect resistive (pulmonary vascular resistance – PVR) and elastic (pulmonary artery compliance-PAC) properties of the pulmonary arteries. This study aimed at evaluating (1) changes in hemodynamics over time and (2) the relationship between PVR and PAC in a large cohort of patients with SVSD. Methods Data of 136 patients with SVSD (median age at diagnosis 14 (IQR 5–48) years, 47% male) were included. Catheterization data was available in 87 patients. Pressures were measured and cardiac output evaluated using the Fick principle. PVR (mean pulmonary artery pressure (mPAP)-wedge pressure/cardiac output) and PAC (stroke volume/pulse pressure) were calculated and indexed for body surface area. Results Ninety percent had abnormal pulmonary venous connection. Median shunt ratio was 2.5 (IQR 2.0–2.9). Repair was performed in 128 (94%) at a median age of 13 (IQR 5–43) years. During a median follow-up time of 31 (IQR 17–55) years, 12 (9%) patients died, 13 (10%) developed heart failure, 19 (14%) atrial arrhythmia, 6 (4%) sick sinus syndrome and 7 (5%) required pacemaker implantation. PVR indexed and PAC indexed was 3.5 (IQR 2.4–7.5) WU·m2 and 1.8 (IQR 1.3–2.5) mL/mmHg·m2. There was an inverse, hyperbolic relationship between PVR and PAC with an RC time of 6.6 (IQR 4.4–8.9) sec. When comparing patients with age at catheterization 50 years, shunt ratio (2.5±0.6 vs 2.5±0.8 vs 2.5±0.6; p=0.836) and PVR indexed (2.9 (2.3–3.4) vs 3.1 (2.0–9.0) vs 6.6 (3.6–8.8) WU·m2; p=0.132) were not statistically different, whereas mPAP (19 (16–22) vs 21 (16–24) vs 28 (22–34) mmHg; p=0.002); wedge (6 (4–8) vs 13 (8–16)vs 14 (10–19) mmHg; p Conclusion SVSD is associated with late morbidity and mortality. In SVSD, PVR and PAC are inversely related. Over time, the changes in elastic resistance (PAC or pulsatile load) are more profound than changes in resistive (PVR – steady load). Figure 1 Funding Acknowledgement Type of funding source: None

OBJECTIVES: This study aimed to describe the safety and efficacy of covered stents in patients with coarctation of aorta (CoA) for immediate and long-term follow-up. BACKGROUND: Covered stents are increasingly being used in (re)CoA, mainly to reduce the risk of aortic wall injuries (AWI). However, limited data are available on intermediate and long-term outcome. METHODS: In 89 patients (67.4% male) with a mean age of 23.9 ± 15.8 (min max range 5.1-71.6) years were 102 covered stents implanted (January 2003 - December 2017). Short-term pre/post-implant hemodynamics and angiographic data were reported. Changes in blood pressure, the use of antihypertensive drugs and complications were recorded during follow-up. RESULTS: The procedural success rate was 100%. The mean invasive ascending-to-descending aorta systolic gradient under general anaesthesia decreased from 25 ± 16 mmHg to 4 ± 7 mmHg (p

BACKGROUND: Pregnancy in women with congenital heart disease (CHD) is associated with increased risk for maternal cardiac complications. Several risk stratification models are used to predict adverse cardiac outcome in women with CHD who become pregnant. This study was set up as an exploratory study to provide a head-to-head comparison of the 4 most commonly used models: CARPREG, CARPREG II and ZAHARA risk scores and mWHO risk classification. METHODS AND RESULTS: We randomly selected 100 women from the database of paediatric and congenital heart disease of the University Hospitals Leuven. Individual pregnancy risk scores were retrospectively calculated and summarized in a weighted average risk for each risk stratification model. To evaluate accuracy of each model, the weighted average risk was plotted against the actual observed number of "cardiac events" as defined in the respective risk models. Maternal adverse cardiac events occurred in 8% of our study population. Weighted average risks were plotted versus the observed number of events for each model: 10.1% versus 4.0% for CARPREG, 8.6% versus 5.0% for CARPREG II, 11.1% versus 8.0% for ZAHARA and 12.4% versus 8.0% for the mWHO classification. CONCLUSION: All risk models overestimated maternal cardiac risk. The ZAHARA risk model appeared to be a closer reflection of maternal risk in our cohort of CHD patients. More research on a larger study population is needed. ispartof: INTERNATIONAL JOURNAL OF CARDIOLOGY vol:323 pages:54-60 ispartof: location:Netherlands status: published

Heart failure is an increasing reason for hospitalization and the leading cause of death in patients with adult congenital heart disease (ACHD). Recently, the European Society of Cardiology and the American Heart Association published consensus documents on the management of chronic heart failure in ACHD patients. However, little data and/or guidelines are available for the management of (sub)acute heart failure. The ACHD population is heterogeneous by definition and often has complex underlying anatomy, which could pose a challenge to the physician confronted with the ACHD patient in (sub)acute heart failure. Recognizing the underlying anatomy and awareness of the possible complications related would result in better treatment, avoid unnecessary delays, and improve outcomes of the ACHD patient with (sub)acute heart failure. This review focuses on the management of (sub)acute heart failure in ACHD with specific attention to lesion-specific issues.

Objectives There is conflicting evidence concerning the role of right ventricular (RV) systolic dysfunction in the long-term clinical outcome after cardiac resynchronization therapy (CRT). Therefore we aimed to assess evolution of RV systolic function during CRT, covariates associated with its improvement, and its impact on outcome. Methods and results All CRT device implantations (Jan 2009-Dec 2011) in our institution were reviewed. Records of 69 patients (25% female, mean age 62.8 ± 9.2 years, mean left ventricular (LV) ejection fraction 27 ± 8%) were analyzed. Baseline RV fractional area change (FAC) 35% was present in 37 patients (54%). At one year, 24 of them (65%) improved in RV FAC. LV remodeling and mitral regurgitation were significantly associated with the likelihood of RV FAC improvement (OR 4.80, 95% CI 1.13-20.46, P = 0.034 and OR 0.32, 95% CI 0.12-0.89, P = 0.029, respectively). The composite endpoint of death or heart transplantation occurred in 23 patients (33%) over a mean follow-up of 2.8 ± 1.4 years. RV FAC at one year (HR 0.90, 95% CI 0.86-0.94, P .001) was, independently of NYHA class and LV remodeling, associated with clinical outcome. Conclusions RV systolic function might improve during CRT. This seems mainly due to changed left-sided hemodynamics and LV remodeling. Good RV systolic function is independently related with better outcome.

