Background
Due to rapid medical and technological progress, more and more pediatric patients with terminal cardiac insufficiency are being implanted with a ventricular assist device as a bridge to ...transplant without legal approval for hospital discharge. EXCOR
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Active is a recently developed mobile driving unit for the EXCOR
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ventricular assist device (EXCOR
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VAD) with a long-lasting battery life that can manage small blood pumps, offering improved mobility for pediatric patients. This study strives to elaborate the requirements necessary for a safe home healthcare environment (HHE) for pediatric patients on EXCOR
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VAD powered by the EXCOR
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Active driving unit.
Materials and methods
Patient- and device-related preconditions (medical, ethical, psychological, technical, structural, organizational) were analyzed with regard to feasibility and safety in three individual patient cases. Included were pediatric patients with terminal cardiac insufficiency in a stable medical condition receiving in-hospital treatment with a univentricular or biventricular EXCOR
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VAD powered by EXCOR
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Active. Analysis was single-center, data was obtained 05/2020–02/2022.
Results
A total of three patients on EXCOR
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VAD were identified for HHE treatment with the EXCOR
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Active driving unit. Switch was performed safely and increased mobility led to improved psychomotor development and improved quality of life. No complications directly related to HHE-treatment occurred. One patient recently underwent an orthotopic heart transplant, one patient remains in HHE, and one patient died due to a complication not related to the HHE. Ethical approval for off-label use was obtained and patients and parents were given the required technical training and psychological support. Caregivers and medical professionals involved in the patients’ care at home were briefed intensely. Remote consultations were implemented and interdisciplinary in-hospital checks reduced to a long-term 4-week-scheme.
Conclusion
While it is challenging to discharge pediatric patients being treated with a paracorporeal ventricular assist device (EXCOR
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VAD) from hospital, it is feasible and can be managed safely with the novel driving unit EXCOR
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Active. A HHE may help to improve patients’ psychomotor development, offer normalized social contacts and strengthen both patients’ and parents’ physical and mental resources. Legal approval and another study with a larger sample size are warranted.
Background
In Fontans, exercise tolerance is poorer compared to their healthy peers. Higher
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represents a strong predictor for mortality and morbidity in these patients. Cardiac ...rehabilitation programs have been shown to improve cardiopulmonary function in Fontans. More habitual physical activity should therefore lead to a better exercise tolerance.
Methods
We performed cardiopulmonary exercise testing in 24 Fontan patients who had engaged in physical activity for a minimum of 3 h per week over their lifetime. As a control we performed cardiopulmonary exercise testing in 20 Fontan patients who had undertaken no physical activity or <3 h per week in the past.
Results
A total of 44 Fontan patients was included (mean age 18.1 years). The mean parameters measured at peak exercise differed significantly between the active and inactive group (peak oxygen uptake
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of 34.0 vs. 25.0 ml/min/kg, peak heart rate (HR) of 169.8/min vs. 139.8/min). Even though the O
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pulse and the EF did not differ significantly between both groups, N-Terminal-Pro-B-Type Natriuretic Peptide (NT-pro BNP) was significantly higher in the inactive group. The two groups did not differ with respect to their cardiac function determined by magnetic resonance imaging (MRI).
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was positively correlated with hours of sports performed by Fontans.
Conclusions
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and maximum HR were significantly higher in Fontans who had been physically active compared to those who had been inactive. The values reported in this study were higher than in other studies and reached normal values for
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for most Fontans in the physically active group. The positive correlation between
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and physical activity is an indicator of the importance of incorporating physical exercise programs into the treatment of Fontan patients.
Ventricular assist devices (VADs) are used to provide mechanical circulatory support to patients with end-stage heart failure. The driveline connecting the external power source to the pump(s) of the ...intra-corporal VAD breaches the protective skin barrier and provides a track for microbes to invade the interior of the patient's body. Driveline infection constitutes a major and potentially fatal vulnerability of VAD therapy. Driveline infection cannot traditionally be salvaged and requires the extraction of the entire VAD system. We report here the successful eradication of a VAD driveline infection with a taurolidine-containing antimicrobial solution used for preventing the infection of cardiac implantable electronic devices. If replicated in more cases, the novel treatment concept described here may provide a valuable alternative management strategy of salvage rather than explantation for VAD driveline infection.
Background:
Protein-losing enteropathy (PLE) is a severe complication of the univentricular Fontan circulation and associated with disturbances in salt and water homeostasis. Fontan patients with PLE ...have a poor prognosis, with increased morbidity and mortality. Due to limited therapeutic strategies, patients are often treated only symptomatically.
Methods:
We report our first experience of Tolvaptan (TLV) treatment in a Fontan patient with PLE, severe volume retention and hyponatraemia, refractory to conventional diuretic therapy. In addition to clinical parameters, we monitored drug effects including tissue sodium and volume status via serial 23Na-magnetic resonance imaging (23Na-MRI) and bioimpedance spectroscopy compared with age-matched controls.
Results:
23Na-MRI identified elevated tissue sodium, which decreased under TLV treatment, as well as volume status, while serum sodium increased and the patient’s symptoms improved. During long-term treatment, we were able to differentiate between sodium and volume status in our patient, suggesting that TLV uncoupled body sodium from water.
Conclusion:
TLV in addition to loop diuretics improved clinical symptoms of PLE and lowered tissue sodium overload. Long-term effects should be further evaluated in Fontan patients.
