Outcomes of deep hypothermic circulatory arrest in pediatric cardiac surgery: A single center experience

AbstractBackgroundDeep hypothermic circulatory arrest (DHCA) is a technique used in the repair of complex congenital cardiac lesions that require aortic arch or pulmonary vein repair. DHCA has been linked to adverse outcomes and neurologic complications. Selective cerebral perfusion (SCP) may be added to DHCA to prevent neurological complication. Air embolism and hyperperfusion injury may be encountered. The aim of this study was to evaluate the safety and efficacy of simple DHCA and to outline the early outcomes especially the neurological ones.MethodsTwenty nine patients underwent surgical repair of congenital cardiac lesion with DHCA at a single institution from January 2010 to November 2015. DHCA was conducted with a target esophageal temperature of 18° and placement of an ice pack on the head. No selective perfusion was done. Demographic, operative and postoperative data were reviewed. Mortality, any neurological complications including seizers, coma, and stroke were recorded.ResultsThe mean age was 20.6 ± 8.2 months (range: 9 days to 154 months). The majority were males (20, 69%). The mean weight was 5.57 ± 4.2 kg (range: 2.3–17.5 kg). DHCA time was 20.03 min (range 3–52 min). There were three (10.3%) deaths. Two deaths occurred after Norwood operation, and one after interrupted aortic arch repair. None of the deaths were related to neurological injury. None of the patients developed seizers, coma, abnormal movement or neurological deficits.ConclusionsSimple DHCA without SCP is a safe, expeditious and reliable method for brain protection during repair of complex cardiac lesions, with acceptable outcomes

Case complexity scores in congenital heart surgery: A comparative study of the Aristotle Basic Complexity score and the Risk Adjustment in Congenital Heart Surgery (RACHS-1) system

ObjectiveThe Aristotle Basic Complexity score and the Risk Adjustment in Congenital Heart Surgery system were developed by consensus to compare outcomes of congenital cardiac surgery. We compared the predictive value of the 2 systems.MethodsOf all index congenital cardiac operations at our institution from 1982 to 2004 (n = 13,675), we were able to assign an Aristotle Basic Complexity score, a Risk Adjustment in Congenital Heart Surgery score, and both scores to 13,138 (96%), 11,533 (84%), and 11,438 (84%) operations, respectively. Models of in-hospital mortality and length of stay were generated for Aristotle Basic Complexity and Risk Adjustment in Congenital Heart Surgery using an identical data set in which both Aristotle Basic Complexity and Risk Adjustment in Congenital Heart Surgery scores were assigned. The likelihood ratio test for nested models and paired concordance statistics were used.ResultsAfter adjustment for year of operation, the odds ratios for Aristotle Basic Complexity score 3 versus 6, 9 versus 6, 12 versus 6, and 15 versus 6 were 0.29, 2.22, 7.62, and 26.54 (P < .0001). Similarly, odds ratios for Risk Adjustment in Congenital Heart Surgery categories 1 versus 2, 3 versus 2, 4 versus 2, and 5/6 versus 2 were 0.23, 1.98, 5.80, and 20.71 (P < .0001). Risk Adjustment in Congenital Heart Surgery added significant predictive value over Aristotle Basic Complexity (likelihood ratio χ2 = 162, P < .0001), whereas Aristotle Basic Complexity contributed much less predictive value over Risk Adjustment in Congenital Heart Surgery (likelihood ratio χ2 = 13.4, P = .009). Neither system fully adjusted for the child’s age. The Risk Adjustment in Congenital Heart Surgery scores were more concordant with length of stay compared with Aristotle Basic Complexity scores (P < .0001).ConclusionsThe predictive value of Risk Adjustment in Congenital Heart Surgery is higher than that of Aristotle Basic Complexity. The use of Aristotle Basic Complexity or Risk Adjustment in Congenital Heart Surgery as risk stratification and trending tools to monitor outcomes over time and to guide risk-adjusted comparisons may be valuable

Conventional and sutureless techniques for management of the pulmonary veins: Evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies

ObjectiveWe have previously reported a limited but favorable experience with a novel sutureless technique for surgical management of postoperative pulmonary vein stenosis occurring after repair of total anomalous pulmonary venous drainage. Because this technique requires integrity of the retrocardiac space for hemostasis, extension of the technique to the primary repair of pulmonary vein anomalies requires evaluation. This analysis reviews our experience with the sutureless technique in patients with postrepair pulmonary vein stenosis, as well as our extension of the technique into primary repair of pulmonary vein anomalies.MethodsRetrospective univariable-multivariable analysis of all pulmonary vein stenosis procedures and sutureless pulmonary vein procedures over a 20-year period was performed. Cox proportional hazards modeling was used to identify variables associated with freedom from reoperation or death.ResultsSixty patients underwent 73 procedures, with pulmonary vein stenosis present in 65 procedures. The sutureless technique was used in 40 procedures. Freedom from reoperation or death at 5 years after the initial procedure was 49%. Unadjusted freedom from reoperation or death was greater with the sutureless technique for patients with postrepair pulmonary vein stenosis (P = .04). By using multivariable analysis, a higher pulmonary vein stenosis score was associated with greater risk of reoperation or death. After adjustment, the sutureless repair was associated with a nonsignificant trend toward greater freedom from reoperation or death (P = .12). Despite the absence of retrocardiac adhesions, operative mortality was not increased with the sutureless technique (P = .64). Techniques to control bleeding (intrapleural hilar reapproximation) and improve exposure (inferior vena cava division) were identified.ConclusionThe sutureless technique for postrepair pulmonary vein stenosis is associated with encouraging midterm results. Extension of the indications for the technique to primary repair appears safe with the development of simple intraoperative maneuvers

Induced pluripotent stem cell modelling of HLHS underlines the contribution of dysfunctional NOTCH signalling to impaired cardiogenesis

Hypoplastic left heart syndrome (HLHS) is among the most severe forms of congenital heart disease. Although the consensus view is that reduced flow through the left heart during development is a key factor in the development of the condition, the molecular mechanisms leading to hypoplasia of left heart structures are unknown. We have generated induced pluripotent stem cells (iPSC) from five HLHS patients and two unaffected controls, differentiated these to cardiomyocytes and identified reproducible in vitro cellular and functional correlates of the HLHS phenotype. Our data indicate that HLHS-iPSC have a reduced ability to give rise to mesodermal, cardiac progenitors and mature cardiomyocytes and an enhanced ability to differentiate to smooth muscle cells. HLHS-iPSC-derived cardiomyocytes are characterised by a lower beating rate, disorganised sarcomeres and sarcoplasmic reticulum and a blunted response to isoprenaline. Whole exome sequencing of HLHS fibroblasts identified deleterious variants in NOTCH receptors and other genes involved in the NOTCH signalling pathway. Our data indicate that the expression of NOTCH receptors was significantly downregulated in HLHS-iPSC-derived cardiomyocytes alongside NOTCH target genes confirming downregulation of NOTCH signalling activity. Activation of NOTCH signalling via addition of Jagged peptide ligand during the differentiation of HLHS-iPSC restored their cardiomyocyte differentiation capacity and beating rate and suppressed the smooth muscle cell formation. Together, our data provide firm evidence for involvement of NOTCH signalling in HLHS pathogenesis, reveal novel genetic insights important for HLHS pathology and shed new insights into the role of this pathway during human cardiac developmen

Custodiol versus blood cardioplegia in pediatric cardiac surgery, two-center study

Background: Cold blood cardioplegia is widely used as a method of myocardial protection in pediatric cardiac surgery. Operative interruption to administer cardioplegia and need for repeated administration and occasional direct coronary ostial cannulation are considered a drawbacks of this technique. Custodiol, a crystalloid solution, has been used in children as an alternative cardioplegia solution with the potential advantage of single dose administration with equivalent myocardial protection. We sought to compare the outcomes of cold blood cardioplegia versus Custodiol cardioplegia in pediatric cardiac surgery. Methods: A retrospective cohort study was performed between November 1st, 2013 and June 30th, 2014. All children who underwent heart surgery at two institutions with the use of cardiopulmonary bypass and cardioplegia were included. Patients were identified from a prospective database and additional data were collected from operative report and electronic and paper charts. Continuous data were represented as median and interquartile range, and tested with Wilcoxon rank-sum test. Categorical data were represented as proportions, and tested with Pearson test. A composite endpoint of all cause death, Low Cardiac Output Syndrome (LCOS), Acute Kidney Injury (AKI), and significant arrhythmia was analyzed with a multiple logistic regression model adjusted for complexity using the Risk Adjustment of Congenital Heart Surgery -1 (RACHS-1) categories. A p-value of less than 0.05 was considered to be significant. Results: Blood cardioplegia was administered in 88 (57.1%) patients, and Custodiol cardioplegia was administered in 66 (42.9%) patients according to surgeon's preference. In the risk adjusted comparison of the composite outcome of all-cause death, LCOS, AKI and significant arrhythmia, Custodiol cardioplegia was found to be an independent predictor of an adverse outcome, OR 3.17 (95% CI 1.41–7.14, P-value = 0.0054). Conclusions: Custodial cardioplegia is associated with less optimal myocardial protection and higher adverse outcomes compared to cold blood cardioplegia in children undergoing cardiac surgery. A randomized comparison is warranted

