Prospective evaluation of acute neurological events after paediatric cardiac surgery.

OBJECTIVES: Children with CHD are at heightened risk of neurodevelopmental problems; however, the contribution of acute neurological events specifically linked to the perioperative period is unclear. AIMS: This secondary analysis aimed to quantify the incidence of acute neurological events in a UK paediatric cardiac surgery population, identify risk factors, and assess how acute neurological events impacted the early post-operative pathway. METHODS: Post-operative data were collected prospectively on 3090 consecutive cardiac surgeries between October 2015 and June 2017 in 5 centres. The primary outcome of analysis was acute neurological event, with secondary outcomes of 6-month survival and post-operative length of stay. Patient and procedure-related variables were described, and risk factors were statistically explored with logistic regression. RESULTS: Incidence of acute neurological events after paediatric cardiac surgery in our population occurred in 66 of 3090 (2.1%) consecutive cardiac operations. 52 events occurred with other morbidities including renal failure (21), re-operation (20), cardiac arrest (20), and extracorporeal life support (18). Independent risk factors for occurrence of acute neurological events were CHD complexity 1.9 (1.1-3.2), p = 0.025, longer operation times 2.7 (1.6-4.8), p < 0.0001, and urgent surgery 3.4 (1.8-6.3), p < 0.0001. Unadjusted comparison found that acute neurological event was linked to prolonged post-operative hospital stay (median 35 versus 9 days) and poorer 6-month survival (OR 13.0, 95% CI 7.2-23.8). CONCLUSION: Ascertainment of acute neurological events relates to local measurement policies and was rare in our population. The occurrence of acute neurological events remains a suitable post-operative metric to follow for quality assurance purposes.

Prophylactic corticosteroids for paediatric heart surgery with cardiopulmonary bypass.

BACKGROUND: Corticosteroids are routinely given to children undergoing cardiac surgery with cardiopulmonary bypass (CPB) in an attempt to ameliorate the inflammatory response. Their use is still controversial and the decision to administer the intervention can vary by centre and/or by individual doctors within that centre. OBJECTIVES: This review is designed to assess the benefits and harms of prophylactic corticosteroids in children between birth and 18 years of age undergoing cardiac surgery with CPB. SEARCH METHODS: We searched CENTRAL, MEDLINE, Embase and Conference Proceedings Citation Index-Science in June 2020. We also searched four clinical trials registers and conducted backward and forward citation searching of relevant articles. SELECTION CRITERIA: We included studies of prophylactic administration of corticosteroids, including single and multiple doses, and all types of corticosteroids administered via any route and at any time-point in the perioperative period. We excluded studies if steroids were administered therapeutically. We included individually randomised controlled trials (RCTs), with two or more groups (e.g. multi-drug or dose comparisons with a control group) but not 'head-to-head' trials without a placebo or a group that did not receive corticosteroids. We included studies in children, from birth up to 18 years of age, including preterm infants, undergoing cardiac surgery with the use of CPB. We also excluded studies in patients undergoing heart or lung transplantation, or both; studies in patients already receiving corticosteroids; in patients with abnormalities of the hypothalamic-pituitary-adrenal axis; and in patients given steroids at the time of cardiac surgery for indications other than cardiac surgery. DATA COLLECTION AND ANALYSIS: We used the Covidence systematic review manager to extract and manage data for the review. Two review authors independently assessed studies for inclusion, extracted data, and assessed risks of bias. We resolved disagreements by consensus or by consultation with a third review author. We assessed the certainty of evidence with GRADE. MAIN RESULTS: We found 3748 studies, of which 888 were duplicate records. Two studies had the same clinical trial registration number, but reported different populations and interventions. We therefore included them as separate studies. We screened titles and abstracts of 2868 records and reviewed full text reports for 84 studies to determine eligibility. We extracted data for 13 studies. Pooled analyses are based on eight studies. We reported the remaining five studies narratively due to zero events for both intervention and placebo in the outcomes of interest. Therefore, the final meta-analysis included eight studies with a combined population of 478 participants. There was a low or unclear risk of bias across the domains. There was moderate certainty of evidence that corticosteroids do not change the risk of in-hospital mortality (five RCTs; 313 participants; risk ratio (RR) 0.83, 95% confidence interval (CI) 0.33 to 2.07) for children undergoing cardiac surgery with CPB. There was high certainty of evidence that corticosteroids reduce the duration of mechanical ventilation (six RCTs; 421 participants; mean difference (MD) 11.37 hours lower, 95% CI -20.29 to -2.45) after the surgery. There was high-certainty evidence that the intervention probably made little to no difference to the length of postoperative intensive care unit (ICU) stay (six RCTs; 421 participants; MD 0.28 days lower, 95% CI -0.79 to 0.24) and moderate-certainty evidence that the intervention probably made little to no difference to the length of the postoperative hospital stay (one RCT; 176 participants; mean length of stay 22 days; MD -0.70 days, 95% CI -2.62 to 1.22). There was moderate certainty of evidence for no effect of the intervention on all-cause mortality at the longest follow-up (five RCTs; 313 participants; RR 0.83, 95% CI 0.33 to 2.07) or cardiovascular mortality at the longest follow-up (three RCTs; 109 participants; RR 0.40, 95% CI 0.07 to 2.46). There was low certainty of evidence that corticosteroids probably make little to no difference to children separating from CPB (one RCT; 40 participants; RR 0.20, 95% CI 0.01 to 3.92). We were unable to report information regarding adverse events of the intervention due to the heterogeneity of reporting of outcomes. We downgraded the certainty of evidence for several reasons, including imprecision due to small sample sizes, a single study providing data for an individual outcome, the inclusion of both appreciable benefit and harm in the confidence interval, and publication bias. AUTHORS' CONCLUSIONS: Corticosteroids  probably do not change the risk of mortality for children having heart surgery using CPB at any time point. They probably reduce the duration of postoperative ventilation in this context, but have little or no effect on the total length of postoperative ICU stay or total postoperative hospital stay. There was inconsistency in the adverse event outcomes reported which, consequently, could not be pooled. It is therefore impossible to provide any implications and policy-makers will be unable to make any recommendations for practice without evidence about adverse effects. The review highlighted the need for well-conducted RCTs powered for clinical outcomes to confirm or refute the effect of corticosteroids versus placebo in children having cardiac surgery with CPB. A core outcome set for adverse event reporting in the paediatric major surgery and intensive care setting is required.