Background: Outcome after cardiopulmonary resuscitation (CPR) in patients with underlying congenital heart disease is uncertain. This study aimed at evaluating outcome after CPR in patients with underlying congenital heart disease, factors related to worse outcome after CPR and whether survivors of sudden cardiac death (SCD) have a worse outcome when compared to an age, gender and disease-matched control population. Methods: Between 1984 and 2015, all patients with congenital heart disease who received in or out-of-hospital CPR were identified from the database of congenital heart disease from the University Hospitals Leuven. Postoperative and neonatal (Results: Thirty-eight patients (66% men; median age 25 years (interquartile range 9–40); 68% out-of-hospital) were identified, of which 27 (66%) survived the event. The main cause of SCD was ventricular tachycardia or fibrillation ( n=21). Heart defect complexity (odds ratio (OR) 5.1; 95% confidence interval (CI) 1.2–21.9; P=0.027), pulmonary hypertension (OR 13.8; 95% CI 2.1–89.5; P=0.006) and time to return of spontaneous circulation (OR 1.1; 95% CI 1.0–1.1; P=0.046) were related to worse outcome. Survivors of SCD had a worse prognosis when compared to an age, gender and disease-matched control group (5-year survival 76% vs. 98%; P=0.002). Conclusions: The complexity of underlying heart defect, pulmonary hypertension and time to return of spontaneous circulation are related to worse outcome in the case of CPR. Survivors of SCD have a worse outcome when compared to matched controls, indicating the need for adequate implantable cardioverter defibrillator indication assessment and for stringent follow-up of patients with worsening haemodynamics.

BACKGROUND: Mortality after cardiac surgery for Ebstein's anomaly ranges from 2.5% to 31%. Independent predictors for mortality and morbidity remain poorly defined because of the low incidence of this congenital anomaly. To identify potentially modifiable factors, this retrospective study investigates the prognostic value of perioperative variables for mortality and morbidity. METHODS: We reviewed the charts of 171 patients with Ebstein's anomaly who were at one point in follow-up at our center. Only patients who underwent cardiac surgery for this anomaly were included. The primary endpoint was a composite of mortality or rehospitalization for cardiac reasons within 1 year of surgery. Logistic regression and Cox regression models were used to study the predictive value of various variables. RESULTS: We identified 32 patients (median age 12 years; range 7 days to 70 years) who underwent a total of 49 surgical procedures for Ebstein's anomaly at our institution between November 1987 and March 2015. The following variables were significantly associated with the primary outcome: increased severity of tricuspid valve stenosis (odds ratio 2.089; 95% confidence interval 1.175 to 3.713) and right ventricular dysfunction (1.826; 1.109 to 3.006), partial corrective surgery (versus corrective surgery) (6.709; 1.436 to 31.344), occurrence of major postoperative complications (5.460; 1.419 to 21.008), and increased length of stay in the intensive care unit (1.051; 1.010 to 1.093). A better outcome was observed with the use of intraoperative cardioplegic arrest (0.185; 0.063 to 0.550), atrial septal defect closure during surgery, and longer duration of surgery (0.991; 0.984 to 0.998). CONCLUSION: Several patient-specific characteristics and perioperative characteristics were associated with a poorer outcome after cardiac surgery for Ebstein's anomaly. The outcome seems to be primarily determined by the severity of the valve dysfunction and right ventricular performance, with only a minor role for perioperative surgical or anesthetic technical determinants. ispartof: HEART SURGERY FORUM vol:23 issue:2 pages:E193-E199 ispartof: location:United States status: published

OnBehalf Ana Moya, 1994 Introduction Although the development of right ventricular (RV) dysfunction in patients who underwent an atrial switch procedure is a major concern, long-term follow-up data on the evolution of myocardial function over time, especially using deformation myocardial imaging, is still lacking. Purpose This study aimed (1) at evaluating regional (base-mid-apex) RV and left ventricular (LV) function using Colour-Doppler myocardial imaging over a 15-year follow-up period (longitudinal analysis) and (2) at comparing results with age- and gender-matched controls (cross-sectional analysis). Methods For the longitudinal analysis, we compared systolic and diastolic function between 2004 and 2019 in 10 Senning patients. For the cross-sectional analysis, we compared the subaortic RV (sRV) of Senning patients with the RV and LV of matched controls and the subpulmonary LV (spLV) of Senning patients with the LV of matched controls. Results The longitudinal analysis (2004-2019) of sRV function showed a significant decrease in peak systolic strain (-16.9 ± 7.1% vs -12.0 ± 4.0%; P = 0.045), peak systolic strain rate (-1.1 ± 0.3s-1 vs -0.8 ± 0.4s-1; P = 0.003) and peak early diastolic velocity (-2.0 ± 1.2 cm/s vs -1.0 ± 0.5 cm/s; P = 0.036) at the apex. spLV function showed a significant decrease in peak systolic velocity (mid: 5.6 ± 1.8 cm/s vs 3.7 ± 1.1 cm/s; P = 0.013 and apex: 5.0 ± 1.7 cm/s vs 2.1 ± 1.2 cm/s; P = 0.011) and peak systolic strain rate (mid: -1.7 ± 0.5s-1 vs -1.0 ± 0.4s-1; P = 0.048). The cross-sectional analysis revealed significant lower values for peak systolic velocity, peak systolic strain rate, peak systolic strain at all myocardial regions of the sRV when compared to both LV and RV of matched controls (all P Conclusion Our study shows little change in systolic and diastolic sRV function over a 15-year period, except in the apical region. Interestingly, there was a decline of spLV systolic function which may be of clinical value. On the other hand, when compared to age- and gender-matched controls, both the sRV and spLV of Senning patients exhibit significantly decreased measurements of longitudinal systolic and diastolic function.

