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- Daene, M, et al.
(författare)
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Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect
- 2023
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Ingår i: International Journal of Cardiology Congenital Heart Disease. - : Elsevier BV. - 2666-6685. ; 12
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Tidskriftsartikel (refereegranskat)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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- Erard, Margot, et al.
(författare)
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Left atrial strain in patients after arterial switch operation for transposition of the great arteries
- 2023
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Ingår i: Acta Cardiologica. - 0001-5385 .- 0373-7934.
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Tidskriftsartikel (refereegranskat)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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- Maessen, Lenn, et al.
(författare)
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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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Ingår i: The Canadian journal of cardiology. - : Elsevier BV. - 0828-282X .- 1916-7075. ; 39:3, s. 292-301
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Tidskriftsartikel (refereegranskat)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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- Santens, Béatrice, et al.
(författare)
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Decreased cardiac reserve in asymptomatic patients after arterial switch operation for transposition of the great arteries.
- 2023
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Ingår i: International journal of cardiology. - 1874-1754. ; 388
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Tidskriftsartikel (refereegranskat)abstract
- Exercise capacity is impaired in patients after arterial switch operation (ASO) for complete transposition of the great arteries. Maximal oxygen consumption is related with outcome.This study assessed ventricular function by advanced echocardiography and cardiac magnetic resonance (CMR) imaging at rest and during exercise, to determine exercise capacity in ASO patients, and to correlate exercise capacity with ventricular function as potential early marker of subclinical impairment.Forty-four patients (71% male, mean age 25±4years - range 18-40years) were included during routine clinical follow-up. Assessment involved physical examination, 12‑lead ECG, echocardiography, and cardiopulmonary exercise test (CPET) (day 1). On day 2 CMR imaging at rest and during exercise was performed. Blood was sampled for biomarkers.All patients reported New York Heart Association class I, the overall cohort had an impaired exercise capacity (80±14% of predicted peak oxygen consumption). Fragmented QRS was present in 27%. Exercise CMR showed that 20% of patients had abnormal contractile reserve (CR) of the left ventricle (LV) and 25% had reduced CR of the right ventricle (RV). CR LV and CR RV were significantly associated with impaired exercise capacity. Pathological patterns on myocardial delayed enhancement and hinge point fibrosis were detected. Biomarkers were normal.This study found that in some asymptomatic ASO patients electrical, LV and RV changes at rest, and signs of fibrosis are present. Maximal exercise capacity is impaired and seems to be linearly related to the CR of the LV and the RV. Therefore, exercise CMR might play a role in detecting subclinical deterioration of ASO patients.
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