2 Rare Syndromes in 1 Patient: Determining the Cause of Sudden Cardiac Arrest.

A 19-year-old man survived sudden cardiac arrest caused by ventricular fibrillation during physical activity. The initial suspicion that this was caused by electrolyte imbalance proved to be wrong. Cardiac computed tomography revealed congenital heart disease. Coronary imaging is an essential component of the comprehensive diagnostic workup after sudden cardiac arrest.

Aortic uncrossing of the left circumflex aorta.

The following video tutorial presents the surgical correction of the left circumflex aortic arch in a 6-month-old boy with severe respiratory distress and stridor. The diagnosis was confirmed using cardiac catheterization and computed tomography. Intraoperative bronchoscopy showed marked compression of the trachea. An operation was planned to translocate the aortic arch anteriorly and to close the atrial septal defect. After a median sternotomy, the mediastinal structures were carefully mobilized and dissected. The trachea was carefully mobilized and the right ligamentum arteriosum was clipped and divided. Control of the aortic arch vessels, as well as the aberrant right subclavian artery from the right descending aorta, was achieved using vessel loops. An arterial line inserted in the femoral artery was connected to the heart-lung machine. Hence the surgical procedure was undertaken in selective antegrade cerebral perfusion combined with distal body perfusion, avoiding the need for deep hypothermic arrest. Careful mobilization of the complete course of the proximal and distal sections of the circumflex arch allowed its translocation from its retro-oesophageal course. The aortic stump distal to the left subclavian artery was closed by running polypropylene suture. An appropriate site on the ascending aorta was selected to ensure tension- and torsion-free anastomoses. Postoperative bronchoscopy confirmed relief of the tracheal compression.

Model-driven survival prediction after congenital heart surgery.

OBJECTIVES: The objective of the study was to improve postoperative risk assessment in congenital heart surgery by developing a machine-learning model based on readily available peri- and postoperative parameters. METHODS: Our bicentric retrospective data analysis from January 2014 to December 2019 of established risk parameters for dismal outcome was used to train and test a model to predict postoperative survival within the first 30 days. The Freiburg training data consisted of 780 procedures; the Heidelberg test data comprised 985 procedures. STAT mortality score, age, aortic cross-clamp time and postoperative lactate values over 24 h were considered. RESULTS: Our model showed an area under the curve (AUC) of 94.86%, specificity of 89.48% and sensitivity of 85.00%, resulting in 3 false negatives and 99 false positives.The STAT mortality score and the aortic cross-clamp time each showed a statistically highly significant impact on postoperative mortality. Interestingly, a child's age was barely statistically significant. Postoperative lactate values indicated an increased mortality risk if they were either constantly at a high level or low during the first 8 h postoperatively with an increase afterwards.When considering parameters available before, at the end of and 24 h after surgery, the predictive power of the complete model achieved the highest AUC. This, compared to the already high predictive power alone (AUC 88.9%) of the STAT mortality score, translates to an error reduction of 53.5%. CONCLUSIONS: Our model predicts postoperative survival after congenital heart surgery with great accuracy. Compared with preoperative risk assessments, our postoperative risk assessment reduces prediction error by half. Heightened awareness of high-risk patients should improve preventive measures and thus patient safety.

Impact of hybrid procedure on pulmonary arterial dimensions and right ventricular load after biventricular repair.

BACKGROUND: Hybrid procedure with ductal stenting and bilateral pulmonary banding offers a temporary approach in high-risk neonates with complex congenital heart defects aiming biventricular repair. This procedure may also have negative impact concerning post-banding pulmonary stenosis resulting in right ventricular pressure load. METHODS: Between 2010 and 2021 we identified 5 patients with interrupted aortic arch and complex congenital heart defect who underwent hybrid procedure and staged biventricular repair ("hybrid-group"). Other 7 cases with interrupted aortic arch were corrected in the neonatal phase without hybrid procedure ("nonhybrid-group"). Detailed intra- and extracardiac features and surgical procedures were documented as well as pulmonary interventions during follow up. Pulmonary vessel size was assessed by diameter of left and right pulmonary artery in absolute and indexed values. RV pressure was evaluated invasively via catheterization. RESULTS: Survival in cases with hybrid procedure and staged biventricular repair was 91% for a follow-up time of 40.7 months (95% CI 26-55 months) and 100% in the non-hybrid-group. Postoperative results concerning left ventricular function showed normal LV dimensions and systolic function without relevant stenosis on distal aortic arch. Hybrid procedure was associated with impaired local pulmonary arterial diameter after debanding resulting in increased right ventricular pressure and need for interventions (number intervention per patient: hybrid group 1.7 ± 0.95, non-hybrid group 0.17 ± 0.41; P 0.003). CONCLUSIONS: Hybrid procedure in high-risk cases with interrupted aortic arch and staged biventricular repair shows good postoperative results with low perioperative mortality and normal left ventricular function. Due to potential risk of relevant pulmonary stenosis and right ventricular pressure load, follow up examinations must not only focus on left but also on the right heart.

Surgical Treatment Following Stent Angioplasty for High-Risk Neonates With Critical Coarctation of the Aorta.