Coronary Stenosis and Cardiogenic Shock Secondary to Aortitis Following Aortic Root Support Procedure.

A woman with recent personalized external aortic root support implant presented in cardiogenic shock with bilateral coronary ostial occlusion and aortic inflammation requiring emergency coronary angioplasty. Subsequent computed tomography with positron emission tomography scanning demonstrated aortitis with extensive inflammation adjacent to the personalized external aortic root support mesh, the first report of this important complication.

Arterial Switch for Transposition of the Great Arteries: Treatment Timing, Late Outcomes, and Risk Factors.

Background: Reports of long-term mortality and reintervention after transposition of the great arteries with intact ventricular septum treatment, although favorable, are mostly limited to single-center studies. Even less is known about hospital resource utilization (days at hospital) and the impact of treatment choices and timing on outcomes. Objectives: The purpose of this study was to describe survival, reintervention and hospital resource utilization after arterial switch operation (ASO) in a national dataset. Methods: Follow-up and life status data for all patients undergoing ASO between 2000 and 2017 in England and Wales were collected and explored using multivariable regressions and matching. Results: A total of 1,772 patients were identified, with median ASO age of 9.5 days (IQR: 6.5-14.5 days). Mortality and cardiac reintervention at 10 years after ASO were 3.2% (95% CI: 2.5%-4.2%) and 10.7% (95% CI: 9.1%-12.2%), respectively. The median time spent in hospital during the ASO spell was 19 days (IQR: 14, 24). Over the first year after the ASO patients spent 7 days (IQR: 4-10 days) in hospital in total, decreasing to 1 outpatient day/year beyond the fifth year. In a subgroup with complete risk factor data (n = 652), ASO age, and balloon atrial septostomy (BAS) use were not associated with late mortality and reintervention, but cardiac or congenital comorbidities, low weight, and circulatory/renal support at ASO were. After matching for patient characteristics, BAS followed by ASO and ASO as first procedure, performed within the first 3 weeks of life, had comparable early and late outcomes, including hospital resource utilization. Conclusions: Mortality and hospital resource utilization are low, while reintervention remains relatively frequent. Early ASO and individualized use of BAS allows for flexibility in treatment choices and a focus on at-risk patients.