Background The development of an aortic pseudoaneurysm is a rather rare but potentially fatal complication after cardiac surgery for aortic valve and aorta disease. If a pseudoaneurysm is left untreated, it carries a substantial risk of rupture, thrombosis with subsequently systemic embolization, and compression of the surrounding structures. Case summary We describe a case of a transcatheter repair of a more complex and uncommon pseudoaneurysm following aortic valve replacement in a patient with a history of arterial switch repair for transposition of the great arteries. The pseudoaneurysm originated from the aortic wall and connected to the left ventricular outflow tract (LVOT). The connection to LVOT was closed with a duct occluder, the neck to the aortic wall with an atrial septal defect occluder. After 1 month, the complete pseudoaneurysm was successfully thrombosed. Discussion Percutaneous closure of a complex pseudoaneurysm after arterial switch repair is feasible and safe. However, lifelong follow-up is needed to determine the late results after transcatheter interventions.

ObjectiveTo evaluate if development of fragmented QRS (fQRS) complexes, a marker of inhomogeneous ventricular activation due to myocardial fibrosis, is associated with adverse outcome in adults after Mustard/Senning repair for d-transposition of the great arteries (d-TGA).MethodsAdults with atrial switch repair for d-TGA were selected from the database of a tertiary care hospital. Exclusion criteria were systemic right ventricular (RV) assist device or heart transplantation (HTx) before the age of 16, or fQRS already present at first visit to the Adult Congenital Heart Disease clinic. A blinded expert reader retrospectively analysed all available ECGs after the age of 16 for the presence of fQRS. The appearance of fQRS was modelled for each patient as a time-dependent variable. Cox regression was performed to assess the relationship between covariates and the composite endpoint of cardiovascular mortality, HTx or systemic RV assist device.ResultsRecords of 89 patients (34% female, 42% Mustard repair) were analysed. At latest follow-up, fQRS was noted in 26 patients (29%). Over a median follow-up time of 16.9 (IQR 12.6–22.9) years, the composite endpoint occurred in nine patients (10%). In multivariable Cox analysis, appearance of fQRS (HR 14.11; 95% CI 1.42 to 140.12) and development of severe RV dysfunction (HR 11.36; 95% CI 2.08 to 62.17) were significantly associated with the composite endpoint.ConclusionsAppearance of fQRS complexes on a 12-lead ECG is associated with adverse outcome in adults after atrial switch repair for d-TGA. In this population, fQRS detection might be a promising and easily implementable tool to identify patients at risk for adverse events.

Discharge from follow-up after closure of isolated congenital shunt lesions in childhood was common practice in the past. The aim of the present study was to recall these patients to evaluate their current status.Patients included in the database of pediatric and congenital heart disease of our tertiary center with repaired secundum atrial septal defect (ASD) or ventricular septal defect (VSD) before the age of 18years, and discharged from follow-up, were invited for clinical and echocardiographic check-up.Forty-six ASD patients (age 30±7years, 37% male) responded. Median age at ASD repair was 6 (IQR 4-8) years. All but one functioned in NYHA class I. Eight (17%) patients reported palpitations. No patient developed pulmonary hypertension (PH). Right ventricular (RV) dilatation was present in 7 (15%). RV fractional area change (FAC) was35% in 7 (15%), TAPSE17mm in 12 (26%). Forty-seven VSD patients (age 34 (IQR 29-40) years, 57% male) participated. Median age at VSD repair was 4 (IQR 1-5) years. Six (13%) patients functioned in NYHA class II. Seventeen (36%) patients reported palpitations. Four (9%) patients presented PH. Left ventricular dilatation was present in 4 (9%), RV dilatation in 6 (13%). RV FAC was35% in 7 (15%), TAPSE17mm in 17 (36%). Seven (15%) patients had dilated ascending aorta.Patients with closure of isolated secundum-type ASD in childhood do well, but some have persistent RV dilatation and dysfunction. By contrast, more patients after VSD closure were symptomatic and presented with RV dilatation and dysfunction, PH, and a dilated ascending aorta.

Background Children from mothers with systemic lupus erythematosus are frequently born with congenital heart block. This study aimed at evaluating long-term outcome because long-term data are scarce. Methods In the database of pediatric and congenital heart disease (University Hospitals Leuven), 19 children from systemic lupus erythematosus mothers and who were born with or developed atrioventricular block were identified. All records were reviewed for disease course and outcome. Results Median follow-up time was 7 years (interquartile ranges [IQR] 4.5–13 years). One child had no heart block at birth and developed only a first-degree block during follow-up. One had a second-degree heart block and developed a complete heart block. Seventeen patients (89%) were born with a complete heart block. Seventeen patients (89%) needed a definitive pacemaker. In all, epicardial leads were used at first implantation. Eighty-two percent received their pacemaker in the first year of life. The first battery had a median lifetime of 5 years (IQR 3.5–5 years), the second 6 years (IQR 4.5–6.3 years), and the third 5 years (IQR 5–6 years). Note that 47% of patients needed a lead replacement due to lead problems. Only one pericardial tamponade after pacemaker implantation. No device or lead infections occurred. The left ventricular systolic function at latest follow-up was normal for all. No patients died. Conclusion In children with heart block born from systemic lupus erythematosus mothers, an early need for pacemaker implantation was documented. The overall battery life was acceptable, but there was a high need for lead replacement. Complication rate was low. Late outcome was good.