BACKGROUND: Neonatal coarctation of the aorta (CoA) is primarily treated by surgical repair. However, under certain high-risk constellations, initial stent angioplasty may be considered followed by surgical repair. We report our experience with this staged approach. Methods: All patients undergoing surgical CoA repair following prior stenting at our institution between January 2011 and December 2019 were included in this retrospective analysis. The patients were classified to be at high risk because of cardiogenic shock, associated complex cardiac malformations, neonatal infection, necrotizing enterocolitis, and extracardiac conditions, respectively. Outcomes were analyzed and compared with neonates who underwent surgical CoA repair without prior stenting in the same observation period. RESULTS: Twenty-six neonates received stent implantation at a median age of 20 days (IQR 9-33 days). Subsequent surgical repair was conducted at an age of 4.2 months (IQR 3.2-6.1 months) with a median body weight of 5.6 kg (IQR 4.5-6.5 kg). Cardiopulmonary bypass was applied in 96% of cases. Extended end-to-end anastomosis was possible in 11 patients. Extended reconstruction with patch material was necessary in the remaining patients. One fatality (3.8%) occurred 33 days postoperatively. At a median follow-up of 5.2 years after initial stenting, all remaining patients were alive; 15/25 patients (60%) were free from re-intervention. Of note, re-intervention rates were comparable in neonates (n = 76) who were operated on with native CoA (28/74 patients; 38%; P = .67). CONCLUSIONS: Neonatal stent angioplasty for CoA results in increased complexity of the subsequent surgical repair. Nevertheless, this staged approach allows to bridge high-risk neonates to later surgical repair with reduced perioperative risk and acceptable midterm outcomes.

Psychosocial Impact of Congenital Heart Diseases on Patients and Their Families: A Parent's Perspective.

BACKGROUND: Currently, over 90% of children with congenital heart disease (CHD) survive into adulthood. As a consequence the psychosocial impact on children and their families has become an important outcome measure. Therefore, the goal of this study was to assess the psychosocial impact from a parent's perspective and to identify possible predictors. METHODS: We included all parents of children who underwent open-heart surgery in the years 2010 and 2011 at the Department of Cardiothoracic Surgery at University Hospital Heidelberg and invited them to complete standardized questionnaires. Psychosocial outcome was measured via parent self- and proxy reporting of family burden (Family Burden Questionnaire, FaBel), health-related quality of life (KidScreen-10), developmental problems (Five-to-Fifteen, FTF), and mental health problems (Strength and Difficulties Questionnaire, SDQ). RESULTS: In total, 113 families returned the questionnaires completely (71.5%). The Aristotle Basic Complexity score and the STAT 2020 Score overall did not predict the psychosocial impact, whereas the number of surgical operations did significantly predict psychosocial impact across all domains in this study cohort. CONCLUSIONS: These data suggest that the number of surgical operations might be a relevant predictor for the long-term psychosocial impact on families suffering from CHD and a potential connecting factor for specialized psychological support. When setting up screening instruments or support programs the entire family must be considered.

Chromatin accessibility landscape of pediatric T-lymphoblastic leukemia and human T-cell precursors.

We aimed at identifying the developmental stage at which leukemic cells of pediatric T-ALLs are arrested and at defining leukemogenic mechanisms based on ATAC-Seq. Chromatin accessibility maps of seven developmental stages of human healthy T cells revealed progressive chromatin condensation during T-cell maturation. Developmental stages were distinguished by 2,823 signature chromatin regions with 95% accuracy. Open chromatin surrounding SAE1 was identified to best distinguish thymic developmental stages suggesting a potential role of SUMOylation in T-cell development. Deconvolution using signature regions revealed that T-ALLs, including those with mature immunophenotypes, resemble the most immature populations, which was confirmed by TF-binding motif profiles. We integrated ATAC-Seq and RNA-Seq and found DAB1, a gene not related to leukemia previously, to be overexpressed, abnormally spliced and hyper-accessible in T-ALLs. DAB1-negative patients formed a distinct subgroup with particularly immature chromatin profiles and hyper-accessible binding sites for SPI1 (PU.1), a TF crucial for normal T-cell maturation. In conclusion, our analyses of chromatin accessibility and TF-binding motifs showed that pediatric T-ALL cells are most similar to immature thymic precursors, indicating an early developmental arrest.

Intermediate monocytes exhibit higher levels of TLR2, TLR4 and CD64 early after congenital heart surgery.

