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Background: For an optimal interplay based on the data-secure exchange of diagnostic data between patients, ambulatory care, and hospital care, we implemented the first tele-medical network for pediatric cardiology in Germany, the Congenital Cardiology Cloud. This study focuses on its feasibility, technical characteristics, and implementation in routine clinical work. Methods: Tele-medical traffic comprised numbers of incoming/outgoing data, related file types, treatment options for tele-medically processed patients, and patient classification with respect to the severity of disease. Proof of feasibility was related to the number of successful tele-medical transmissions of discharge documents at the end of the observation period (02/2020–10/2020). Results: Analysis of Congenital Cardiology Cloud's data communication showed a number of 1178 files for a total of 349 patients, favoring transmissions towards the clinic. Incoming traffic was predominantly characterized by diagnostic data regarding tele-consultations (76.6%), consisting of a multitude of file types, whereas 93.4% of the dispatched data corresponded to discharge letters. The number of tele-consultations counted up to 61, with a necessary subsequent treatment or diagnostic procedure in 90.2% of the presented cases. Tele-medically processed patients generally showed to be more complex (severe chronic heart disease 42.4% vs. 23.7%). At the end of the observation period, 97.6% of the discharge documents were transmitted via telemedicine. Discussion: The implementation of the first tele-medical network for pediatric cardiology in Germany proves recent technological developments to successfully enable innovative patient care, connecting the ambulatory and hospital sector for a joint patient advice, predominantly in more complex cases. Possible governmentally guided refinancing concepts will show its long-term feasibility. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Complications after Fontan surgery have been associated with arise and classification of abnormal thoracic lymphatic perfusion pattern. This study compiles abnormal abdominal lymphatic perfusion patterns and investigates their impact on serum protein readings. METHODS We performed a retrospective analysis of patients who underwent magnetic resonance imaging with T2-weighted lymphatic imaging and serum protein measurements 6 months after having Fontan surgery. The abdominal lymphatic images were classified according to the anatomical lymphatic drainage patterns into 2 categories: (1) para-aortic (types 1–4); (2) portal-venous (types 1–3). Thoracic lymphatic images were classified (types 1–4) as described earlier. RESULTS A total of 71 patients were included in the study. Para-aortic lymphatic perfusion patterns were classified as type 1 in 4, type 2 in 13, type 3 in 37 and type 4 in16 out of 71 patients. Portal-venous lymphatic perfusion patterns were classified as type 1 in 20, type 2 in 10 and type 3 in 41 patients. Thoracic lymphatic perfusion patterns were classified as type 1 in 8, type 2 in 11, type 3 in 39 and type 4 in 13 patients. The serum protein level was 66 (interquartile range: 7.5) g/l (< standard value in 37%). Higher-grade para-aortic (p = 0.0062), portal-venous (p = 0.022) and thoracic (p = 0.011) lymphatic abnormalities were correlated with lower total serum protein levels. Higher ratings of para-aortic lymphatic abnormalities were significantly associated with higher ratings of portal-venous abnormalities (p < 0.0001). Ratings of para-aortic and portal-venous classifications were correlated with the thoracic classification (p < 0.001). CONCLUSIONS Abnormal abdominal lymphatic perfusion patterns can be classified according to anatomical structures with increasing severity. Higher grade abdominal and thoracic lymphatic perfusion patterns are associated with lower serum protein values. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Primary repair of common arterial trunk (CAT) is burdened by high mortality rates, especially in the presence of multiple risk factors. Timing, possible palliative methods, optimal management of associated cardiac lesions and handling of a poor preoperative state are still under discussion. METHODS We retrospectively analysed all patients who underwent surgery for CAT in our institution between 2008 and November 2020. We included 22 patients, 11 of whom received primary correction (PC) and 11 of whom underwent initial palliation by partial repair, leaving the ventricular septal defect open and connecting the right ventricle to the pulmonary arteries with a small valveless right ventricle-to-pulmonary artery conduit. A delayed correction (DC) was performed after 11.5 ± 3.6 