Background Reduced ventricular function and decreased exercise capacity are widespread in adults with complete transposition of the great arteries after atrial switch ( TGA ‐Mustard/Senning) and congenitally corrected TGA (cc TGA ). Advanced imaging techniques may help to better phenotype these patients and evaluate exercise cardiac response. Methods and Results Thirty‐three adults with a systemic right ventricle (70% TGA ‐Mustard/Senning, 37±9 years of age, 24% female, 94% New York Heart Association class I‐ II ) underwent echocardiogram, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging at rest and during a 4‐stage free‐breathing bicycle test. They were compared with 12 healthy controls (39±10 years of age, 25% female, all New York Heart Association class I). TGA ‐Mustard/Senning patients had a higher global circumferential strain (−15.8±3.6 versus −11.2±5.2%, P =0.008) when compared with cc TGA , whereas global longitudinal strain and systemic right ventricle contractility during exercise were similar in both groups. Septal extracellular volume ( ECV ) in cc TGA was significantly higher than in TGA ‐Mustard/Senning (30.2±2.0 versus 27.1±2.7%, P =0.005). During exercise, TGA ‐Mustard/Senning had a fall in end‐diastolic volume and stroke volume (11% and 8%, respectively; both P ≤0.002), whereas cc TGA could increase their stroke volume in the same way as healthy controls. Because of a greater heart rate reserve in TGA ‐Mustard/Senning ( P for interaction=0.010), cardiac index and peak oxygen uptake were similar between both patient groups. Conclusions Caution should be exercised when evaluating pooled analyses of systemic right ventricle patients, given the differences in myocardial contraction pattern, septal extracellular volume, and the exercise response of TGA ‐Mustard/Senning versus cc TGA patients. Longitudinal follow‐up will determine whether abnormal exercise cardiac response is a marker of earlier failure.

AIMS: The aim of the study was to report up to 11 years of follow-up after Melody valve implantation in the pulmonary position. METHODS AND RESULTS: This was a single-institution non-randomised prospective observational study of all Melody valves in the pulmonary position after discharge between 2006 and 2017 (n=188). Mean age was 19.4 years (SD 13.2). The indication was stenosis (45%), regurgitation (33%) and mixed (22%). Pre-stenting was performed in all except the initial four patients. In stenotic lesions the peak gradient was 36 mmHg PIG (SD 12.0) after 11 years and in regurgitant lesions the maximal regurgitation was 2/4. Stent fractures were observed in 8.6%; only one grade III fracture required redo PPVI. Surgical removal was carried out in seven (3.7%), redo PPVI in five (2.7%). Endocarditis was diagnosed in 19 (10.2%) patients at a median of 2.3 years (0.7-8.8) after Melody implantation. Three were surgically removed early because of persistent infection, 16 were sterilised; six required replacement (three surgical, three redo PPVI). There were no valve- or procedure-related deaths. CONCLUSIONS: The Melody valve shows a good preserved leaflet function up to 11 years after implantation. The main reason for graft failure was endocarditis, although in half of those patients no reintervention was needed. After pre-stenting, stent fractures led very exceptionally to reintervention. ispartof: EUROINTERVENTION vol:14 issue:9 pages:E988-E994 ispartof: location:France status: published

As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2]. The scientific adult CHD (ACHD) community has been working hard to identify variables related to worse outcomes, modifying those where possible in order to improve survival. Indeed, survival in adults with CHD has increased, but consequently, on top of CHD-related complications, patients are increasingly exposed to the standard cardiovascular risk factors. Therefore, a program for lifelong coaching on health behavior and life style management becomes indispensable. More emerging is that a substantial number of patients, in particular those with complex heart defects, will eventually end up in a stage with hardly any medical or interventional options left. Our healthcare provision has to be prepared to organize care for this specific group of patients who will die prematurely and require the timely development and establishment of advanced care planning. Advanced care planning should preferentially be set-up in expert CHD centers. The long-lasting relationship in ACHD care with healthcare providers offers an excellent basis with regards to prognosis, advanced care planning and end-of-life issues. ispartof: INTERNATIONAL JOURNAL OF CARDIOLOGY vol:279 pages:57-61 ispartof: location:Netherlands status: published

Background Melody valved stents (Medtronic Inc, Minneapolis, Minnesota, USA) have become a very competitive therapeutic option for pulmonary valve replacement in patients with congenital heart disease. After adequate prestenting of the right ventricular outflow tract (RVOT) Melody valved stents have a good medium term functional result but are exposed to infective endocarditis (IE). Patients and methods Retrospective study of tertiary centre Congenital Heart Disease database; to compare incidence of IE in three different types of valved conduits in RVOT: Melody valved stent, cryopreserved homograft (European Homograft Bank) and Contegra graft (Medtronic Inc). Results Between 1989 and 2013, 738 conduits were implanted in 677 patients. 107 Melody valved stents were implanted in 107 patients; IE occurred in 8 (7.5%) patients during a follow-up of 2.0 years (IQR 2.4 years, range 0.3–7.8 years). 577 Homografts were implanted in 517 patients; IE occurred in 14 patients (2.4%) during a median follow-up of 6.5 years (IQR 9.2 years; range 0.1–23.7 years). Finally, 54 Contegra grafts were implanted in 53 patients; 11 patients (20.4%) had IE during a follow-up of 8.8 years (IQR 7.7 years; range 0.2–3.5 years). Survival free of IE by Kaplan–Meier for homografts was 98.7% at 5 years and 97.3% at 10 years; for Contegra 87.8% at 5 years and 77.3% at 10 years and for Melody 84.9% at 5 years (log-rank test; p Conclusions The Contegra conduit and Melody valved stents have a significantly higher incidence of IE than homografts. IE is a significant threat for long-term conduit function.

