Perioperative Risk Factors for Mortality in Patients Undergoing Surgery for Ebstein's Anomaly at a Single Institution: A Retrospective Analysis.

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.

Management of acute heart failure in adult patients with congenital heart disease.

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.

Prognostic significance of improvement in right ventricular systolic function during cardiac resynchronization therapy.

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.

Long-Term Follow-Up of Children with Heart Block Born from Mothers with Systemic Lupus Erythematosus: A Retrospective Study from the Database Pediatric and Congenital Heart Disease in University Hospitals Leuven.

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.

Right heart morphology in elevated pulmonary artery pressure: relationship between echocardiographic and right heart catheterization data.

BACKGROUND: 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. SUBJECTS AND METHODS: 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. RESULTS: 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 PAP > or = 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). CONCLUSIONS: 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.

Predicting outcome after Fontan palliation: a single-centre experience, using simple clinical variables.

BACKGROUND: 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. METHODS: 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. RESULTS: 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; P < or =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). CONCLUSIONS: 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.

Biomarkers in transposition of the great arteries after arterial switch operation: A pilot trial with deep phenotyping.

INTRODUCTION: Transposition of the great arteries (TGA) is a cyanotic congenital heart defect for which the arterial switch operation (ASO) is the preferred surgical repair. This study wanted to investigate whether a panel of biomarkers could identify morphologic as well as hemodynamic changes obtained by cardiac magnetic resonance (CMR). METHODS: Forty-four adult patients were included. Blood samples were collected to measure a broad range of biomarkers (galectin-3, ST2, GDF-15, PINP, ICTP, PIIINP, IGF-1, NT-proBNP, and hs-Tn). CMR was performed at rest and during exercise to assess cardiac function and morphology. Explorative statistics were performed between biomarker levels and CMR findings. RESULTS: All patients were asymptomatic. While galectin-3, GDF-15, and NT-proBNP levels were within normal ranges, increased ST2, PINP, PIIINP, and ICTP levels were found in 20.5%, 34.1%, 45.5%, and 27.3% of patients, respectively. Moreover, 3 and 2 patients, respectively, showed elevated IGF-1 and hs-Tn levels. Although the ejection fraction of both ventricles was within normal limits, impaired cardiac reserve was found in 20 and 25% of patients for left and right ventricle, respectively. CMR revealed no evidence of diffuse interstitial fibrosis, while 4 patients showed focal ischemic scarring. However, no significant associations between serum biomarkers and CMR data could be detected. CONCLUSION: The results suggest that in asymptomatic ASO-repaired TGA patients serum level biomarkers are elevated and that this increase is not associated with morphological changes nor with a decreased cardiac reserve. Further study with larger sample sizes is required to draw conclusions with greater confidence.

Aorta pathology and pregnancy-related risks in adult congenital cardiac disease: does the aorta dilate during pregnancy?

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.

Off-label use of percutaneous pulmonary valved stents in the right ventricular outflow tract: time to rewrite the label?

INTRODUCTION: Percutaneous pulmonary valve implantation is now considered feasible and safe. "Native" right ventricular outflow tract (RVOT), small diameter conduits (<16 mm) and relatively large RVOT with a dynamic outflow aneurysm are currently considered off-label uses. Extending indications creates concerns of safety, ethics, reimbursement, and liability. AIM OF STUDY: To report the safety and feasibility of off-label application of percutaneous pulmonary valve implantation. DESIGN: Retrospective analysis of prospectively collected data. PATIENTS AND METHODS: Off-label indications: conduit-free RVOT or patients with an existing but undersized conduit. RESULTS: Twenty-one Melody® valves and two Sapien® valves were successfully implanted in 23 patients (16.9 years; range 6.1-80.5 years). In 22 patients, prestenting was performed 4.8 months (range 0-69.2) before valve implantation (15 covered and 13 bare stents). Stent endothelial ingrowth was allowed for at least 2 months prior to implantation of the percutaneous valve if stent stability or sealing by the covering was presumed to be insufficient. Group 1 patients (n = 8) had a "conduit-free" RVOT after transannular/infundibular patch and after prestenting underwent percutaneous pulmonary valve implantation (PPVI), with a final RVOT diameter of 21.5 mm (range 16-26 mm). Group 2 patients consisted of two elderly patients with pulmonary valve stenosis and severe RVOT calcifications. Group 3 (n = 13) had an existing conduit (nominal 15.9 ± 3.2 mm; range 10-20 mm). The conduit was augmented from 14.7 ± 3.5 to 20 ± 1.6 mm with PPVI. The RVOT preparation and valve implantations were uneventful. CONCLUSIONS: PPVI is safe and feasible in selected patients with an off-label indication. Creating an adequate "landing zone" by prestenting makes the procedure safe and predictable. Updating the indications for PPVI should be considered.

Short-term results of serial cardiopulmonary exercise testing in adults with repaired coarctation of the aorta.