months. RESULTS The overall operative mortality was 4.5%; 1 patient (affected by severe truncal valve stenosis and presenting in a poor state preoperatively) in the DC group died after palliation. The incidence of postoperative pulmonary hypertensive crisis was significantly higher in the PC group (P = 0.027). No patient from either group required postoperative extracorporeal support. Survival rates after 6 years differed slightly (PC group, 90%; DC group, 70%; log-rank = 0.270). CONCLUSIONS PC of CAT remains an optimal surgical approach for patients with an expected low mortality. However, our data support palliation and DC as a suitable alternative strategy, especially in the presence of significant risk factors like interrupted aortic arch, poor preoperative condition or complex surgical anatomy. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Aortic coarctation with distal aortic arch hypoplasia can be effectively addressed by coarctation resection with extended end-to-end-anastomosis (REEEA). Particularly, when unilateral cerebral perfusion (UCP) is established by clamping of left-sided supra-aortic vessels, the extent of cerebral blood flow distribution during repair remains undetermined, so far. Transfontanellar contrast-enhanced ultrasound (T-CEUS) can be utilized for real-time visualization and quantitative evaluation of cerebral blood flow. This study quantitatively evaluates cerebral perfusion during REEEA by using intraoperative T-CEUS. METHODS In a prospective study, 9 infants with open fontanelle undergoing REEEA [median age: 13 days (range 1–34) and median weight 3.1 kg (range 2.2–4.4)] were intraoperatively examined with T-CEUS at 3 consecutive time-points: before skin incision, during UCP and after skin suture. A software-based analysis of 11 parameters was used for data evaluation. Absolute and relative blood flow in contralateral hemispheres was measured in side-by-side comparison, and referenced to baseline measurements. RESULTS No side-depend absolute or relative cerebral perfusion differences were found during REEEA, except for an increased relative 'wash-out-rate' (P  =   0.0013) in favour of the right hemisphere after surgery. Compared to ipsilateral baseline levels, 'rise time' was transiently increased in right (P  =   0.0277) and 'time-to-peak' in both hemispheres (right: P  =   0.0403 and left: P  =   0.0286), all during UCP. CONCLUSIONS The use of T-CEUS provided evidence for homogenous distribution of contrast agent in both hemispheres during UCP. T-CEUS can be utilized for the postprocedural evaluation of cerebral perfusion during congenital cardiac surgery. Clinical Trial Registration URL: http://www.clinicaltrials.gov Unique, Identifier: NCT03215628. [ABSTRACT FROM AUTHOR]

Dendritic cells in overdrive: Upon detection of danger signals, such as molecules associated with infectious pathogens, dying cells, or tumor cells, antigen-presenting cells called dendritic cells initiate adaptive immune responses. This response is enhanced through inflammasome signaling that induces cytokine secretion but usually leads to death of the activated cells. Hatscher et al. analyzed human primary DCs from the blood, spleen, and thymus and found that type 2 conventional dendritic cells (cDC2) uniquely did not die after inflammasome-induced cytokine secretion. Instead, these cells entered a "hyperactive" state that enhanced the stimulation of certain T helper cell subsets. The findings suggest that cDC2 could be critical to the efficacy of vaccines and immunotherapies as well as for therapeutically controlling inflammation. The detection of microorganisms and danger signals by pattern recognition receptors on dendritic cells (DCs) and the consequent formation of inflammasomes are pivotal for initiating protective immune responses. Although the activation of inflammasomes leading to secretion of the cytokine IL-1β is typically accompanied by pyroptosis (an inflammatory form of lytic programmed cell death), some cells can survive and exist in a state of hyperactivation. Here, we found that the conventional type 2 DC (cDC2) subset is the major human DC subset that is transcriptionally and functionally poised for inflammasome formation and response without pyroptosis. When cDC2 were stimulated with ligands that relatively weakly activated the inflammasome, the cells did not enter pyroptosis but instead secreted IL-12 family cytokines and IL-1β. These cytokines induced prominent T helper type 1 (TH1) and TH17 responses that were superior to those seen in response to Toll-like receptor (TLR) stimulation alone or to stronger, classical inflammasome ligands. These findings not only define the human cDC2 subpopulation as a prime target for the treatment of inflammasome-dependent inflammatory diseases but may also inform new approaches for adjuvant and vaccine development. [ABSTRACT FROM AUTHOR]