BACKGROUND: To evaluate the relationship between right ventricular (RV) systolic dysfunction at rest and reduced exercise capacity in patients with a systemic RV (sRV). METHODS: All patients with congenitally corrected transposition of the great arteries (ccTGA) or complete TGA after atrial switch (TGA-Mustard/Senning) followed in our institution between July 2011 and September 2017 who underwent cardiac imaging within a six-month time period of cardiopulmonary exercise testing (CPET) were analyzed. We assessed sRV systolic function with TAPSE and fractional area change on echocardiogram and, if possible, with ejection fraction, global longitudinal and circumferential strain on cardiac magnetic resonance (CMR) imaging. RESULTS: We studied 105 patients with an sRV (median age 34 [IQR 28-42] years, 29% ccTGA and 71% TGA-Mustard/Senning) of which 39% had either a pacemaker (n = 17), Eisenmenger physiology (n = 6), severe systemic atrioventricular valve regurgitation (n = 14), or peak exercise arterial oxygen saturation

When elevated pulmonary artery pressure (PAP) is assessed by echocardiography, right heart morphology is always considered. The goal of this study was to evaluate how right heart geometry changes with increasing right ventricular pressure load.Data from patients undergoing transthoracic echocardiography with subsequent right heart catheterization within a time period of 6 months were retrospectively analysed. First, Spearman-rho coefficients between mean PAP and right heart parameters were calculated. Second, the population was divided into tertiles according to mean PAP and Kruskal-Wallis variance analysis between variables was performed.Fifty-four patients (23 female, median age 77 years, IQ range 63-83) were selected. Mean PAP (median 27 mmHg, IQ range 24-36), right atrial (RA) dilatation (median 1, IQ range 0-2), tricuspid insufficiency (TI) severity (median 1.5, IQ range 0-2) and right ventricular (RV) dilatation (median 0, IQ range 0) were included. Significant correlations with mean PAP were found for RA dilatation (rho = 0.380; P = 0.005) and TI severity (rho = 0.294; P = 0.032). No correlation with RV dilatation could be shown (rho = 0.241; P = 0.081). Across the tertiles [(1) mean PAP 25 mmHg; (2) mean PAP 26-30 mmHg; (3) mean PAPor = 31 mmHg)], variance analysis showed a significant increase in RA dilatation (P = 0.009) and TI severity (P = 0.040). No change in RV dilatation across groups was observed (P = 0.216).RA dilatation and TI severity significantly increase with increasing RV pressure load. No increase in RV dilatation was observed in the studied population. Hence, absence of RV dilatation does not exclude the presence of elevated PAP.

INTRODUCTION: Data on long term function of the Melody valve are scarce. Patients and methods: single institution; results of percutaneous pulmonary valve implantation (PPVI) from 2006 to 2014. The function of the valved conduit was analyzed by Doppler echocardiography. Annual Chest X-ray after implant and permanent screening for events (e.g. Endocarditis). RESULTS: 112 Melody valves were implanted in 111 patients; mean age 19.3 years (4.5-81.6). No pre-stenting of the RVOT was performed (n = 4) at first. In the next 107 patients pre-stenting was always performed. In 82 patients 1 pre-stent, 18 patients 2, in 6 patients 3 stents and 1 patient 4 stents were used. The Melody stent was dilated up to 24 mm (n = 4), 22 mm (n = 72), 20 mm (n = 28) and 18 mm (n = 6). When stenotic, the Doppler gradient reduced from 67.0 mm Hg (SD 13.9) to 18.9 mm Hg (SD 10.4) (p

Introduction Percutaneous pulmonary valve implantation is now considered feasible and safe. "Native" right ventricular outflow tract (RVOT), small diameter conduits (

OBJECTIVES Although the effects of pulmonary regurgitation after tetralogy of Fallot repair are detrimental, timing of pulmonary valve replacement (PVR) is unclear. Our goal was to evaluate the midterm efficacy and safety of early PVR. METHODS Patients with tetralogy of Fallot who underwent repair from 1962 to 2015 were included from the local database. Statistical analyses compared patients who underwent early PVR (age ≤16 years), late PVR and no PVR. The timing of the intervention was compared for efficacy-all-cause mortality and the combined end-point of all-cause mortality, ventricular tachycardia and defibrillator implantation-and for safety-the combined end-point of 1-year postoperative mortality after PVR, endocarditis and reintervention. Echocardiographic and electrocardiographic data at the last follow-up examination were compared across the 3 groups. RESULTS Two hundred seventy-three patients (age 21 ± 5 years; 52% female) were included. The mean follow-up was 24 (95% confidence interval 22.7-26.2) years; the observed median was 21 years (interquartile range 11-31). No significant difference in survival was found between the early PVR (n = 106; 39%), the late PVR (n = 47; 17%) and the no PVR groups (n = 120; 44%) (P = 0.990). No significant difference in the combined efficacy end-point was noted between patients who underwent early PVR compared with patients who underwent late PVR (P = 0.247). Worse event-free survival for the 3-point safety end-point was observed after early PVR (P

Congenital heart defects (CHD) are associated with the recurrent 10q22q23 deletion syndrome and with partially overlapping distal 10q23.2.q23.31 microdeletions. We report on a de novo intragenic deletion of the BMPR1A gene in a normally developing adolescent boy with short stature, delayed puberty, facial dysmorphism and an atrioventricular septal defect. Based on this finding, complemented with computational prioritization data and molecular evidence in literature, the critical region for CHD on 10q23 can be downsized to a single gene, BMPR1A. Although loss-of-function mutations in BMPR1A typically result in juvenile polyposis syndrome, none of the patients with the typical 10q22q23 microdeletion syndrome, comprising this gene, were reported to have juvenile polyposis thus far. We reason that, even in the absence of juvenile polyposis syndrome, sequencing and copy number analysis of BMPR1A should be considered in patients with (atrioventricular) septal defects, especially when associated with facial dysmorphism and anomalous growth.

Several patients with trisomy 21 developed the Eisenmenger syndrome (ES) because the underlying congenital heart defect was not corrected. However, little is known about their prognosis.This study aimed at (1) identifying risk factors for worse prognosis in ES patients, and (2) evaluating whether outcome of ES patients with trisomy 21 differs from ES patients without trisomy 21.Data on all Eisenmenger patients in follow-up at the paediatric and adult congenital heart disease clinic of the University Hospitals Leuven were collected for retrospective analysis. Regression analysis was performed where applicable and survival rate was compared between patients with and without trisomy 21.One hundred thirty-four patients (mean age at latest follow-up 33.2 +/- 13.6 years, 41.8% male, 44.8% trisomy 21) were included in the study. Complex lesions, right heart failure, impaired renal function, lower transcutaneous saturation and lower body mass index were predictive of impaired outcome. Mean survival of the global ES group was 44.9 +/- 2.2 years. However, long-term survival of trisomy 21 patients was not statistically different from patients without trisomy 21 (mean survival 44.5 +/- 2.6 years vs 44.5 +/- 2.9 years, respectively, P = 0.80, log rank test).Long-term survival is markedly reduced in Eisenmenger patients. Complex lesions, right heart failure, impaired renal function, lower transcutaneous saturation and lower body mass index were related to worse prognosis. However, survival of trisomy 21 patients did not differ from patients without trisomy 21.

