Purpose
Cardiopulmonary exercise testing (CPET) in preschoolers (4–6 years) represents a challenge. Most studies investigating CPET have been limited to older children (> 8 year). However, knowledge ...of the performance of small children is essential for evaluating their cardiorespiratory fitness. This study strives to compare a modified Bruce protocol with a new age-appropriate incremental CPET during natural movement running outdoors, using a mobile device.
Methods
A group of 22 4–6-year-old healthy children was tested indoor on a treadmill (TM) using the modified Bruce protocol. The results were compared with a self-paced incremental running test, using a mobile CPET device in an outdoor park. The speeds were described as (1) slow walking, (2) slow running, (3) regular running, and (4) running with full speed as long as possible.
Results
Mean exercise time outdoors (6,57 min) was significantly shorter than on the treadmill (11,20 min),
V
˙
O
2
p
e
a
k
(51.1 ml/min/kg vs. 40.1 ml/min/kg), RER (1.1 vs. 0.98) and important CPET parameters such as
V
˙
E
max
, O
2
pulse, heart rate and breath rate were significantly higher outdoors. The submaximal parameter OUES was comparable between both the tests.
Conclusions
Testing very young children with a mobile device is a new alternative to treadmill testing. With a significantly shorter test duration, significantly higher values for almost all cardiopulmonary variables can be achieved without losing the ability to determine VT1 and VT2. It avoids common treadmill problems and allows for individualized exercise testing. The aim is to standardize exercise times with individual protocols instead of standardizing protocols with individual exercise times, allowing for better comparability.
Introduction
Neutrophil‐to‐lymphocyte ratio (NLR), platelet‐to‐lymphocyte ratio (PLR), and lymphocyte‐to‐monocyte ratio (LMR) are emerging biomarkers for systemic inflammation and have been shown to ...predict morbidity and mortality for several diseases. However, lack of pediatric reference intervals (RIs) prevents their comprehensive use in patient care and medical research.
Material and Methods
We calculated reference intervals and corresponding confidence intervals for NLR, PLR, and LMR from birth to 18 years using a data‐mining approach: We analyzed 232 746 blood counts from 60 685 patients performed during patient care and excluded patients with elevated C‐reactive protein and procalcitonin. Test results were separated according to age and sex, and the distribution of physiological ratios was estimated using an indirect approach (refineR). Additionally, we investigated the ratios’ diagnostic benefit for different inflammatory diseases (acute appendicitis, asthma, Bell's palsy, Henoch‐Schonlein purpura, and cystic fibrosis) using the newly obtained reference intervals.
Results
We estimated age‐ and sex‐specific reference intervals from birth to adulthood for NLR, PLR, and LMR. Analyses in pediatric inflammatory diseases showed that PLR and LMR were poor markers to detect the examined inflammatory diseases, while NLR was significantly increased in patients with appendicitis and asthma.
Conclusion
We provide pediatric reference intervals for NLR, PLR, and LMR to improve the interpretation of these biomarkers in children.
Microplegia in paediatric hearts Münch, Frank; Kwapil, Nicola; Teske, Andreas ...
Perfusion,
11/2023, Letnik:
38, Številka:
8
Journal Article
Recenzirano
Odprti dostop
Introduction
A basic prerequisite for a good surgical outcome in heart surgery is optimal myocardial protection. However, cardioplegia strategies used in adult cardiac surgery are not directly ...transferable to infant hearts. Paediatric microplegia, analogous to Calafiore cardioplegia used in adult cardiac surgery, offers the advantage of safe myocardial protection without haemodilution. The use of concentration-dependent paediatric microplegia is new in clinical implementation.
Material and methods
Paediatric microplegia has been in clinical use in our institution since late 2014. It is applied via an 1/8 inch tube of a S5-HLM roller pump (LivaNova, Italy). As cardioplegic additive, a mixture of potassium (K) 20 mL (2 mmol/mL potassium chloride 14.9% Braun) and magnesium (Mg) 10 mL (4 mmol/mL Mg-sulphate Verla® i. v. 50%) is fixed into a syringe-pump (B. Braun, Germany). This additive is mixed with arterial patient blood from the oxygenator in different flow
dependent ratios to form an effective cardioplegia.