Hospital resource utilization in a national cohort of functionally single ventricle patients undergoing surgical treatment.

Objective: The study objective was to provide a detailed overview of health resource use from birth to 18 years old for patients with functionally single ventricles and identify associated risk factors. Methods: All patients with functionally single ventricles treated between 2000 and 2017 in England and Wales were linked to hospital and outpatient records using data from the Linking AUdit and National datasets in Congenital HEart Services project. Hospital stay was described in yearly age intervals, and associated risk factors were explored using quantile regression. Results: A total of 3037 patients with functionally single ventricles were included, 1409 (46.3%) undergoing a Fontan procedure. During the first year of life, the median days spent in hospital was 60 (interquartile range, 37-102), mostly inpatient days, mirroring a mortality of 22.8%. This decreases to between 2 and 9 in-hospital days/year afterward. Between 2 and 18 years, most hospital days were outpatient, with a median of 1 to 5 days/year. Lower age at the first procedure, hypoplastic left heart syndrome/mitral atresia, unbalanced atrioventricular septal defect, preterm birth, congenital/acquired comorbidities, additional cardiac risk factors, and severity of illness markers were associated with fewer days at home and more intensive care unit days in the first year of life. Only markers of early severe illness were associated with fewer days at home in the first 6 months after the Fontan procedure. Conclusions: Hospital resource use in functionally single ventricle cases is not uniform, decreasing 10-fold during adolescence compared with the first year of life. There are subsets of patients with worse outcomes during their first year of life or with persistently high hospital use throughout their childhood, which could be the target of future research.

Allogeneic Mesenchymal Stromal Cell Injection to Alleviate Ischemic Heart Failure Following Arterial Switch Operation.

Cell therapy is a promising tool to prevent and treat heart failure in congenital heart disease. We report the first case of intramyocardial injection of allogeneic mesenchymal stromal cells as rescue therapy in a neonate with ischemic heart failure following arterial switch procedure for isolated transposition of the great arteries. (Level of Difficulty: Advanced.).

Adult coronary artery bypass grafting by congenital surgeons-a propensity matched analysis.

OBJECTIVES: Surgical myocardial revascularization will be increasingly needed in adult patients with congenital heart disease. We investigated the results of coronary artery bypass grafting (CABG) performed on adults by congenital cardiac surgeons at our institution. METHODS: We conducted a retrospective, single-centre study. Adults undergoing isolated or combined CABG from 2004 to 2017 were included. Early and late outcomes were analyzed for the whole cohort. Furthermore, a propensity matched analysis was conducted comparing the results of isolated CABG between congenital and adult surgeons. RESULTS: A total of 514 and 113 patients had isolated and combined CABG for acquired heart disease, respectively. A total of 33 patients had myocardial revascularization at the time of surgery for congenital heart disease. Overall early mortality was 1.2%, the rate of re-exploration for bleeding was 4.5%, and an internal mammary artery to left anterior descending artery graft was used in 85.6% patients. One-year survival was 97.5% (96.2-98.8%), and 5-year survival was 88.0% (84.8-91.3%). After propensity matching (468 pairs), early mortality (0.6% vs 1.2%, P = 0.51), re-exploration for bleeding (3.6% vs 3.0%, P = 0.72), use of internal mammary artery to left anterior descending artery graft (92.7% vs 91.9%, P = 0.70) and late survival did not differ between congenital surgeons and adult surgeons, respectively. CONCLUSIONS: Surgical myocardial revascularization can be required for adult congenital patients in a broad spectrum of clinical situations. Despite lower volumes, congenital cardiac surgeons perform CABG safely and with results that are comparable to those of the adult surgeons at our centre.

Factors associated with unplanned reinterventions and their relation to early mortality after pediatric cardiac surgery.