Background: Doubly committed ventricular septal defect (dcVSD) is the least common type of VSD. Because published studies are rather scarce, this study aimed at evaluating the midterm outcome of dcVSDs. Methods: The records of all patients registered in the database of Paediatric and Congenital Cardiology, University Hospitals Leuven, with a dcVSD at 16 years of age were reviewed. Clinical, electrocardiographic and transthoracic echocardiographic changes from baseline, defined as of the age of 16 years, until the latest follow-up were compared. Results: Thirty-three patients (20 males, median age 26 years, interquartile range 12) were followed for a median time of 7.9 years (interquartile range 9.8, time range 2–25.9). No deaths occurred. In 15 patients (45%), the defect remained patent at baseline. During follow-up, two spontaneous closures (13%) occurred. Eighteen patients (55%) required closure before the age of 16 years. Five (28%) needed reoperation. In the dcVSD closure group, left ventricular ejection fraction decreased from 69 ± 12 to 61 ± 6% (p = 0.028). No significant changes in pulmonary arterial hypertension were noticed. Conclusions: Patients with persistently patent dcVSD remained nearly event free during follow-up. Event-free survival after dcVSD closure was markedly lower. These patients developed reduced left ventricular function and had a high risk of reintervention.

A study on Bearded Reedling Panurus biarmicus feeding habits in combination with a sample-wise breeding bird survey of the marshland zones of the Dutch wetland Oostvaardersplassen shows clear-cut spatial differences in densities and habitat use. The more mature stands of Reed Phragmites australis constitute the main nesting area, while the more open, younger stands, particularly when inundated and grazed by moulting Greylag Geese, provide the main feeding areas. Arthropod, and especially Chironomid, densities were generally higher in grazed Reed stands and frequent feeding flights were carried out by parent birds between grazed and ungrazed parts of the area during chick raising. Chironomids also proved to constitute the main prey items brought to the nests. Apart from higher prey densities in grazed stands, better detectability as well as better accessibility of the lower vegetation layers are also likely to contribute to the habitat preference of insect-feeding Bearded Reedlings. When in winter the birds shift to Reed seeds, patches with higher seed index hold higher densities of feeding birds. When seed index drops below a certain level, density of birds is low and independent of seed index. The highest seed production is associated with rejuvenated Reed stands, recovering from previous grazing. Bearded Reedlings thus highly depend on the early successional stages of Reed stands. Temporal and spatial habitat diversification is mediated by changing water levels and rejuvenation caused by grazing geese. A number of other marshland bird species depend on this type of vegetation, and wetland management should therefore aim at favouring the natural processes governing Reed succession.

Background - More and more children. who suffered from Kawasaki disease in childhood. reach coday adulthood. The future perspectives of these patients are not yet well defined, therefore. we wanted to determine mid-term outcome of our Kawasaki patients. Methodology - All patients with the diagnosis of Kawasaki disease were selected from the data­ base of paediatric and congenital cardiology of our hospital. The records were reviewed for disease characteristics and follow-up data. Descriptive statistics were performed. Results - Thirty-five patients were included (18 boys. median age at diagnosis 1.9years. range from 0.4 to 12.2 years). In 94% of the cases, the leh coronary artery was affected, whereas in 58% the right coronary artery was involved in the disease process. In three patients the leh ventricular ejection fraction worsened below 50%. One patient underwent a balloon dilatation and stenting of a coronary artery, and in another patient inotropic support was needed. Aspirin was given in all. sandoglobulin in thirty patients; in 10% of the cases corticosteroids were ad ministered. The active disease process terminated aher a median of 0.5 months, ranging from 0.1 to 2.0 months.The patient cohort was followed for a median of 4 years (range from 0.1 co 17.7 years). During this follow-up time, no reoccurrences, no significant arrhythmias, and no deaths occurred. In all patients, the leh ventricular ejection fraction remained normal or normalized. However. at the latest follow-up. 43% was still treated with a low dose of aspirin. Conclusions - The mid-term outcome of patients who suffered from Kawasaki disease during child­ hood is excellent. However. almost half of them were treated with oral aspirin during follow-up.

OBJECTIVE Intra-cardiac echocardiography (ICE) is used to guide percutaneous interventions on the atrial septum. However, ICE catheters are expensive. We questioned the impact of the use of ICE catheters on hospitalization costs for patent foramen ovale (PFO) or atrial septal defect (ASD) closure. METHODS AND RESULTS Patients, scheduled for atrial septal closure, were randomly selected to use the AcuNav catheter (Biosense Webster Inc, Diamond Bar, CA, US) on top of the standard procedure (three or two days of hospitalization, procedure with transoesophageal echocardiography (TEE) and general anaesthesia). The AcuNav catheter was provided for free and the total hospitalization cost for each patient was calculated by verification of the bills, sent to the patient and the national health insurance. This was compared with a fictive hospitalization cost when ICE alone would have been used (three or two days of hospitalization, procedure without TEE, and local anaesthesia). Feasibility and safety were also evaluated. Three PFOs and two ASDs were successfully closed (3F/2M, age 55 +/- 12 years). The total hospitalization cost for a standard closing procedure was EUR 9345 +/- 132 and EUR 9303 +/- 132 for three and two days of hospitalization, respectively. With a free ICE catheter and no general anaesthesia, hospitalization cost lowered to EUR 8464 +/- 131 and EUR 8422 +/-131, respectively. Cost saving would be EUR 881 +/- 3, but the price of a single-use ICE catheter varies between EUR 2000 and 2500. In all patients, adequate images were obtained and no complications related to the ICE catheter occurred. CONCLUSIONS Single-use ICE catheters remain expensive in percutaneous ASD or PFO closure. However, general anaesthesia might be avoided, which could open a discussion on cost savings.

