| 1. |
- Acuña Mora, Mariela, 1990, et al.
(author)
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The longitudinal association between patient empowerment and patient-reported outcomes: What is the direction of effect?
- 2022
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In: PloS one. - : Public Library of Science (PLoS). - 1932-6203. ; 17:11
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Journal article (peer-reviewed)abstract
- Theoretical literature and cross-sectional studies suggest empowerment is associated with other patient-reported outcomes (PROs). However, it is not known if patient empowerment is leading to improvements in other PROs or vice versa.The present study aimed to examine the direction of effects between patient empowerment and PROs in young persons with congenital heart disease (CHD).As part of the STEPSTONES-CHD trial, adolescents with CHD from seven pediatric cardiology centers in Sweden were included in a longitudinal observational study (n = 132). Data were collected when patients were 16 (T0), 17 (T1) and 18 ½ years old (T2). The Gothenburg Young Persons Empowerment Scale (GYPES) was used to measure patient empowerment. Random intercepts cross-lagged panel models between patient empowerment and PROs (communication skills; patient-reported health; quality of life; and transition readiness) were undertaken.We found a significant cross-lagged effect of transition readiness over patient empowerment between T1 and T2, signifying that a higher level of transition readiness predicted a higher level of patient empowerment. No other significant cross-lagged relationships were found.Feeling confident before the transition to adult care is necessary before young persons with CHD can feel in control to manage their health and their lives. Clinicians interested in improving patient empowerment during the transitional period should consider targeting transition readiness.
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| 2. |
- Avdikos, Vasileios, et al.
(author)
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Outcomes following surgical repair of absent pulmonary valve syndrome : 30 years of experience from a Swedish tertiary referral centre
- 2022
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In: Interactive Cardiovascular and Thoracic Surgery. - : Oxford University Press (OUP). - 1569-9293 .- 1569-9285. ; 35:2
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Journal article (peer-reviewed)abstract
- Surgical approach with reduction pulmonary artery plasty and valved conduit in patients with respiratory compromise prior to repair is associated with excellent long-term survival at the cost of a higher reintervention rate. OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable. METHODS: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018. RESULTS: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency. CONCLUSIONS: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.
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| 3. |
- Back, Julia, et al.
(author)
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Evidence of support used for drug treatments in pediatric cardiology
- 2021
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In: Health Science Reports. - : WILEY. - 2398-8835. ; 4:2
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Journal article (peer-reviewed)abstract
- Background and aims: Clinical support systems are widely used in pediatric care. The aim of this study was to assess the support for drug treatments used at pediatric cardiac wards and intensive care units in Sweden.Methods: Drug information, such as type of drug, indication, dose, and route of administration, for all in-hospital pediatric cardiac patients, was included in the study. Treatments were classified as either on-label (based on product information) or off-label. Support for off-label treatment was stratified by the use of clinical support systems (the national database on drugs, local, or other clinical experience guidelines).Results: In all, 28 patients were included in the study. The total number of drug treatments was 233, encompassing 65 different drugs. Overall, 175 (75%) treatments were off-label. A majority of off-label drug treatments were supported by other sources of information shared by experts. A total of 7% of the drug treatments were used without support.Conclusion: Off-label drug treatment is still common in Swedish pediatric cardiac care. However, the majority of treatments were supported by the experience shared in clinical support systems.Key Points:Seventy-five percent of all prescriptions in pediatric cardiology care were off-label.A majority of patients received three or more drug treatments off-label.Use of clinical support systems and guidelines was common, but in 7% of all drug treatments, no support was found for the chosen treatment.
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| 4. |
- Bhat, Misha, et al.