OBJECTIVES Open in new tab Download slide Open in new tab Download slide Left superior vena cava (LSVC)-related obstruction of mitral inflow is a rare finding in patients with complex cardiac anomalies like hypoplastic left heart complex. We report our experience by establishing a left superior to right superior caval vein continuity (innominate vein creation by direct LSVC–right superior vena cava end-to-side-anastomosis), and coronary sinus unroofing if indicated for LSVC-related mitral inflow obstruction. METHODS Nineteen patients (median age: 1.0 ± 0.3 years; range: 7 days–4.8 years) underwent anatomical correction of LSVC without the use of foreign material in conjunction with repair or palliation of congenital anomalies in a single centre between April 2015 and November 2019. Indications for the procedure were LSVC-related obstruction of left ventricular inflow due to a dilated coronary sinus. Additional procedures included mitral (n  = 7) or atrioventricular (n  = 3) valve surgery, right ventricular to pulmonary artery conduit (n  = 3), first stage palliation (n  = 3) or biventricular repair (n  = 5) of hypoplastic left heart complex. Three patients needed secondary mitral valve replacement (n  = 3). RESULTS All LSVC or coronary sinus-related obstructions were effectively relieved. No patient died early, 2 patients died late after the procedure. One patient needed stenting of the superior vena cava below the unobstructed cephalad vein anastomosis at the former right superior vena cava-cannulation-site. Follow-up was complete and demonstrated an 89.5% survival after 2.5 ± 0.4 years. Innominate vein patency was 100% documented by echocardiography (n  = 19), cardiac catheterization (n  = 6) or both. Mean mitral valve z -scores before the operation were −1.7 ± 0.2 (range −3.8 to 0.3) and increased to 0.7 ± 0.2 (range −0.7 to 1.9) after LSVC repair. CONCLUSIONS Anatomical correction by surgical creation of an innominate vein is an effective method to relieve LSVC-related obstructions and promotes mitral valvar growth. Mitral ring sizes were at least normalized after surgery at the time of discharge. Further prospective follow-up studies to evaluate the growth potential of left-sided heart structures by reporting cardiac z -scores are needed to evaluate the true impact of coronary sinus unroofing. [ABSTRACT FROM AUTHOR]

Introduction: Plastic can be toxic and hazardous to an organism's health, but it is being widely used in our daily lives. Di-2-ethylhexyl-phthalate is the most common plasticizer in medical devices made of polyvinylchloride and is commonly found in soft bags storing red blood cell units. Di-2-ethylhexyl-phthalate and its degradation product mono-2-ethylhexyl-phthalate can migrate into human body fluids, for example, blood and tissues. The aim of the study was to assess the concentration of plasticizers in red blood cell units according to storage time and after mechanical rinsing using a cell salvage device. Methods: Levels of di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate were analysed in 50 unwashed red blood cell units using liquid chromatography coupled with tandem mass spectrometry. In addition, phthalate concentrations were measured before and after mechanical rinsing in six more washed red blood cell units with storage times ranging between 36 and 56 days. A linear regression model was determined by the daily increase of di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate in the stored red blood cell units subject to their storage time (range = 4-38 days), and the effect of mechanical rinsing on their phthalate concentration was calculated. Results: A linear correlation was found between storage time of unwashed red blood cell units and the concentration of di-2-ethylhexyl-phthalate (p < 0.001) or mono-2-ethylhexyl-phthalate (p < 0.001). Stored red blood cell units older than 14 days had significantly higher concentrations of both contaminants than red blood cell units of shorter storage time (p < 0.001). Mechanical rinsing in washed red blood cell units attained a reduction in the di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate concentration by a median of 53% (range = 18-68%; p = 0.031) and 87% (range = 68-96%; p = 0.031), respectively. Conclusion: Leaching of di-2-ethylhexyl-phthalate and mono-2-ethylhexyl-phthalate into red blood cell units depends on the duration of storage time. Plasticizers can be significantly reduced by mechanical rinsing using cell salvage devices, and thus, red blood cell units can be regenerated with respect to chemical contamination. [ABSTRACT FROM AUTHOR]

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