OBJECTIVE: Unplanned reintervention (uRE) is used as an indicator of patient morbidity and quality of care in pediatric cardiac surgery. We investigated associated factors and early mortality after uREs. METHODS: Morbidity data were prospectively collected in 5 UK centers between 2015 and 2017; uRE included surgical cardiac, interventional transcatheter cardiac, permanent pacemaker, and diaphragm plication procedures. Mortality (30-day and 6-month) in uRE/no-uRE patients was reported before and after matching. Predicted 30-day mortality was calculated using the Partial Risk Adjustment in Surgery score. RESULTS: A total of 3090 procedures (2861 patients) were included (median age, 228 days). There were 146 uREs, resulting in an uRE rate of 4.7%. Partial Risk Adjustment in Surgery score, 30-day mortality and 6-month mortality in uRE and no-uRE groups were 2.4% versus 1.3%, 8.9% versus 1%, and 17.1% versus 2.4%, respectively. After matching, mortality at 6 months remained higher in uRE compared with no-uRE (12.2% vs 1.4%; P = .02; 74 pairs). In the uRE group, 21 out of 25 deaths at 6 months occurred when at least 1 additional postoperative complication was present. In multivariable analysis, neonatal age (P = .002), low weight (P = .009), univentricular heart (P < .001), and arterial shunt (P < .001) were associated with increased risk of uRE, but Partial Risk Adjustment in Surgery score was not (only in univariable analysis). CONCLUSIONS: uREs are a relatively frequent complication after pediatric cardiac surgery and are associated with some patient characteristics, but not the Partial Risk Adjustment in Surgery risk score. Early mortality was higher after uRE, independent of preoperative factors, but linked to other postoperative complications.

Outcomes following aortic valve procedures in 201 complex congenital heart disease cases-results from the UK National Audit.

OBJECTIVES: Some patients with complex congenital heart disease (cCHD) also require aortic valve (AoV) procedures. These cases are considered high risk but their outcome has not been well characterized. We aim to describe these scenarios in the current practice, and provide outcome data for counselling and decision-making. METHODS: This was a retrospective study using the UK National Congenital Heart Disease Audit data on cCHD patients undergoing aortic valve replacement, balloon dilation (balloon aortic valvuloplasty) or surgical repair (surgical aortic valve repair) between 2000 and 2012. Coarsened exact matching was used to pair cCHD with patients undergoing AoV procedures for isolated valve disease. RESULTS: A total of 201 patients with a varied spectrum of cCHD undergoing 242 procedures were included, median age 9.4 years (1 day-65 years). Procedure types were: balloon aortic valvuloplasty (n = 31, 13%), surgical aortic valve repair (n = 57, 24%) and aortic valve replacement (n = 154, 63%). Mortality at 30 days was higher in neonates (21.8% vs 5.3%, P = 0.02). Survival at 10 years was 83.1%, freedom from aortic valve replacement 83.8% and freedom from balloon aortic valvuloplasty/surgical aortic valve repair 86.3%. Neonatal age (P < 0.001), single ventricle (P = 0.08), concomitant Fontan/Glenn (P = 0.002) or aortic arch procedures (0.02) were associated with higher mortality. cCHD patients had lower survival at 30 days (93% vs 100%, P = 0.003) and at 10 years (86.4% vs 96.1%, P = 0.005) compared to matched isolated AoV disease patients. CONCLUSIONS: AoV procedures in cCHD can be performed with good results outside infancy, but with higher mortality than in isolated AoV disease. Neonates and patients with single ventricle defects, especially those undergoing concomitant Fontan/Glenn, have worse outcomes.

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.

OBJECTIVES: Many adults with repaired tetralogy of Fallot will require a pulmonary valve replacement (PVR), but there is no consensus on the best timing. In this study, we aim to evaluate the impact of age at PVR on outcomes. METHODS: This is a national multicentre retrospective study including all patients >15 years of age with repaired tetralogy of Fallot who underwent their first PVR between 2000 and 2013. The optimal age cut-off was identified using Cox regression and classification and regression tree analysis. RESULTS: A total of 707 patients were included, median age 26 (15-72) years. The mortality rate at 10 years after PVR was 4.2%, and the second PVR rate of 6.8%. Age at PVR of 35 years was identified as the optimal cut-off in relation to late mortality. Patients above 35 years of age had a 5.6 fold risk of death at 10 years compared with those with PVR under 35 years (10.4% vs 1.3%, P < 0.001), more concomitant tricuspid valve repair/replacement (15.1% vs 5.7%, P < 0.001) and surgical arrhythmia treatment (18.4% vs 5.9%, P < 0.001). In those under 50 years, there was an 8.7 fold risk of late death compared with the general population, higher for those with PVR after 35 than those with PVR below 35 years (hazard ratio 9.9 vs 7.4). CONCLUSIONS: Patients above 35 years of age with repaired tetralogy of Fallot have significantly worse mortality after PVR, compared with younger patients and a higher burden of mortality relative to the general population. This suggests that there are still cases where the timing of initial PVR is not optimal, warranting a re-evaluation of criteria for intervention.