Survival outcomes after rescue extracorporeal cardiopulmonary resuscitation in pediatric patients with refractory cardiac arrest

ObjectivesWe report our experience with extracorporeal cardiopulmonary resuscitation with extracorporeal membrane oxygenation in children having cardiac arrest refractory to conventional cardiopulmonary resuscitation and explore predictors for favorable outcome (survival with grossly intact neurologic status).MethodsWe reviewed all patients who required extracorporeal cardiopulmonary resuscitation from 2000 to 2005. Multivariable regression analysis determined factors associated with favorable outcome and time-related survival.ResultsEighty children, median age 150 days (range: 1 day–17.6 years), required venoarterial extracorporeal cardiopulmonary resuscitation. There were several categories of disease among the patients: postcardiotomy (n = 39), unoperated congenital heart disease (n = 17), cardiomyopathy (n = 12), respiratory failure (n = 9), or myocarditis (n = 3). Cannulation sites were neck (n = 45) or chest (n = 36). Median duration of extracorporeal membrane oxygenation was 4 days (range: 1–22). Extracorporeal membrane oxygenation was successfully discontinued in 42 (54%) patients: wean (n = 35), heart transplantation (n = 7). Survival till hospital discharge was 27 (34%) patients. Most common cause of death was ischemic brain injury (n = 17). Twenty-four (30%) patients had a favorable outcome. Median duration of cardiopulmonary resuscitation for patients with favorable versus unfavorable outcome was 46 minutes (range: 14–95; interquartile range: 29–55) versus 41 minutes (range: 19–110; interquartile range: 30–55), P = .916. According to the logistic regression model, none of the following factors was a significant predictor of favorable outcome: age, weight, sex, etiology (cardiac vs noncardiac), duration of cardiopulmonary resuscitation, cannulation site, timing, or location of extracorporeal membrane oxygenation institution.ConclusionsAcceptable survival and neurologic outcomes (30%) can be achieved with extracorporeal cardiopulmonary resuscitation in children after prolonged cardiac arrest (up to 95 minutes) refractory to conventional resuscitation measures. Heart transplantation is often needed for successful extracorporeal cardiopulmonary resuscitation exit strategy. Lack of predictors of poor outcome support aggressive attempts to initiate extracorporeal cardiopulmonary resuscitation in all patients, followed by subsequent assessment of organ salvage

The influence of age and weight on the outcomes of complete atrioventricular septal defect repair

Abstract Background The appropriate age and weight for surgical repair of atrioventricular septal defect (AVSD) is an area of controversy. We aimed to study the effect of weight and age at the time of surgical repair for complete AVSD in children less than 2 years of age on postoperative outcomes. A retrospective data review was performed for patients who underwent the AVSD repair from 2012 to 2019 at our institutions. Our primary outcome was the postoperative in-hospital length of stay (LOS). Secondary outcomes included total positive pressure ventilation (PPV), ventilation time, maximum vasoactive–inotropic score (max VIS), and other postoperative complications. Results The study included fifty patients. The median age was 191 days, and the median weight was 4.38 kg at the time of surgery. Weight < 4 kg was associated with longer PPV time and postoperative in-hospital LOS (p value of 0.033 and 0.015, respectively). Additionally, they had higher max VIS at 24 h and 48 h than the other groups with bodyweight 4–5.9 kg or ≥ 6 kg (p value of 0.05 and 0.027, respectively). Patients with older age or lower weight at operation had a longer in-hospital LOS and total length of PPV after surgery. There were no postoperative in-hospital deaths. Conclusions Older age and lower weight at the time of surgical repair of atrioventricular septal defect could be independent predictors of prolonged postoperative in-hospital length of stay and total length of positive pressure ventilation