BACKGROUND: 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. METHODS: 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. RESULTS: 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 VO2 was 85.6 ± 20.4% of the predicted value at the initial test, and 87.0 ± 20.5% at the final test (p = 0.294). There were no significant differences in the mean values of oxygen pulse, VO2 at anaerobic threshold, systolic and diastolic blood pressures and peak heart rate between the two assessments. There was a slightly higher VE/VCO2 slope at the final test (p = 0.047). Higher age and Borg scale were found to be related with a decline in percent-predicted peak VO2 from initial to final assessment. CONCLUSION: In adults with repaired CoA, we found no significant change in peak VO2 during a mean follow-up of 3.5 years, yet a small increase in VE/VCO2 slope was observed. Higher age was predictive for a decline in percent-predicted peak VO2, starting in the third decade of life.

Short-term Prognostic Value of Heart Failure Diagnosis in a Contemporary Cohort of Patients With Adult Congenital Heart Disease.

BACKGROUND: Heart failure (HF) is the primary cause of premature death in adult congenital heart disease (ACHD). This study aimed to describe the impact of a HF diagnosis on short-term prognosis and to investigate the added prognostic value of an HF diagnosis to the ACHD Anatomic and Physiologic classification (ACHD-AP). METHODS: This study included 3995 patients followed in a tertiary care centre (last follow-up after January 1, 2010). Survival curves were plotted, and predictors of the primary end point (death, heart transplantation, or ventricular assist device [VAD]) were identified with the use of Cox proportional hazard models and compared with the use of Harrell's C-statistic. RESULTS: 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 years (IQR 2.1-3.6 years), 27.3% of ACHD-HF patients reached the primary end point, compared with 1.4% of ACHD patients without HF. Event-free survivals were 78.3%, 61.9%, and 57.5% at 1, 3, and 5 years in ACHD-HF patients, compared with 99.3%, 98.3%, and 98.0% in ACHD patients without HF (P < 0.001). An HF diagnosis (HR 6.9, 95% CI 4.3-11.2) and the physiologic classification (HR 2.6, 95% CI 1.9-3.7) were independently associated with the primary end point. 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). CONCLUSIONS: The risk of mortality, transplantation, or VAD is increased in ACHD-HF patients. An HF diagnosis appears to be a valuable prognostic marker in addition to the ACHD-AP classification.

Left atrial strain in patients after arterial switch operation for transposition of the great arteries.

BACKGROUND: Left atrial (LA) strain, comprising LA reservoir, conduit and contractile function could add mechanistic information of patients after arterial switch operation (ASO) for transposition of the great arteries (TGA). ASO patients might have abnormal ventriculoarterial coupling, which makes them vulnerable to left ventricle (LV) dysfunction and results in reduced exercise capacity. This explorative study aimed to evaluate the relation between LA strain, atrial size, ventricular function, and exercise data obtained by cardiopulmonary exercise testing (CPET). METHODS: In a cohort of 44 patients (71% male, mean age 25 ± 4 years) LA strain was measured using transthoracic speckle-tracking echocardiography. Further assessment involved standard echocardiography, CPET evaluation, and blood sampling. LA strain values were compared to normal values. Correlations were calculated. Regression analysis with all strain variables to the CPET data was performed. RESULTS: LA reservoir, conduit and contractile strain were normal in 30%, 89% and 50% of the patients, respectively. LA reservoir/contractile strain correlated to LV ejection fraction (ρ 0.310/-0.159, respectively) and LA reservoir/conduit strain correlated to the LA volume index (ρ 0.336/-0.357, respectively). None of the individual LA strain parameters were associated with the CPET variables. In multivariate regression analysis, LA contractile strain was significantly associated with the percentage of predicted maximal heart rate (ß - 2.555). CONCLUSIONS: These data suggest that in TGA patients after ASO repair LA strain is impaired and correlates with LA size and LV function. However, impaired LA strain wasn't associated with the standard CPET parameters. As such, clinical significance needs to be further unravelled.

Visualization of the intracavitary blood flow in systemic ventricles of Fontan patients by contrast echocardiography using particle image velocimetry.

BACKGROUND: Flow patterns in univentricular hearts may have clinical value. Therefore, it is our objective to asses and characterize vortex flow patterns with Fontan circulation in comparison with healthy controls. METHODS: Twenty-three patients (8 Fontan and 15 normal patients) underwent echocardiography with intravenous contrast agent (Sonovue®) administration. Dedicated software was used to perform particle image velocimetry (PIV) and to visualize intracavitary flow in the systemic ventricles of the patients. Vortex parameters including vortex depth, length, width, and sphericity index were measured. Vortex pulsatility parameters including relative strength, vortex relative strength, and vortex pulsation correlation were also measured. RESULTS: The data from this study show that it is feasible to perform particle velocimetry in Fontan patients. Vortex length (VL) was significantly lower (0.51 ± 0.09 vs 0.65 ± 0.12, P = 0.010) and vortex width (VW) (0.32 ± 0.06 vs 0.27 ± 0.04, p = 0.014), vortex pulsation correlation (VPC) (0.26 ± 0.25 vs -0.22 ± 0.87, p = 0.05) were significantly higher in Fontan patients. Sphericity index (SI) (1.66 ± 0.48 vs 2.42 ± 0.62, p = 0.005), relative strength (RS) (0.77 ± 0.33 vs 1.90 ± 0.47, p = 0.0001), vortex relative strength (VRS) (0.18 ± 0.13 vs 0.43 ± 0.14, p = 0.0001) were significantly lower in the Fontan patients group. CONCLUSIONS: PIV using contrast echocardiography is feasible in Fontan patients. Fontan patients had aberrant flow patterns as compared to normal hearts in terms of position, shape and sphericity of the main vortices. The vortex from the Fontan group was consistently shorter, wider and rounder than in controls. Whether vortex characteristics are related with clinical outcome is subject to further investigation.