MicroRNAs as potential biomarkers in congenital heart surgery.

OBJECTIVE: Pediatric congenital heart surgery (CHS) involves intracardiac, valvular, and vascular repairs. Accurate tools to aid short-term outcome prediction in pediatric CHS are lacking. Clinical scores, such as the vasoactive-inotrope score and ventilation index, are used to define outcome in clinical studies. MicroRNA-1-3p (miR-1) is expressed by both cardiomyocytes and vascular cells and is regulated by hypoxia. In adult patients, miR-1 increases in the circulation after open-heart cardiac surgery, suggesting its potential as a clinical biomarker. Thus, we investigated whether perioperative circulating miR-1 measurements can help predict post-CHS short-term outcomes in pediatric patients. METHODS: Plasma miR-1 was retrospectively measured in a cohort of 199 consecutive pediatric CHS patients (median age 1.2 years). Samples were taken before surgery and at the end of the operation. Plasma miR-1 concentration was measured by reverse transcription-quantitative polymerase chain reaction and expressed as miR-1 copies/µL and as relative expression to spiked-in exogenous cel-miR-39. RESULTS: Baseline plasma miR-1 did not vary across different diagnoses, increased during surgery (204-fold median relative increase, P < .001), and was associated with aortic crossclamp duration postoperatively (P < .001). Importantly, miR-1 levels at the end of the operation positively correlated with intensive care stay (P < .001), early severe cardiovascular events (P = .01), and with high vasoactive-inotrope score (P = .001) and ventilation index (P < .001), suggesting that miR-1 could accelerate the identification of patients with cardiopulmonary bypass-related ischemic complications, requiring more intensive support. CONCLUSIONS: Our study suggests miR-1 as a novel potential circulating biomarker to predict early postoperative outcome and inform clinical management in pediatric heart surgery.

Long-term results of heart operations performed by surgeons-in-training.

BACKGROUND: We investigated the association between trainees performing supervised operations and late outcomes of patients undergoing cardiac surgery. METHODS AND RESULTS: Data were prospectively collected on patients who underwent coronary artery bypass graft surgery, aortic valve replacement, or a combination of these between 1998 and 2005 at the Maritime Heart Center, Halifax, Canada. In-hospital mortality and a composite outcome of in-hospital mortality, stroke, bleeding, intra-aortic balloon pump insertion, renal failure, and sternal infection was compared between teaching (n=1054) and nonteaching cases (n=5877). Late survival and cardiovascular hospital readmissions were also examined. To adjust for baseline risk disparities, we used logistic regression for dichotomous in-hospital outcomes and Cox proportional hazards regression for survival data. Resident cases were significantly more likely to have high-risk features such as depressed ventricular function, redo operation, and urgent or emergent procedure. Resident as primary operator was not independently associated with in-hospital mortality (OR, 1.09; 95% CI, 0.75 to 1.58; P=0.66) or with the composite outcome (OR, 1.01; 95%, CI 0.82 to 1.26; P=0.90). The Kaplan-Meier event-free survival of the 2 groups was equivalent at 1, 3, and 5 years (log-rank P=0.06). By Cox regression, resident cases were not associated with late death or cardiovascular rehospitalization (hazard ratio, 1.05; 95% CI, 0.94 to 1.17; P=0.42). CONCLUSIONS: Cases performed by senior-level cardiac surgery residents were more likely to have greater acuity and complexity than staff surgeon-performed cases. However, clinical outcomes were similar in the short- and long-term. Allowing residents to perform cardiac surgery is not associated with adverse patient outcomes.

Surgical Repair of Tetralogy of Fallot With Absent Pulmonary Valve: Favorable Long-Term Results.