The number of adolescents and young adults with congenital heart defects, including ventricular septal defect (VSD), increases continuously. We evaluated the mid-term outcome of small and unclosed perimembranous VSDs (pmVSDs). All patients with a known unrepaired pmVSD at 16 years of age were selected from our database. The clinical, electrocardiographic, and echocardiographic changes between baseline and the latest follow-up examination were compared. A total of 220 patients (119 males, median age 18 years, interquartile range 7) could be included. During a median follow-up of 6 years (interquartile range 4, range 38), 2 patients died (1%; 1 from sudden death and 1 from end-stage heart failure). Endocarditis occurred in 8 patients (4%). One patient required pacemaker implantation (0.5%) and one required implantable cardioverter-defibrillator implantation (1%). Fifteen patients (7%) required a closing procedure. In 8 patients (4%), the pmVSD closed spontaneously. In the remaining 203 patients (93%), the QRS morphology changed in 5% and 1% lost sinus rhythm (p = 0.0001 and p = 0.015, respectively). The left ventricular ejection fraction and stroke volume index increased from 62 + or - 7% to 67 + or - 8% and from 41 + or - 11 to 44 + or - 15 ml/m(2) (p = 0.0001 and p = 0.035, respectively), the end-systolic diameter decreased, and the end-diastolic diameter did not change. Finally, patients with an open pmVSD developed more pulmonary arterial hypertension during follow-up (from 3% to 9%, p = 0.002). In conclusion, mid-term follow-up of adolescents and young adults with a small and unrepaired pmVSD was not uneventful. Some patients required intervention, but in others, spontaneous closure occurred. Electrocardiographic and structural changes were noticed, for which the clinical significance needs to be determined.

Children from mothers with systemic lupus erythematosus are frequently born with congenital heart block. This study aimed at evaluating long-term outcome because long-term data are scarce.In the database of pediatric and congenital heart disease (University Hospitals Leuven), 19 children from systemic lupus erythematosus mothers and who were born with or developed atrioventricular block were identified. All records were reviewed for disease course and outcome.Median follow-up time was 7 years (interquartile ranges [IQR] 4.5-13 years). One child had no heart block at birth and developed only a first-degree block during follow-up. One had a second-degree heart block and developed a complete heart block. Seventeen patients (89%) were born with a complete heart block. Seventeen patients (89%) needed a definitive pacemaker. In all, epicardial leads were used at first implantation. Eighty-two percent received their pacemaker in the first year of life. The first battery had a median lifetime of 5 years (IQR 3.5-5 years), the second 6 years (IQR 4.5-6.3 years), and the third 5 years (IQR 5-6 years). Note that 47% of patients needed a lead replacement due to lead problems. Only one pericardial tamponade after pacemaker implantation. No device or lead infections occurred. The left ventricular systolic function at latest follow-up was normal for all. No patients died.In children with heart block born from systemic lupus erythematosus mothers, an early need for pacemaker implantation was documented. The overall battery life was acceptable, but there was a high need for lead replacement. Complication rate was low. Late outcome was good.

BACKGROUND: Pulmonary outflow tract obstruction (POTO) reduces systemic atrioventricular valve (SAVV) regurgitation severity in congenitally corrected transposition of the great arteries (ccTGA). Therefore, pulmonary artery banding is proposed as a palliative intervention. We aimed to investigate the effect of native or surgically induced POTO on event-free survival, defined as the composite of all-cause mortality, heart transplantation, or congestive heart failure (CHF). METHODS AND RESULTS: Patients with ccTGA (n=62; median age 27.5 (IQR 18.4-39.4) years; 39% with POTO) were selected from the Adult Congenital Heart Disease database of a tertiary hospital. At first visit, SAVV regurgitation ≥ 3/4, systemic RV dysfunction ≥ moderate, and CHF were present in 26%, 26%, and 15% of patients, respectively. Over a mean follow-up time of 10.1 ± 6.1 years, all-cause mortality, rate of heart transplantation, and CHF were 18%, 8% and 40%, respectively. SAVV regurgitation (HR: 1.99; 95% CI: 1.01-3.92; P=0.048) and systemic RV dysfunction severity (HR: 1.89; 95% CI: 1.05-3.37; P=0.033) were associated with the composite endpoint, independently of age at baseline, POTO, Ebstein-like malformation, and systemic RV dilatation. Patients with POTO had lower risk for developing SAVV regurgitation ≥ 3/4 (HR: 0.18; 95% CI: 0.05-0.58; P=0.004) and moderate systemic RV dysfunction (HR: 0.34; 95% CI: 0.15-0.78; P=0.011). When POTO was present, the mean progression-free interval for the composite endpoint increased from 11.2 to 18.1 years (P=0.035). CONCLUSIONS: POTO is associated with an improved event-free survival in adults with ccTGA. publisher: Elsevier articletitle: Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries journaltitle: International Journal of Cardiology articlelink: http://dx.doi.org/10.1016/j.ijcard.2015.05.142 content_type: article copyright: Copyright © 2015 Elsevier Ireland Ltd. All rights reserved. ispartof: International Journal Of Cardiology vol:196 pages:1-6 ispartof: location:London, UK status: published

Empirical evidence indicates that patients with congenital cardiac anomalies may be prone to developing coronary heart disease. Although primary prevention of ischaemic heart disease in patients with congenital heart defects is important, data on the prevalence of cardiovascular risk factors in these patients are not available. The aims of this study are therefore to describe the prevalence of risk factors for coronary heart disease in a large sample of adults with congenital cardiac anomalies, and compare this with the prevalence in the general population.A retrospective analysis of computerized patient records.At our outpatient clinic, all patients are examined by an advanced practice nurse and a congenital heart disease cardiologist. Data on smoking behaviour, sports participation, blood pressure, body mass index, and the diagnosis of diabetes are recorded systematically. Data on the general population were derived from national health surveys.In a 4-year period, we collected data on 1976 individual patients. Male patients had a significantly higher prevalence of smoking and elevated blood pressure, whereas women were less engaged in sports activities and were more often obese. In comparison with the general population, our patients reported less smoking and more participation in sports, but presented more often with hypertension or diabetes. Only 20.4% of men and 21.0% of women have a fully heart-healthy lifestyle, as they presented without any risk factor.A substantial number of patients had one or more cardiovascular risk factors. Therefore, primary prevention by strengthening educational efforts becomes critically relevant in patients with congenital heart disease, to avoid the additional burden of coronary events in this growing population of patients.