INTRODUCTION: Congenital heart surgery with cardiopulmonary bypass (CPB) initiates an immune response which frequently leads to organ dysfunction and a systemic inflammatory response. Complications associated with exacerbated immune responses may severely impact the postoperative recovery. The objective was to describe the characteristics of monocyte subpopulations and neutrophils at the level of pattern recognition receptors (PRR) and the cytokine response after CPB in infants. METHODS: An observational cohort study was conducted between June 2016 and June 2017 of infants < 2 years of age, electively admitted for surgical correction of acyanotic congenital heart defects using CPB. Fourteen blood samples were collected sequentially and processed immediately during and up to 48 h following cardiac surgery for each patient. Flow cytometry analysis comprised monocytic and granulocytic surface expression of CD14, CD16, CD64, TLR2, TLR4 and Dectin-1 (CLEC7A). Monocyte subpopulations were further defined as classical (CD14++/CD16-), intermediate (CD14++/CD16+) and nonclassical (CD14+/CD16++) monocytes. Plasma concentrations of 14 cytokines, including G-CSF, GM-CSF, IL-1ß, IL-1RA, IL-4, IL-6, IL-8, IL-10, IL-12p40, IL-12p70, TNF-α, IFN-γ, MIP-1ß (CCL4) and TGF-ß1, were measured using multiplex immunoassay for seven points in time. RESULTS: Samples from 21 infants (median age 7.4 months) were analyzed by flow cytometry and from 11 infants, cytokine concentrations were measured. Classical and intermediate monocytes showed first receptor upregulation with an increase in CD64 expression four hours post CPB. CD64-expression on intermediate monocytes almost tripled 48 h post CPB (p < 0.0001). TLR4 was only increased on intermediate monocytes, occurring 12 h post CPB (p = 0.0406) along with elevated TLR2 levels (p = 0.0002). TLR4 expression on intermediate monocytes correlated with vasoactive-inotropic score (rs = 0.642, p = 0.0017), duration of ventilation (rs = 0.485, p = 0.0259), highest serum creatinine (rs = 0.547, p = 0.0102), postsurgical transfusion (total volume per kg bodyweight) (rs = 0.469, p = 0.0321) and lowest mean arterial pressure (rs = -0.530, p = 0.0135). Concentrations of IL-10, MIP-1ß, IL-8, G-CSF and IL-6 increased one hour post CPB. Methylprednisolone administration in six patients had no significant influence on the studied surface receptors but led to lower IL-8 and higher IL-10 plasma concentrations. CONCLUSIONS: Congenital heart surgery with CPB induces a systemic inflammatory process including cytokine response and changes in PRR expression. Intermediate monocytes feature specific inflammatory characteristics in the 48 h after pediatric CPB and TLR4 correlates with poorer clinical course, which might provide a potential diagnostic or even therapeutic target.

Depth of anesthesia by Narcotrend® and postoperative characteristics in children undergoing cardiac surgery under extracorporeal circulation: a retrospective comparison of two anesthetic regimens.

BACKGROUND: Depth of anesthesia may be insufficient in pediatric cardiac anesthesia if a total intravenous anesthetic regimen with opioids and midazolam is used during cardiopulmonary bypass. The advantages of sevoflurane-based balanced anesthesia may be (1) a more graduated regulation of the depth of anesthesia during cardiopulmonary bypass and (2) a reduction in postoperative ventilation time for children in comparison with total intravenous anesthesia. AIM: To evaluate a possibly positive effect of sevoflurane-based balanced anesthesia in children undergoing cardiac surgery we analyzed whether this anesthetic regimen had a significant effect related to (1) depth of anesthesia, (2) the need for opioids during cardiopulmonary bypass as well as on postoperative characteristics such as (3) time of postoperative ventilation, and (4) duration of stay in the intensive care unit in comparison with total intravenous anesthesia. METHODS: In a retrospective analysis, data from heart-lung machine protocols from 2013 to 2016 were compared according to anesthetic regimen (sevoflurane-balanced anesthesia, n = 70 vs. total intravenous anesthesia, n = 65). Children (age: 8 weeks to 14 years) undergoing cardiac surgery with cardiopulmonary bypass were included. As a primary outcome measure, we compared Narcotrend® system-extracted data to detect insufficient phases of anesthetic depth during extracorporeal circulation under moderate hypothermia. Postoperatively, we measured the postoperative ventilation time and the number of days in the intensive care unit. Furthermore, we analyzed patients' specific characteristics such as opioid consumption during cardiopulmonary bypass. Regression analysis relating primary objectives was done using the following variables: anesthetic regimen, age, severity of illness/surgery, and cumulative dosage of opiates during cardiopulmonary bypass. RESULTS: No significant differences were observed in descriptive patient characteristics (age, body weight, height, and body temperature) between the two groups. Further, no significant differences were found in depth of anesthesia by analyzing phases of superficial B1-C2-electroencephalography Narcotrend® data. No marked difference between the groups was observed for the duration of postoperative intensive care unit stay. However, the postoperative ventilation time (median (95% CI, hours)) was significantly lower in the sevoflurane-based balanced anesthesia group (6.0 (2.0-15.0)) than in the total intravenous anesthesia group (13.5 (7.0-25)). A higher dosage of opioids and midazolam was required in the total intravenous anesthesia group to maintain adequate anesthesia during cardiopulmonary bypass. Regression analysis showed an additional, significant impact of the following factors: severity of illness and severity grade of cardiac surgery (according to Aristotle) on the primary endpoint. CONCLUSION: In children undergoing cardiac surgery in our department, the use of sevoflurane-balanced anesthesia during cardiopulmonary bypass showed no superiority of inhalational agents over total intravenous anesthesia with opioids and benzodiazepines preventing phases of superficial anesthesia, but a marked advantage for the postoperative ventilation time compared with total intravenous anesthesia.