Tetralogy of Fallot with absent pulmonary valve syndrome (ToF-APVS) is a rare variant of tetralogy, associated with severe pulmonary valve regurgitation and aneurysmal dilation of the pulmonary arteries (PAs). Reported outcomes after surgical corrections are limited to single center, older series and might not reflect the current outcome. We aim to use data from a national registry to evaluate short- and long-term outcomes after surgical repair of ToF-APVS, to serve for counselling and planning. All children undergoing ToF-APVS repair in the UK between 2002 and 2013 were included. Survival and freedom from reintervention were estimated using the Kaplan-Meier method, and univariable analysis was done using the Weibull regression model. A total of 98 children, 45% male, 10% with DiGeorge syndrome, median age of 213 days (1 day to 13 years) were included. Mortality at 30 days was 3.3%, higher for neonates (6.7% vs 2.7%, P = 0.4) and those on preoperative mechanical ventilatory support (16.7% vs 1.3%, P = 0.04). Survival was 92.1% and freedom from pulmonary valve or conduit replacement (PVR) 73.2% at 10 years. Neonates had worse survival (hazard ratio [HR] 6.2, P = 0.02), freedom from PVR (HR 4.5, P = 0.01), freedom from PAs arterioplasty (HR 6.6, P = 0.001), and overall freedom from any reintervention (HR 5.3, P < 0.001). Low weight at repair was associated with worse freedom from PVR (P = 0.02) and from PAs arterioplasty (P = 0.009), preoperative ventilatory support with increased mortality (P = 0.009), the presence of DiGeorge syndrome was associated with worse freedom from PVR (HR 4, P = 0.02). Surgical repair of ToF-APVS can be performed with low early and late mortality, with improving results even in those with preoperative mechanical ventilatory support. The need for right ventricular outflow tract reintervention, including on the PAs, is an expected issue in the long term for the majority of patients.

Corticosteroids in Pediatric Heart Surgery: Myth or Reality.

Background: Corticosteroids have been administered prophylactically for more than 60 years in pediatric heart surgery, however, their use remains a matter of debate. There are three main indications for corticosteroid use in pediatric heart surgery with the use of cardiopulmonary bypass (CPB): (1) to blunt the systemic inflammatory response (SIRS) induced by the extracorporeal circuit; (2) to provide perioperative supplementation for presumed relative adrenal insufficiency; (3) for the presumed neuroprotective effect during deep hypothermic circulatory arrest operations. This review discusses the current evidence behind the use of corticosteroids in these three overlapping areas. Materials and Methods: We conducted a structured research of the literature using PubMed and MEDLINE databases to November 2017 and additional articles were identified by cross-referencing. Results: The evidence suggests that there is no correlation between the effect of corticosteroids on inflammation and their effect on clinical outcome. Due to the limitations of the available evidence, it remains unclear if corticosteroids have an impact on early post-operative outcomes or if there are any long-term effects. There is a limited understanding of the hypothalamic-pituitary-adrenal axis function during cardiac surgery in children. The neuroprotective effect of corticosteroids during deep hypothermic circulatory arrest surgery is controversial. Conclusions: The utility of steroid administration for pediatric heart surgery with the use of CPB remains a matter of debate. The effect on early and late outcomes requires clarification with a large multicenter randomized trial. More research into the understanding of the adrenal response to surgery in children and the effect of corticosteroids on brain injury is warranted.

Primary repair versus surgical and transcatheter palliation in infants with tetralogy of Fallot.

OBJECTIVES: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study. METHODS: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated. RESULTS: Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09). CONCLUSIONS: We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.

Aortic Valve Replacement and the Ross Operation in Children and Young Adults.

BACKGROUND: There are several options available for aortic valve replacement (AVR), with few comparative reports in the literature. The optimal choice for AVR in each age group is not clear. OBJECTIVES: The study sought to report and compare outcomes after AVR in the young using data from a national database. METHODS: AVR procedures were compared after advanced matching, both in pairs and in a 3-way manner, using a Bayesian dynamic survival model. RESULTS: A total of 1,501 patients who underwent AVR in the United Kingdom between 2000 and 2012 were included. Of these, 47.8% had a Ross procedure, 37.8% a mechanical AVR, 10.9% a bioprosthesis AVR, and 3.5% a homograft AVR, with Ross patients being significantly younger when compared to the other groups. Overall survival at 12 years was 94.6%. In children, the Ross procedure had a 12.7% higher event-free probability (death or any reintervention) at 10 years when compared to mechanical AVR (p = 0.05). We also compared all procedures except the homograft in a matched population of young adults, where the bioprosthesis had the lowest event-free probability of 78.8%, followed by comparable results in mechanical AVR and Ross, with 86.3% and 89.6%, respectively. Younger age was associated with mortality and pulmonary reintervention in the Ross group and with aortic reintervention in the mechanical AVR. Of all 3 options, only the patients undergoing the Ross procedure approached the survival of the general population. CONCLUSIONS: AVR in the young achieves good results, with the Ross being overall better suited for this age group, especially in children. Although freedom from aortic valve reintervention is superior after the Ross procedure, the need for homograft reinterventions is an issue to take into account. All methods have advantages and limitations, with reinterventions being an issue in the long term for all, more crucially in smaller children.

