32 research outputs found
Clinical Course of TGA After Arterial Switch Operation in the Current Era
Background: The number of patients with an arterial switch operation (ASO) for transposition of the great arteries (TGA) is steadily growing; limited information is available regarding the clinical course in the current era. Objectives: The purpose was to describe clinical outcome late after ASO in a national cohort, including survival, rates of (re-)interventions, and clinical events. Methods: A total of 1,061 TGA-ASO patients (median age 10.7 years [IQR: 2.0-18.2 years]) from a nationwide prospective registry with a median follow-up of 8.0 years (IQR: 5.4-8.8 years) were included. Using an analysis with age as the primary time scale, cumulative incidence of survival, (re)interventions, and clinical events were determined. Results: At the age of 35 years, late survival was 93% (95% CI: 88%-98%). The cumulative re-intervention rate at the right ventricular outflow tract and pulmonary branches was 36% (95% CI: 31%-41%). Other cumulative re-intervention rates at 35 years were on the left ventricular outflow tract (neo-aortic root and valve) 16% (95% CI: 10%-22%), aortic arch 9% (95% CI: 5%-13%), and coronary arteries 3% (95% CI: 1%-6%). Furthermore, 11% (95% CI: 6-16%) of the patients required electrophysiological interventions. Clinical events, including heart failure, endocarditis, and myocardial infarction occurred in 8% (95% CI: 5%-11%). Independent risk factors for any (re-)intervention were TGA morphological subtype (Taussig-Bing double outlet right ventricle [HR: 4.9, 95% CI: 2.9-8.1]) and previous pulmonary artery banding (HR: 1.6, 95% CI: 1.0-2.2). Conclusions: TGA-ASO patients have an excellent survival. However, their clinical course is characterized by an ongoing need for (re-)interventions, especially on the right ventricular outflow tract and the left ventricular outflow tract indicating a strict lifelong surveillance, also in adulthood.</p
Preserved Myocardial Deformation after Successful Coarctation Repair: A CMR Feature-Tracking Study
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Surgical Repair of Ventricular Septal Defect; Contemporary Results and Risk Factors for a Complicated Course
Surgical closure of the ventricular septal defect is the most commonly performed procedure in pediatric cardiac surgery. There are conflicting data on weight at operation as risk factor for a complicated course. We performed a retrospective evaluation of mortality and morbidity in all patients undergoing surgical ventricular septal defect closure at our institution between 2004 and 2012 to identify risk factor for a complicated course. Multivariate logistic regression modeling was performed to identify risk factors for a complicated course. 243 patients who underwent surgical ventricular septal defect closure were included. Median age at operation was 168.0 days (range 17-6898), the median weight 6.0 kg (range 2.1-102.0). No deaths occurred. Two patients (0.8%) required a pacemaker for permanent heart block. Five patients (2.1%) underwent reoperation for a hemodynamically important residual ventricular septal defect. No other major adverse events occurred. No risk factors for major adverse events could be established. Multivariate analysis identified a genetic syndrome, long bypass time and low weight at operation as independent risk factors for a prolonged intensive care stay (>1 day) and prolonged ventilation time (>6 h). Contemporary results of surgical VSD closure are excellent with no mortality and low morbidity in this series. Although it is associated with increased ventilation time and a longer hospital stay, low bodyweight at operation is not associated with an increased risk of complications or major adverse events in our series
Similarities and Differences: the Operation of the Exclusionary Rule in the US, Germany and the Netherlands
Treatment of fetal heart block with maternal steroid therapy: case report and review of the literature.