Adverse functional remodelling of the subpulmonary left ventricle in patients with a systemic right ventricle is associated with clinical outcome.

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.

Doubly committed ventricular septal defect: single-centre experience and midterm follow-up.

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.

Influence of percutaneous transcatheter closure of an atrial septal defect on the atrioventricular conduction.

OBJECTIVE: This study aimed at determining the effect of percutaneous ASD closure on the AV conduction. Percutaneous closure of ostium secundum atrial septal defects (ASDs) has been widely accepted and has become the standard treatment of this malformation. However, some complications have been reported. Because of the close proximity between the discs of the device and the atrioventricular (AV) node, atrioventricular conduction disturbances (AVCD) are a likely complication. METHODS AND RESULTS: From the database of congenital heart disease, 46 patients (mean age 54 +/- 12 y) who underwent percutaneous ASD closure and 46 patients who underwent a percutaneous patent foramen ovale (PFO) closure were matched according to age, gender and follow-up time. Demographic, clinical, biochemical, and ECG variables were compared. Before closure, the PR interval in the ASD group was significantly longer than in the PFO group (169 +/- 22 ms versus 158 +/- 25 ms, P= 0.031), which persisted after a mean follow-up time of 4.5 years (180 +/- 39 ms versus 163 +/- 23 ms, P= 0.007). PR interval increased significantly from closure to latest follow-up in the ASD (P=0.038), but not in the PFO group (P=0.058). CONCLUSIONS: ASD patients presented with significantly longer PR interval than PFO patients before closure. In contrast with the PFO group, the PR interval increased in the ASD group after medium-term follow-up. These findings indicate the need for further follow-up after ASD repair in order to detect potential late AVCD.

The outcome of Eisenmenger patients with trisomy 21 does not differ from patients without trisomy 21.

OBJECTIVE: 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. DESIGN: 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. RESULTS: 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). CONCLUSION: 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.

Comparison of risk stratification models for pregnancy in congenital heart disease.

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.

Covered stent placement for treatment of coarctation of the aorta: immediate and long-term results.

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 < 0.001). After a mean follow-up time of 6.6 ± 3.7 years, there was a persistent improvement of the mean systolic blood pressure gradient between right arm and leg (-7 ± 18 vs 38 ± 24 mmHg; p < 0.001). A larger proportion of patients required antihypertensive medication (33.7% vs 50.0%, p = 0.017) and needed ≥ 2 drugs (20.2% vs 27.4%, p = 0.066) to control blood pressure. Long-term adverse events were found in 4.5% of patients [covered stent fracture (n = 3), aneurysm formation (n = 2)]. CONCLUSIONS: Covered stent implantation for CoA is highly successful, safe and results in a persistent hemodynamic improvement in the immediate and long-term outcome. Lifelong follow-up with additional antihypertensive drug treatment is mandatory to maintain favourable hemodynamic results after stenting. CONDENSED ABSTRACT: Long-term follow-up data on covered stents in patients with coarctation of the aorta are scarce. A cohort of 89 patients was reviewed. The procedural implantation success rate was 100%. The invasive gradient decreased from 25 ± 16 mmHg to 4 ± 7 mmHg (p < 0.001). After follow-up of 6.6 ± 3.7 years, there was a persistent improvement of the clinical systolic blood pressure gradient (-7 ± 18 vs 38 ± 24 mmHg; p < 0.001). However, a larger proportion of patients required antihypertensive medication (33.7% vs 50.0%, p = 0.017). Covered stent implantation results in favourable hemodynamic effects, but lifelong follow-up with additional antihypertensive drug treatment is mandatory to maintain these results.

15-Year follow-up of regional right and left ventricular function after the Senning operation: a Colour-Doppler myocardial imaging study.

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 < 0.05). CONCLUSION: Our study showed that systolic and diastolic sRV function did not change over a 15-year follow-up period, except in the apical region. There was a decline in spLV systolic function, which may be of clinical value. On the other hand, when compared to age- and gender-matched controls, the sRV of Senning patients exhibits significantly decreased measurements of longitudinal systolic function.