(1) Purpose: to compare right ventricular (RV) functional parameters in children with surgically repaired congenital heart disease (CHD) using single/double breath hold (BH) and free-breathing (FB) ...real-time compressed sensing (CS) cine cardiac magnetic resonance (cMRI) with standard retrospective segmented multi breath hold (RMB) cine cMRI. (2) Methods: Twenty patients with CHD underwent BH and FB, as well as RMB cine cMRI, at 3T to obtain a stack of continuous axial images of the RV. Two radiologists independently performed qualitative analysis of the image quality (rated on a 5-point scale; 1 = non-diagnostic to 5 = excellent) and quantitative analysis of the RV volume measurements. (3) Results: The best image quality was provided by RMB (4.5; range 2-5) compared to BH (3.9; range 3-5;
= 0.04) and FB (3.6; range 3-5;
< 0.01). The RV functional parameters were comparable among BH, FB, and RMB with a difference of less than 5%. The scan times for BH (44 ± 38 s,
< 0.01) and FB (24 ± 7 s,
< 0.01) were significantly reduced compared to for RMB (261 ± 68 s). (4) Conclusions: CS-FB and CS-BH real-time cine cMRI in children with CHD provides diagnostic image quality with excellent accuracy for measuring RV function with a significantly reduced scan time compared to RMB.
Objectives In light of the ongoing discussion about flow-mediated arterial remodeling, it was the aim of this report to demonstrate the detailed assessment of 3-dimensional vascular hemodynamics by ...high-field magnetic resonance imaging in healthy volunteers and to illustrate its potential in comparison with results in a patient with stenosis. Materials and Methods All examinations consisted of flow-sensitive 4-dimensional magnetic resonance imaging at 3 Tesla. Retrospective blood flow visualization and segmental quantification of wall shear stress and oscillatory shear index were performed. The results from 11 healthy individuals were compared with a 13-year-old patient with aortic stenosis who received a combined protocol with time-resolved 3-dimensional magnetic resonance angiography before and 5 and 9 months after intervention. Results Evaluation of normal blood flow characteristics demonstrated predominantly right-handed helical flow in the ascending aorta. Vortex formation was observed in 1 of 11 volunteers. Consistently high segmental wall shear stress was found along the circumference of the ascending aorta (average wall shear stress = 0.191 ± 0.06 N/m2 ) and descending aorta (average 0.191 ± 0.06 N/m2 ). Compared with volunteers, the patient revealed substantial flow changes proximal and distal to the stenosis. Blood flow alterations in the ascending aorta were also observed associated with changes in velocities and wall shear stress that gradually normalized after intervention. Conclusion Flow-sensitive 4-dimensional magnetic resonance imaging at 3 Tesla can provide deeper insights into hemodynamic alterations in the diagnosis and follow-up of aortic pathologies. These findings indicate the potential of the methodology for the evaluation of effects of localized pathologies on the entire vascular system, which will have to be confirmed in future studies.
Myocarditis represents one of the most common causes of Sudden Cardiac Death in children. Myocardial involvement during a viral infection is believed to be higher as a consequence of intensive ...exertion. Recommendations for return to sports are based on cohort and case studies only. This study aims to investigate the relationship between physical activity and myocarditis in the young.
Every patient in the MYKKE registry fulfilling criteria for suspicion of myocarditis was sent a questionnaire regarding the physical activity before, during and after the onset of myocarditis.
This study is a subproject within the MYKKE registry, a multicenter registry for children and adolescents with suspected myocarditis. The observation period for this analysis was 93 months (September 2013-June 2021). Anamnestic, cardiac magnetic resonance images, echocardiography, biopsy and laboratory records from every patient were retrieved from the MYKKE registry database.
58 patients (mean age 14.6 years) were enrolled from 10 centers. Most patients participated in curricular physical activity and 36% in competitive sports before the onset of myocarditis. There was no significant difference of heart function at admission between the physically active and inactive subjects (ejection fraction of 51.8 ± 8.6% for the active group vs. 54.4 ± 7.7% for the inactive group). The recommendations regarding the return to sports varied widely and followed current guidelines in 45%. Most patients did not receive an exercise test before returning to sports.
Sports before the onset of myocarditis was not associated with a more severe outcome. There is still a discrepancy between current literature and actual recommendations given by health care providers. The fact that most participants did not receive an exercise test before being cleared for sports represents a serious omission.
Right ventricular hypertrophy (RVH) occurs in high pressure afterload, e.g., tetralogy of Fallot/pulmonary stenosis (TOF/PS). Such RVH is associated with alterations in energy metabolism, ...neurohormonal and epigenetic dysregulation (e.g., microRNA), and fetal gene reprogramming in animal models. However, comprehensive expression profiling of competing endogenous RNA in human RVH has not been performed. Here, we unravel several previously unknown circular, long non-coding, and microRNAs, predicted to regulate expression of genes specific to human RVH in the non-failing heart (TOF/PS). These genes are significantly overrepresented in pathways related to regulation of glucose and lipid metabolism (SIK1, FABP4), cell surface interactions (THBS2, FN1), apoptosis (PIK3IP1, SIK1), extracellular matrix composition (CTGF, IGF1), and other biological events. This is the first unbiased RNA sequencing study of human compensated RVH encompassing coding and non-coding RNA expression and predicted sponging of miRNAs by non-coding RNAs. These findings advance our understanding of adaptive RVH and highlight future therapeutic targets.
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•First comprehensive transcriptomic study of human RVH via RNA expression and network analysis•First human RVH study using exclusively freshly isolated myocardium•Known hypertrophy genes are regulated the strongest by competing endogenous RNA networks in RVH•Epigenetic mRNA regulation in RVH by ncRNAs is dependent on sex and age
Molecular Biology; Omics
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