Abstract The outcome for all children born in Sweden between January 1, 1980 and December 31, 2008 with ventriculo-coronary arterial communications (VCACs) and pulmonary atresia with intact ...ventricular septum (PAIVS) was assessed. Fifty-one subjects, 42%, of all 125 children born with PAIVS had identifiable VCACs. Cumulative survival after surgery was 67%, as compared with 73% for all subjects born with PAIVS (n.s.). Survival improved over time, and there was no late death after 3 years of age. Median follow-up time was 12 years (range 1–28 years). At follow-up, 33 subjects were alive (12 female/21 male) with the following repairs: six had a biventricular repair, one had 1.5 ventricle repair, 25 were palliated with a single ventricle repair (final stage in 20), and one had a heart transplantation. Notably after decompression ( n = 9 survivors), the disease in the coronary arteries did not progress, although four subjects in this group had large VCACs. In the group of subjects with single ventricle repair and large VCACs, eleven were found to have stenoses in the coronary arteries. The disease in the coronary arteries in this group, however, often progressed with an adverse effect on myocardial perfusion. Four of the survivors had documented myocardial infarctions.
ABSTRACT
The types of coumadin anticoagulants registered and available for use differ between countries. Most frequently used coumadin anticoagulants are warfarin and acenocoumarol. Under several ...specific conditions, transition from one coumarin to another is required. Because of different pharmacokinetic and pharmacodynamic characteristics, the transition from one type of coumarol to another type can be challenging. There are no studies that address this issue in children. We present the case report of transition treatment between acenocoumarol and warfarin in a 12‐year‐old child with prosthetic mitral valve.
To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood.
Retrospective international collaborative study involving 19 paediatric cardiology ...centres in the UK, Ireland and Sweden.
Cases of PVS presenting between 1 January 1995 and 31 December 2004 were identified. Cases where pulmonary veins connected to a morphological left atrium were included. Functionally univentricular hearts and total anomalous pulmonary venous connection were excluded. All available data and imaging were reviewed.
58 cases were identified. In 22 cases (38%) there was premature delivery. 46 (79%) had associated cardiac lesions; 16 (28%) had undergone previous cardiac surgery before PVS diagnosis. 16 children (28%) had a syndrome or significant extracardiac abnormality. 36 presented with unilateral disease of which 86% was on the left. Where there was adequate sequential imaging, disease progression was shown with discrete stenosis leading to diffusely small pulmonary veins. Collateral vessels often developed. 13 patients had no intervention. Initial intervention was by catheter in 17 and surgery in 28. Overall 3-year survival was 49% (95% CI 35% to 63%) with patients undergoing initial surgical intervention having greater freedom from death or re-intervention (hazard ratio 0.44, 95% CI 0.2 to 0.99, p = 0.023).
PVS is a complex disease of uncertain cause and frequently associated with prematurity. Early intervention may be indicated to deter irreversible secondary changes.
The aim of this study was to study the type and frequency of complications and change in weight after a laparoscopic gastrostomy procedure in 31 children with congenital heart disease, comparing ...patient groups of children with univentricular and biventricular circulation, and with completed and uncompleted cardiac surgery.
The method used was that of a retrospective study of all 31 children with congenital heart disease who underwent a laparoscopic gastrostomy at our center from 1995 to 2004.
Postoperative complications and body weight changes during follow-up were the main outcome measures used in this study.
Minor stoma-related problems were common in both groups. Two severe complications requiring an operative intervention occurred in the univentricular circulation group. Weight was normal at birth, low at the time of the gastrostomy procedure, and did not catch up completely during the follow-up period of a mean of 20 months. There were no significant differences regarding mean weight gain between the groups.
The complication rate after the laparoscopic gastrostomy procedure was higher in our patient group, compared to previously studied children with various diseases. Comparisons regarding mean weight gain between the groups showed no significant differences. The mean weight gain was low, suggesting that the energy expenditure in this patient group of children with severe congenital heart disease may be even higher than previously assumed.
Children who undergo surgery for complex congenital cardiac disease are reported to be at increased thrombotic risk. Our aim was to evaluate long-term changes in the haemostatic system after surgery, ...to compare markers of activated coagulation in children having surgery with those in a healthy control population, and to relate them to adverse clinical outcome.
We studied, prior to surgery, the coagulation profiles of a cohort of 28 children admitted for a modified Fontan operation, studying them again after a period of mean follow-up of 9.6 years. Median age at the time of final surgery was 18.5 months, with a range from 12 to 76 months. We compared generation of thrombin, and levels of the activated protein C-protein C inhibitor complex to controls at follow-up. Thrombophilia and clinical outcome were evaluated.
At long-term follow-up, a lower incidence of procoagulant abnormalities was observed compared to that before surgery. Of 27 patients, 3 (11%), but none of 45 controls, had levels of activated protein C-protein C inhibitor complex above the reference range. There were no significant differences in generation of thrombin between patients and controls. No thrombotic events were recorded, and the patients were generally in good clinical condition.
Overall, haemostasis appeared to be in balance, and less prothrombotic, after surgery. A subset of the cohort did show indications of activated coagulation. The current therapeutic approach seems to be sufficient to protect the majority of patient. New tests of global coagulation, nonetheless, may be helpful in improving identification of individuals at increased thrombotic risk.
Purpose: Adolescents with congenital heart disease transition from childhood to adulthood and transfer from pediatric-oriented to adult-oriented care. High-level empirical evidence on the ...effectiveness of transitional care is scarce. This study investigated the empowering effect (primary outcome) of a structured person-centered transition program for adolescents with congenital heart disease and studied its effectiveness on transition readiness, patient-reported health, quality of life, health behaviors, disease-related knowledge, and parental outcomes e.g., parental uncertainty, readiness for transition as perceived by the parents (secondary outcomes). Methods: The STEPSTONES-trial comprised a hybrid experimental design whereby a randomized controlled trial was embedded in a longitudinal observational study. The trial was conducted in seven centers in Sweden. Two centers were allocated to the randomized controlled trial-arm, randomizing participants to intervention or control group. The other five centers were intervention-na & iuml;ve centers and served as contamination check control group. Outcomes were measured at the age of 16 years (baseline), 17 years, and 18.5 years. Results: The change in empowerment from 16 years to 18.5 years differed significantly between the intervention group and control group (mean difference = 3.44; 95% confidence interval = 0.27-6.65; p = .036) in favor of intervention group. For the secondary outcomes, significant differences in change over time were found in parental involvement (p = .008), disease-related knowledge (p = .0002), and satisfaction with physical appearance (p = .039). No differences in primary or secondary outcomes were detected between the control group and contamination check control group, indicating that there was no contamination in the control group. Discussion: The STEPSTONES transition program was effective in increasing patient empowerment, reducing parental involvement, improving satisfaction with physical appearance, and increasing disease-related knowledge. (c) 2023 Society for Adolescent Health and Medicine. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As ...the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making.
PDF