Background The number of adult patients with surgical repaired congenital heart defects increases continuously. We were interested to compare late outcome after partial and complete atrioventricular defect repair (pAVSD and cAVSD, respectively) and to determine the most important reason for re-intervention. Methods All patients older than 16 years, who underwent partial or complete atrioventricular defect repair, were selected from the database. The medical files were reviewed for descriptive statistics. Kaplan Meier analysis was used to determine event free survival for both groups. Log rank testing was performed where applicable. Results One hundred thirty-eight patients were included. Two patients in each group with early post-operative mortality were excluded (pAVSD: 33/30 male/female, mean age 35.9 +/- 15.6 years; cAVSD: 27/ 44 male/female, mean age 25.7 +/- 11.9 years). Fourteen pAVSD-patients and 23 cAVSD-patients needed a surgical re-intervention: in two and 13 patients, respectively, a mitral valve repair was performed, and in five and one patient, respectively, a mechanical valve was replaced. Eight cAVSD-patients and 10 pAVSD-patients developed atrial arrhythmias. Seven patients (3 cAVSD and 4 pAVSD) received a pacemaker. Median event-free survival time was significantly shorter in the cAVSD-group (22.9 (95% CI 15.4-30.5) years) when compared to the pAVSD-group (34.0 (95% CI 20.1-47.9) years) (Log rank testing, P=0.017). Conclusion Late outcome was characterized by a longer event free follow-up time of pAVSD-patients when compared to cAVSD-patients. The most important reason for re-intervention in both groups was mitral valve regurgitation. However, atrial arrhythmias and conduction disorders were not uncommon late after atrioventricular septal defect repair.

In adult patients with mild-to-moderate pulmonary valve (PV) stenosis, exercise capacity and haemodynamics have not been extensively studied, although regular exercise is recommended. Therefore, we aimed to assess exercise capacity to study the increase in PV gradient during exercise and to evaluate the impact of this increased pressure load on the RV.Nineteen patients (8 female; 29±6.4 years) with isolated mild-to-moderate PV stenosis and no prior cardiac interventions were consecutively enrolled from the outpatient clinic of adult congenital heart disease. All patients underwent cardiopulmonary exercise testing, transthoracic echocardiography and bicycle stress echocardiography. Results for exercise testing were compared with age-matched and gender-matched control patients.In the studied population, resting heart rate (89±11 vs 75±14 bpm; p=0.001), peak power (199±66 vs 263±68 W; p=0.006); peak VO2 (31.2±9.9 vs 39±7.4 mL/kg/min; p=0.011); oxygen uptake efficiency slope (2430±913 vs 3292±943(mL/min)/(L/min); p=0.007) and VE/VCO2 slope (26.8±5.2 vs 22.6±4.3; p=0.01) differed significantly from controls. A linear increase of peak PV gradient with increasing flow was observed in the pooled dataset (Pearson's R=0.947; p0.0001) and slopes identical as for control patients were obtained for the oxygen pulse-workload relationship. Right heart morphology and function were preserved in the studied patients.Patients with mild-to-moderate PV stenosis have decreased exercise capacity. A linear increase in PV gradient with flow suggests a fixed valve area throughout the exercise. Although systolic RV pressure load increases during exercise, good ventricular performance was observed without signs of functional or morphological changes of the right heart.NCT01444222.

The Fontan procedure has become the procedure of choice for patients with one functional ventricle. Although perioperative mortality has decreased, late failure of the Fontan circulation remains a major concern. We aimed at (i) describing Fontan patient characteristics and (ii) identifying simple risk factors for outcome.Seventy-three patients (median age 23 y (IQR 19-29 y), 60.3% male) were selected from the database of congenital heart defects. Followup data were collected. The primary end point was composed by death, resuscitation, or heart transplantation.The most frequently occurring defect was tricuspid atresia (41.1%). Twenty-five (34.2%) and 48 (65.8%) patients received an intra- and extracardiac conduit, respectively. Ten patients reached the primary end point (13.7%) after a median follow-up time of 16 years (IQR 14-19 y). NYHA classification (OR 63.0; 95% Cl 6.7-592.4; Por =0.001), atrioventricular-valve regurgitation (OR 10.6; 95% Cl: 1.2-94.1; P = 0.034), ventricular function (OR 4.8; 95% Cl 1.7-13.7; P = 0.003), oxygen saturation (OR 0.7; 95% Cl 0.1-1.0; P = 0.002) and the presence (OR 8.6; 95% Cl 1.6-45.2; P = 0.011) or history of supraventricular arrhythmia (OR 6.7; 95% CI: 1.3-35.0; P = 0.025), all parameters gathered at the latest follow-up, were associated with outcome. An association was also found with the presence of an intracardiac conduit (OR 5.8; 95% Cl 1.4-25.1; P = 0.018), higher age at Fontan procedure (OR 1.2; 95% Cl 1.0-1.3; P = 0.007) and male gender (OR 0.2; 95% Cl 0.1-1.0; P = 0.047).Complications were not uncommon later after Fontan surgery. Several demographic and procedure-related data were associated with adverse outcome. Interestingly, the strongest correlation was found with clinical and basic echocardiographic characteristics at the latest follow-up.