Technique
After microplegia application of initially 25 mmol/L K with 11 mmol/L Mg for 2 min, a safe cardioplegic cardiac arrest is achieved, which after release of the coronary circulation, immediately returns to a spontaneous cardiac-rhythm. In the case of prolonged aortic clamping, microplegia is repeated every 20 min with a reduction of the application dose of K by 20% and Mg by 30% (20 mmol/L K; 8.5 mmol/L Mg) and a further reduction down to a maintenance dose (15 mmol/L K; 6 mmol/L Mg) after additional 20 min.
Summary
The microplegia adapted to the needs of paediatric myocardium is convincing due to its simple technical implementation for the perfusionist while avoiding haemodilution. However, the required intraoperative interval of microplegia of approx. 20 min demands adapted intraoperative management from the surgeon.
This paper aims to assess the usability and advantages of three-dimensional rotational angiography (3DRA) in patients with congenital heart disease (CHD) and its application in the cath lab. Up to ...now, its use in CHD is not widespread or standardized. We analyzed all patients with CHD who underwent a 3DRA at our facility between January 2010 and May 2019. The 3DRAs were evaluated for radiation exposure, contrast dye consumption, diagnostic utility and image quality. We performed 872 3DRAs. 3DRA was used in 67.1% of the cases for interventional procedures and in 32.9% for diagnostic purposes. Two different acquisition programs were applied. The median dose-area product (DAP) for all 872 rotations was 54.1 µGym
(21.7-147.5 µGym
) and 1.6 ml/kg (0.9-2.07 ml/kg) of contrast dye was used. Diagnostic utility of the generated 3D-model was rated superior to the native 3D angiography in 94% (819/872). 3DRA is an excellent and save diagnostic and interventional tool. However, 3DRA has not become a standard imaging procedure in pediatric cardiology up to now. Effort and advantage seems to be unbalanced, but new less invasive techniques may upgrade this method in future.
Numerous genetic studies have established a role for rare genomic variants in Congenital Heart Disease (CHD) at the copy number variation (CNV) and de novo variant (DNV) level. To identify novel ...haploinsufficient CHD disease genes, we performed an integrative analysis of CNVs and DNVs identified in probands with CHD including cases with sporadic thoracic aortic aneurysm. We assembled CNV data from 7,958 cases and 14,082 controls and performed a gene-wise analysis of the burden of rare genomic deletions in cases versus controls. In addition, we performed variation rate testing for DNVs identified in 2,489 parent-offspring trios. Our analysis revealed 21 genes which were significantly affected by rare CNVs and/or DNVs in probands. Fourteen of these genes have previously been associated with CHD while the remaining genes (FEZ1, MYO16, ARID1B, NALCN, WAC, KDM5B and WHSC1) have only been associated in small cases series or show new associations with CHD. In addition, a systems level analysis revealed affected protein-protein interaction networks involved in Notch signaling pathway, heart morphogenesis, DNA repair and cilia/centrosome function. Taken together, this approach highlights the importance of re-analyzing existing datasets to strengthen disease association and identify novel disease genes and pathways.
Cinematic rendering (CR) is based on a new algorithm that creates a photo-realistic three-dimensional (3D) picture from cross-sectional images. Previous studies have shown its positive impact on ...preoperative planning. To date, CR presentation has only been possible on 2D screens which limited natural 3D perception. To depict CR-hearts spatially, we used mixed-reality technology and mapped corresponding hearts as holograms in 3D space. Our aim was to assess the benefits of CR-holograms in the preoperative planning of cardiac surgery. Including 3D prints allowed a direct comparison of two spatially resolved display methods. Twenty-six patients were recruited between February and September 2019. CT or MRI was used to visualize the patient's heart preoperatively. The surgeon was shown the anatomy in cross-sections on a 2D screen, followed by spatial representations as a 3D print and as a high-resolution hologram. The holographic representation was carried out using mixed-reality glasses (HoloLens®). To create the 3D prints, corresponding structures were segmented to create STL files which were printed out of resin. In 22 questions, divided in 5 categories (3D-imaging effect, representation of pathology, structure resolution, cost/benefit ratio, influence on surgery), the surgeons compared each spatial representation with the 2D method, using a five-level Likert scale. The surgical preparation time was assessed by comparing retrospectively matched patient pairs, using a paired
-test. CR-holograms surpassed 2D-monitor imaging in all categories. CR-holograms were superior to 3D prints in all categories (mean Likert scale 4.4 ± 1.0 vs. 3.7 ± 1.3,
< 0.05). Compared to 3D prints it especially improved the depth perception (4.7 ± 0.7 vs. 3.7 ± 1.2) and the representation of the pathology (4.4 ± 0.9 vs. 3.6 ± 1.2). 3D imaging reduced the intraoperative preparation time (
= 24, 59 ± 23 min vs. 73 ± 43 min,
< 0.05). In conclusion, the combination of an extremely photo-realistic presentation via cinematic rendering and the spatial presentation in 3D space via mixed-reality technology allows a previously unattained level of comprehension of anatomy and pathology in preoperative planning.