The presence of maternal autoantibodies to SS-A/Ro and/or SS-B/La is associated with the development of fetal heart block. There are data suggesting that maternal treatment with steroids might reverse heart block. We report on a pregnancy in a mother with secondary Sjögren syndrome and systemic lupus erythematosus with presence of autoantibodies to SS-A/Ro and SS-B/La, which was complicated by the development of incomplete fetal heart block. Oral dexamethasone treatment could not prevent progression to complete heart block and was associated with a number of complications.A review of the literature revealed 19 studies (including ours) in which 93 cases of fetal heart block were treated with maternal steroid therapy. Complete heart block proved irreversible in all cases; and of 13 fetuses with incomplete heart block which received maternal steroid therapy, three had a reduction in their degree of block and one reverted to sinus rhythm. Maternal steroid therapy, initiated early in pregnancy and potentially preventing the onset of heart block, did not decrease the incidence of heart block in nine studies with 43 cases. Furthermore, the literature review revealed numerous serious side effects of maternal steroid administration during pregnancy. Data on these potential side effects are lacking in the 28 studies discussed in this review. Maternal dexamethasone therapy to prevent or treat fetal heart block remains, in our opinion, a questionable intervention and can as yet not be recommended in the clinical situation
Normative Values of Aortic Arch Structures in Premature Infants
BACKGROUND: Aortic arch abnormalities represent 5% to 8% of all congenital heart disease. Measurements of the aortic arch dimensions on two-dimensional echocardiographic images remain of critical importance in the diagnosis of aortic arch pathology. To define aortic hypoplasia or coarctation, measured dimensions must be compared with normal values. Normal values have been described for children of all ages in earlier studies. However, normative data for premature infants are not yet available. Therefore, the aim of this study was to develop normative data in a cohort of premature infants, which could be used in the diagnosis of aortic arch abnormalities. METHODS: A single-center study was conducted in a large population of premature infants with gestational ages of </=32 weeks without hemodynamically important congenital heart disease, chromosomal abnormalities, and/or major cerebral congenital malformations. Two-dimensional echocardiographic measurements of four aortic arch structures were made on the second, fourth, and sixth days after birth. RESULTS: Three hundred eighty-five preterm patients were included. No differences in dimensions were found among days 2, 4, and 6. The dimension of the isthmus showed no significant relation to the existence of a patent ductus arteriosus. Reference intervals with mean and SD were calculated across the range of birth weight. Regression analysis was performed with multiple determinants in different models. The best predictive value was found for birth weight in a cubic model. CONCLUSIONS: This work provides regression equations for the calculation of Z scores and reference intervals for aortic arch dimensions in a cohort of preterm infants born at gestational ages of </=32 weeks. The normative data can be used in diagnosis and decision making involving aortic arch pathology in premature infants
Five years of Kawasaki disease in the Netherlands a national surveillance study
Background: The aim of this study was to evaluate the incidence, disease presentation, treatment and cardiac outcome of Kawasaki disease (KD) in The Netherlands. Methods: The national Dutch Pediatric Surveillance Unit was used to prospectively register new KD cases from 2008 through 2012. Questionnaires were sent to pediatricians to obtain clinical information. Results: Nationwide 341 cases were reported during the 5-year study period, of which 319 questionnaires (93.0%) were returned. The mean incidence of KD was estimated to be 5.8/100,000 children 10 days) and IVIG retreatment were independent risk factors for coronary artery aneurysms development. Conclusions: This prospective study of KD in The Netherlands revealed a mean annual incidence of 5.8/100,000 children <5 years of age. Clinicians should consider the diagnosis of KD in young (male) children with persistent inexplicable fever to start IVIG treatment within 10 days to prevent development of coronary artery aneurysms. Copyright © 2014 by Lippincott Williams & Wilkins
Evaluation of Left Ventricular Function Long Term After Arterial Switch Operation for Transposition of the Great Arteries
Long-term after arterial switch operation for transposition of the great arteries, abnormal coronary anatomy and altered loading conditions could compromise ventricular function. The current study investigates whether left ventricular function, measured with echocardiographic bi-plane ejection fraction and deformation imaging, in patients long term after arterial switch operation for transposition of the great arteries differs from healthy peers. A cross-sectional cohort study of patients at least 12 years after arterial switch operation was analyzed with bi-plane Simpson's left ventricular ejection fraction (LVEF) and deformation (speckle tracking) echocardiography. 81 patients, median age 20.6 (interquartile range 13.5-28.4) years, were included. LVEF was normal on average at 55.5 +/- 6.1%. Global longitudinal strain (GLS) was lower in patients compared to healthy peers throughout all age groups and on pooled average (- 15.4 +/- 1.1% vs. - 23.2 +/- 0.9%). Although LVEF is normal on average in patients after arterial switch operation for transposition of the great arteries, GLS is impaired compared to healthy peers. The reduced GLS could indicate sub-clinical myocardial dysfunction