Endothelial activation in the transplanted human heart from organ retrieval to 3 months after transplantation: an observational study.

BACKGROUND: Endothelial activation in the donor heart has been described variably after brain death and transplantation. We aimed to characterize the time course of endothelial activation in right ventricle (RV) and left ventricle (LV) during the acute phase of clinical transplantation. METHODS: We studied biopsy specimens from the RVs and the LVs of 40 donor hearts: at initial assessment of the donor, at end-ischemia, and after 10 minutes of reperfusion. We also included follow-up RV biopsy specimens at 1 week, 1 month, and 3 months after surgery. Six of the patients had cystic fibrosis and were domino donors. RESULTS: P-selectin and vascular cell adhesion molecule 1 (VCAM-1), but not E-selectin were up-regulated in brain-dead and in domino donors vs controls. Unused donor hearts (n = 6) had significantly less up-regulation of P-selectin and of VCAM-1. We found no difference between the RV and the LV during surgery, but we did see important time-dependent variations. P-selectin was present in 85% of vessels throughout transplantation and decreased to approximately 60% after transplantation (p < 0.001). We initially detected VCAM-1 in 20% of vessels, which decreased to 5% during storage, then increased to 47% at reperfusion, and gradually decreased thereafter (p < 0.001). E-selectin expression increased progressively from 15% initially to 45% at reperfusion and then decreased after surgery (p = 0.001). Thrombomodulin expression was decreased at baseline, and the decrease was accentuated afterward (p = 0.02). Patients with donor organ failure did not have a specific pattern of endothelial activation. CONCLUSION: Cardiac transplantation is associated with marked endothelial activation, with no difference between the two ventricles. The changes persist in the post-operative period, even in the absence of acute rejection.

Anomalous Left Coronary From the Pulmonary Artery Presenting as Ventricular Fibrillation After Persistent Ductus Arteriosus Ligation.

An anomalous left coronary artery from the pulmonary artery (ALCAPA) is rarely associated with persistent ductus arteriosus (PDA). A large PDA can maintain perfusion in the left coronary artery, delaying presentation. Assessing the origin of the coronary arteries before PDA ligation is difficult, often being performed in very small or even preterm babies. We present the case of a 5-month-old infant with echocardiographic features of mitral regurgitation and subendocardial ischemia who experienced ischemia and cardiac arrest after PDA ligation. Transesophageal echocardiography demonstrated ALCAPA, and left coronary translocation was performed. The infant was discharged after 10 days.

Noradrenaline use in the human donor and relationship with load-independent right ventricular contractility.

BACKGROUND: Experimental and clinical studies suggest that brain death (BD)-associated cardiac dysfunction is related to the neurohormonal storm and subsequent exposure to intravenous catecholamines. We aimed to describe the relationship between empirical noradrenaline treatment and donor heart function, described for the first time with load-independent indices of right ventricular contractility. METHODS: Twenty-seven BD patients were divided in three groups based on noradrenaline at time of offer, started by the donor hospital: group 1=no noradrenaline (n=11); group 2=low dose (n=8); group 3=high dose (n=8). After protocol-guided optimization by our retrieval team using a Swan-Ganz catheter, pressure-volume data were obtained from the right ventricle. Ten patients undergoing coronary revascularization served as controls. RESULTS: Twenty hearts were transplanted, seven of them as heart and lung blocks. Right ventricular end-systolic elastance (E(es)) was lower in BD donors compared with controls (mean 0.28 vs. 0.46 mm Hg/mL, P< or =0.01). There was no difference in terms of Swan-Ganz derived data between the BD subgroups, but E(es) was lower in groups 2 and 3 (P=0.04). Eight patients died within a year (four from graft failure), and they had a donor heart E(es) significantly lower than that of survivors (mean 0.20 vs. 0.33 mm Hg/mL, P=0.01). CONCLUSION: Hearts from BD donors have subclinical right ventricular impairment in contractility. E(es), a load-independent measure of contractile function, seems to be inversely correlated with empirical use of noradrenaline in the donor and with recipient survival at 1 year. This has implications for refining donor selection and management.