(author)
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Longitudinal ECG changes in tetralogy of Fallot and association with surgical repair
- 2024
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In: Frontiers in Cardiovascular Medicine. - 2297-055X. ; 11
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Journal article (peer-reviewed)abstract
- Background: ECG abnormalities have been linked to adverse changes in right ventricular (RV) morphology and poor clinical outcomes in repaired Tetralogy of Fallot (rTOF). Our aim was to describe how ECG changes progress in early and intermediate follow-up and whether types of surgical strategy at the time of primary repair affected these changes. Methods: We studied patients with rTOF born 2000–2018 operated at our institution. Seven time points in relation to primary repair, follow-up, and pulmonary valve replacement (PVR) were identified. Patients correct with valve sparing repair (VSR), trans-annular patch (TAP) including with a monocusp valve (TAP + M) and with at least 3 ECGs were included. PQ interval, QRS duration, dispersion, and fragmentation, QTc duration and dispersion, JTc as well as presence of a right bundle branch block (RBBB) were analyzed. Medical records were reviewed for demographic and surgical data. Results: Two hundred nineteen patients with 882 ECGs were analyzed with a median follow-up time of 12.3 years (8.4, 17) with 41 (19%) needing PVR during the study period. QRS duration increased at time of primary repair to discharge from 66 msec (IQR 12) to 129 msec (IQR 27) (p < 0.0001) and at 1- and 6- year follow-up but showed only a modest and temporary decrease after PVR. QTc increased at the time of primary repair as well as prior to PVR. PQ interval showed a small increase at the time of primary repair, was at its highest prior to PVR and decreased with PVR. Type of surgical repair affected mainly QTc and JTc and was consistently longer in the TAP + M group until PVR. In VSR, QTc and JTc were prolonged initially compared to TAP but were similar after 1 year. After PVR, there were no differences in adverse ECG changes between surgical groups. Conclusions: PQ interval and QRS duration best correspond to the assumed volume load whereas the relationship with QTc and JTc is more complex, suggesting that these represent more complex remodeling of the myocardium. Before PVR, QTc and JTc are longer in the TAP + M group which may be due to a longer surgical incision.
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| 5. |
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| 6. |
- Bratt, Ewa-Lena, 1970, et al.
(author)
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Effectiveness of the STEPSTONES Transition Program for Adolescents With Congenital Heart Disease : A Randomized Controlled Trial
- 2023
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In: Journal of Adolescent Health. - : Elsevier. - 1054-139X .- 1879-1972.
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Journal article (peer-reviewed)abstract
- 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-naïve 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. © 2023 Society for Adolescent Health and Medicine
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| 7. |
- Fricke, Katrin, et al.
(author)
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Fetal cardiac magnetic resonance imaging of the descending aorta in suspected left-sided cardiac obstructions
- 2023
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In: Frontiers in Cardiovascular Medicine. - 2297-055X. ; 10, s. 01-10
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Journal article (peer-reviewed)abstract
- Background: Severe left-sided cardiac obstructions are associated with high morbidity and mortality if not detected in time. The correct prenatal diagnosis of coarctation of the aorta (CoA) is difficult. Fetal cardiac magnetic resonance imaging (CMR) may improve the prenatal diagnosis of complex congenital heart defects. Flow measurements in the ascending aorta could aid in predicting postnatal CoA, but its accurate visualization is challenging.Objectives: To compare the flow in the descending aorta (DAo) and umbilical vein (UV) in fetuses with suspected left-sided cardiac obstructions with and without the need for postnatal intervention and healthy controls by fetal phase-contrast CMR flow. A second objective was to determine if adding fetal CMR to echocardiography (echo) improves the fetal CoA diagnosis.Methods: Prospective fetal CMR phase-contrast flow in the DAo and UV and echo studies were conducted between 2017 and 2022.Results: A total of 46 fetuses with suspected left-sided cardiac obstructions [11 hypoplastic left heart syndrome (HLHS), five critical aortic stenosis (cAS), and 30 CoA] and five controls were included. Neonatal interventions for left-sided cardiac obstructions (n = 23) or comfort care (n = 1 with HLHS) were pursued in all 16 fetuses with suspected HLHS or cAS and in eight (27%) fetuses with true CoA. DAo or UV flow was not different in fetuses with and without need of intervention. However, DAo and UV flows were lower in fetuses with either retrograde isthmic systolic flow [DAo flow 253 (72) vs. 261 (97) ml/kg/min, p = 0.035; UV flow 113 (75) vs. 161 (81) ml/kg/min, p = 0.04] or with suspected CoA and restrictive atrial septum [DAo flow 200 (71) vs. 268 (94) ml/kg/min, p = 0.04; UV flow 89 vs. 159 (76) ml/kg/min, p = 0.04] as well as in those without these changes. Adding fetal CMR to fetal echo predictors for postnatal CoA did not improve the diagnosis of CoA.Conclusion: Fetal CMR-derived DAo and UV flow measurements do not improve the prenatal diagnosis of left-sided cardiac obstructions, but they could be important in identifying fetuses with a more severe decrease in blood flow across the left side of the heart. The physiological explanation may be a markedly decreased left ventricular cardiac output with subsequent retrograde systolic isthmic flow and decreased total DAo flow.
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| 8. |
- Fricke, Katrin, et al.
