| 1. |
- Conings, Nele, et al.
(author)
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Biomarkers in transposition of the great arteries after arterial switch operation: A pilot trial with deep phenotyping
- 2024
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In: International Journal of Cardiology. - 0167-5273 .- 1874-1754. ; 397
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Journal article (peer-reviewed)abstract
- 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.
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| 2. |
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| 3. |
- Arnaert, Stijn, et al.
(author)
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Heart failure related to adult congenital heart disease: prevalence, outcome and risk factors.
- 2021
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In: ESC heart failure. - : Wiley. - 2055-5822. ; 8:4, s. 2940-2950
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Journal article (peer-reviewed)abstract
- 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.We included 3905 patients (age 35.4±13.2years) 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±16.7years), 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 10years; 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 10bpm; 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.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.
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| 4. |
- Daene, M, et al.
(author)
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Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect
- 2023
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In: International Journal of Cardiology Congenital Heart Disease. - : Elsevier BV. - 2666-6685. ; 12
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Journal article (peer-reviewed)abstract
- Background and aims Patients with Down Syndrome (DS) are frequently born with an atrioventricular septal defect (AVSD). Surgical repair of the defect aims to minimize mortality and morbidity. However, a surgical intervention, specifically in DS patients, is not without risk and a subgroup of patients underwent only conservative non-surgical treatment. Outcome data of these different approaches are scarce. The aim of this retrospective study was to compare the long-term outcome of DS patients with and without surgery for AVSD. Methods DS patients registered with AVSD in the hospital's database from January 1980 till December 2020 were selected. Patient characteristics, peri-operative if appropriate, and follow-up data were obtained from the medical files. Results In total, 72 unrepaired (36 male, 50%) and 134 repaired patients (61 male, 46%) were included. After a maximum of 60 years of follow-up, the all-cause mortality was 45.8% and 17.1%, respectively. Thirty-six percent and 13%, respectively, were labeled as non-cardiovascular death. Mean survival time for unrepaired AVSD was 40.7 years (95% CI 36.1–45.2) and for repaired AVSD 38.5 years (95% CI 35.3–41.6) (Log rank p = 0.465). However, the survival rate 35 years after birth was 62.1% for unrepaired patients versus 81.7% for repaired patients. Mortality rates were the highest the first months after surgical repair. Conclusions The mean survival rate of Down patients, born with an AVSD, did not differ between repair or not. However, long-term survival rate was higher in patients who underwent surgical repair. Mortality was highest the first months after surgery.
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| 5. |
- Denayer, Nathalie, et al.
(author)
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Comparison of risk stratification models for pregnancy in congenital heart disease.
- 2021
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In: International journal of cardiology. - : Elsevier BV. - 1874-1754 .- 0167-5273. ; 323, s. 54-60
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Journal article (peer-reviewed)abstract
- 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.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.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.
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| 6. |
- Erard, Margot, et al.
(author)
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Left atrial strain in patients after arterial switch operation for transposition of the great arteries
- 2023
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In: Acta Cardiologica. - 0001-5385 .- 0373-7934.
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Journal article (peer-reviewed)abstract
- 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 (β − 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.
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| 7. |
- Gabriels, Charlien, et al.
(author)
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Serial pulmonary vascular resistance assessment in patients late after ventricular septal defect repair.
- 2019
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In: International journal of cardiology. - : Elsevier BV. - 1874-1754 .- 0167-5273. ; 282, s. 38-43
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Journal article (peer-reviewed)abstract
- The long-term evolution of pulmonary vascular resistance (PVR) after ventricular septal defect (VSD) repair is unknown. This study serially evaluated resting and exercise PVR after VSD repair in childhood.Patients were enrolled from the outpatient Adult Congenital Heart Disease clinic of the University Hospitals Leuven and compared to age- and gender-matched controls. Participants underwent resting and exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. Total PVR was calculated as the ratio of mean pulmonary artery pressure (mPAP) to cardiac output (CO). The slope of the mPAP-CO curve (exercise PVR) was obtained using linear regression analysis.Twenty-seven patients (mean age 31±7years, 70% male) and 18 controls were included. At baseline, patients had larger right ventricular (RV) end-diastolic areas (10±2 vs 9±1cm2/m2, p=0.001) and lower tricuspid annular plane systolic excursion (TAPSE) (17 (17-19) vs 26 (22-28) mm, p<0.001). After 1.1 (1.0-1.5) years follow-up, similar differences in RV areas and TAPSE were found. Patients reached lower peak workload and cardiac index compared to controls at each time point. Peak total PVR was higher (Baseline: 2.7±0.8 vs 2.2±0.3mmHg/L/min, p=0.005; Follow-up: 2.9±0.9 vs 2.1±0.3mmHg/L/min, p<0.001) and the mPAP-CO slope was steeper (Baseline: 2.2±0.8 vs 1.7±0.3mmHg/L/min, p=0.008; Follow-up: 2.5±0.9 vs 1.6±0.3mmHg/L/min, p<0.001) in patients. The mPAP-CO slope in patients correlated inversely with peak oxygen uptake (R=-0.41 and-0.45, p=0.036 and 0.022, baseline and follow-up, respectively).Despite repair, VSD patients seem to show altered pulmonary hemodynamics and RV impairment at rest and exercise, supporting life-long follow-up.
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| 8. |
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| 9. |
- Maessen, Lenn, et al.
(author)
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Short-term Prognostic Value of Heart Failure Diagnosis in a Contemporary Cohort of Patients With Adult Congenital Heart Disease.
- 2023
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In: The Canadian journal of cardiology. - : Elsevier BV. - 0828-282X .- 1916-7075. ; 39:3, s. 292-301
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Journal article (peer-reviewed)abstract
- 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).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.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).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.
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| 10. |
- Santens, Béatrice, et al.
(author)
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Adverse functional remodelling of the subpulmonary left ventricle in patients with a systemic right ventricle is associated with clinical outcome.
- 2022
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In: European heart journal. Cardiovascular Imaging. - : Oxford University Press (OUP). - 2047-2412 .- 2047-2404. ; 23:5, s. 680-688
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Journal article (peer-reviewed)abstract
- 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.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 3years. 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.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.
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