Primary surgical repair remains the traditional treatment for patients with critical duct-dependent coarctation of the aorta (CoA). Initial surgical repair might not be possible or associated with ...higher risks if additional comorbidities arise in small infants and neonates. Balloon angioplasty (BA) has been described as a rescue strategy for these children. We describe the feasibility of a palliative BA and rescue stent implantation via an alternative antegrade right-axillary artery approach in an initially inoperable infant with pneumonia and respiratory failure and severe CoA, where the stenosis was not passable by traditional retrograde femoral access. This case adds new aspects to the therapy of critical CoA: Stent implantation provides a bridge to surgery in critically ill infants and does not preclude successful surgical repair. Further, if the classic retrograde approach is not possible, the right axillary artery access should be considered as an alternative to pass the stenosis.
Lymphatic congestion in single-ventricle patients has been associated with increased morbidity and poor outcomes. Little is known about the dynamics of lymphatic abnormalities over time, on their ...association with clinical presentation or response to catheter interventions. This retrospective, single-center study describes Fontan patients who underwent at least two magnetic resonance imaging (MRI) studies. T2-weighted lymphatic imaging was used to classify thoracic and abdominal (para-aortic and portal-venous) lymphatic abnormalities. The relationship between lymphatic congestion and hemodynamic changes after cardiac catheter interventions, clinical presentation and MRI data was analyzed. A total of 33 Fontan patients underwent at least two cardiac MRI studies. Twenty-two patients had two, eight had three and three had four lymphatic imaging studies (total of 80 MRIs studies). No significant changes in lymphatic classification between MRI 1 and 2 were observed for thoracic (
= 0.400), para-aortic (0.670) and portal-venous (
= 0.822) abnormalities. No significant correlation between lymphatic classification and hemodynamic changes after intervention or MRI parameters was found. This study illustrates thoracic and abdominal lymphatic abnormalities in serial T2-weighted imaging after Fontan. Fontan patients did not demonstrate significant changes in their lymphatic perfusion, despite clinical or hemodynamic changes. We assume that lymphatic congestion might develop after total cavopulmonary connection (TCPC) and remain relatively stable, despite further intervention targeting hemodynamic parameters.
Rare genetic variants in KDR, encoding the vascular endothelial growth factor receptor 2 (VEGFR2), have been reported in patients with tetralogy of Fallot (TOF). However, their role in disease ...causality and pathogenesis remains unclear.
We conducted exome sequencing in a familial case of TOF and large-scale genetic studies, including burden testing, in >1,500 patients with TOF. We studied gene-targeted mice and conducted cell-based assays to explore the role of KDR genetic variation in the etiology of TOF.
Exome sequencing in a family with two siblings affected by TOF revealed biallelic missense variants in KDR. Studies in knock-in mice and in HEK 293T cells identified embryonic lethality for one variant when occurring in the homozygous state, and a significantly reduced VEGFR2 phosphorylation for both variants. Rare variant burden analysis conducted in a set of 1,569 patients of European descent with TOF identified a 46-fold enrichment of protein-truncating variants (PTVs) in TOF cases compared to controls (P = 7 × 10-11).
Rare KDR variants, in particular PTVs, strongly associate with TOF, likely in the setting of different inheritance patterns. Supported by genetic and in vivo and in vitro functional analysis, we propose loss-of-function of VEGFR2 as one of the mechanisms involved in the pathogenesis of TOF.
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