(author)
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Impact of Left Ventricular Morphology on Adverse Outcomes Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome: 20 Years of National Data From Sweden
- 2022
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In: Journal of the American Heart Association. - : John Wiley & Sons. - 2047-9980. ; 11:7
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Journal article (peer-reviewed)abstract
- BACKGROUND: Hypoplastic left heart syndrome is associated with significant morbidity and mortality. We aimed to assess the influence of left ventricular morphology and choice of shunt on adverse outcome in patients with hypoplastic left heart syndrome and stage 1 palliation. METHODS AND RESULTS: This was a retrospective analysis of patients with hypoplastic left heart syndrome with stage 1 palliation between 1999 and 2018 in Sweden. Patients (n=167) were grouped based on the anatomic subtypes aortic-mitral atresia, aortic atresia-mitral stenosis (AA-MS), and aortic-mitral stenosis. The left ventricular phenotypes including globular left ventricle (Glob-LV), miniaturized and slit-like left ventricle (LV), and the incidence of major adverse events (MAEs) including mortality were assessed. The overall mortality and MAEs were 31% and 41%, respectively. AA-MS (35%) was associated with both mortality (all other subtypes versus AA-MS: interstage-I: hazard ratio [HR], 2.7; P=0.006; overall: HR, 2.2; P=0.005) and MAEs (HR, 2.4; P=0.0009). Glob-LV (57%), noticed in all patients with AA-MS, 61% of patients with aortic stenosis-mitral stenosis, and 19% of patients with aortic atresia-mitral atresia, was associated with both mortality (all other left ventricular phenotypes versus Glob-LV: interstage-I: HR, 4.5; P=0.004; overall: HR, 3.4; P=0.0007) and MAEs (HR, 2.7; P=0.0007). There was no difference in mortality and MAEs between patients with AA-MS and without AA-MS with Glob-LV (P>0.15). Patients with AA-MS (35%) or Glob-LV (38%) palliated with a Blalock-Taussig shunt had higher overall mortality compared with those palliated with Sano shunts, irrespective of the stage 1 palliation year (AA-MS: HR, 2.6; P=0.04; Glob-LV: HR, 2.1; P=0.03). CONCLUSIONS: Glob-LV and AA-MS are independent morphological risk factors for adverse short-and long-term outcome, especially if a Blalock-Taussig shunt is used as part of stage 1 palliation. These findings are important for the clinical management of patients with hypoplastic left heart syndrome.
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| 9. |
- Giang, Kok Wai, 1984, et al.
(author)
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Congenital heart disease: changes in recorded birth prevalence and cardiac interventions over the past half-century in Sweden
- 2023
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In: European Journal of Preventive Cardiology. - : Oxford University Press (OUP). - 2047-4873 .- 2047-4881. ; 30:2, s. 169-176
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Journal article (peer-reviewed)abstract
- Aims Our objective was to assess changes in the birth prevalence of CHD over a half-century in a high-resource, nationwide setting, as well as changes in the prevalence of cardiac interventions in this population. Methods and results The Swedish National Patient and Cause of Death registers were linked to estimate the annual rates of CHD and cardiac interventions among live-born infants from 1970 to 2017. Additionally, separate estimates were obtained by lesion complexity, from mild to the most complex forms of CHD. Overall, the numbers of live-born infants with a CHD identified varied from 624 to 2459 annual cases, with rates increasing steadily from 5.7 to an average of 20 per 1000 live births at the end of the study period, and with a more pronounced increase from 1996 to 2005. The largest increase over time was observed for mild CHD lesions. Overall, the proportion of cardiac interventions among patients with CHD declined from 40.7% in 1970 to below 15.0% after 2014. However, in the most complex CHD lesion groups, overall cardiac interventions increased from 57.1 to 76.8% in patients with conotruncal defects and from 32.8 to 39.5% in those with severe non-conotruncal defects. Conclusion The live-birth prevalence of CHD in Sweden more than tripled during the past half-century, most likely resulting from more accurate diagnostic capabilities. The largest increase over time was observed among patients with simple defects. During the same period, overall cardiac interventions decreased whereas interventions for the most complex CHD groups increased.
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| 10. |
- Jacobs, Jeffrey P., et al.
(author)
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Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database : The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC)
- 2023
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In: World Journal for Pediatric and Congenital Heart Surgery. - 2150-1351. ; 14:4, s. 464-473
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Journal article (peer-reviewed)abstract
- The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.
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