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Background: Acute kidney injury (AKI) following cardiopulmonary bypass (CPB) is associated with increased morbidity and mortality. Serum Cystatin C (CysC) is a novel biomarker synthesized by all nucleated cells that may act as an early indicator of AKI following infant CPB. Methods: Prospective observational study of infants (< 1 year) requiring CPB during cardiac surgery. CysC was measured at baseline and 12, 24, 48, and 72 hours following CPB initiation. Each post-op percent difference in CysC (e.g. %CysC12hr) from baseline was calculated. Clinical variables along with urine output (UOP) and serum creatinine (SCr) were followed. Subjects were divided into two groups: AKI and non-AKI based upon the Kidney Disease Improving Global Outcomes (KDIGO) classification. Results: AKI occurred in 41.9% (18) of the 43 infants enrolled. Patient demographics and baseline CysC levels were similar between groups. CysC levels were 0.97 ± 0.28 mg/L over the study period, and directly correlated with SCr (R = 0.71, p < 0.0001). Although absolute CysC levels were not significant between groups, the %CysC12hr was significantly greater in the AKI group (AKI: -16%±22% vs. Non-AKI -28%±9% mg/L; p=0.003). However, multivariate analysis demonstrated that a lower UOP (Odds Ratio:0.298; 95% CI:0.073, 0.850; p=0.02) but not %CysC12hr was independently associated with AKI. Conclusions: Despite a significant difference in the %CysC12hr, only UOP was independently associated with AKI. Larger studies of a more homogenous population are needed to understand these results and to explore the variability in this biomarker seen across institutions.

Longitudinal follow-up, resource utilization, and health disparities are top congenital heart research and care priorities. Medicaid claims include longitudinal data on inpatient, outpatient, emergency, pharmacy, rehabilitation, home health utilization, and social determinants of health-including mother-infant pairs.The New York Congenital Heart Surgeons Collaborative for Longitudinal Outcomes and Utilization of Resources linked robust clinical details from locally held state and national registries from 10 of 11 New York congenital heart centers to Medicaid claims, building a novel, statewide mechanism for longitudinal assessment of outcomes, expenditures, and health inequities.The authors included all children 18 years of age undergoing cardiac surgery in The Society of Thoracic Surgeons Congenital Heart Surgery Database or the New York State Pediatric Congenital Cardiac Surgery Registry from 10 of 11 New York centers, 2006 to 2019. Data were linked via iterative, ranked deterministic matching on direct identifiers. Match rates were calculated and compared. Proportions of the linked cohort trackable over 3, 5, and 10 years were described.Of 14,097 registry cases, 59% (n = 8,322) reported Medicaid use. Of these, 7,414 were linked to New York claims, at an 89% match rate. Of matched cases, the authors tracked 79%, 74%, and 65% of children over 3, 5, and 10 years when requiring near-continuous Medicaid enrollment. Allowing more lenient enrollment criteria, the authors tracked 86%, 82%, and 76%, respectively. Mortality over this time was 7.7%, 8.4%, and 10.0%, respectively. Manual validation revealed ∼100% true matches.This establishes a novel statewide data resource for assessment of longitudinal outcome, health expenditure, and disparities for children with congenital heart disease.

Background Although resting blood pressures following aortic arch repair or the extended end-to-end anastomosis (EEA) repair for coarctation can be physiologic, factors associated with an abnormal blood pressure response after exercise are unknown. We measured blood pressure gradients following exercise in children who had undergone previous repair in accordance with a surgical selection algorithm and sought to identify factors associated with an abnormal blood pressure response. Methods In accordance with our practice's surgical algorithm for repair of coarctation, infants were stratified to aortic arch repair when the distal transverse arch-to-left carotid artery ratio (DTA:LCA) ≤ 1.0, or when a brachiocephalic trunk or intra-cardiac lesion requiring repair was present. A thoracotomy and EEA were otherwise used. A follow-up exercise stress test (EST) measured the arm:leg blood pressure gradient after exercise, and a gradient ≥ 20 mm Hg was defined as an abnormal blood pressure response. Results Thirty-seven infants who had previously undergone coarctation repair (aortic arch repair-19, EEA-18) completed an EST at 12.3 ± 2.2 years of age. Thirteen (35%) children (aortic arch repair-5, EEA-8; p = .3) exhibited an abnormal blood pressure response. Factors associated with an abnormal blood pressure response included: smaller DTA:LCA ratios prior to repair (1.0 ± .2 vs. 1.2 ± .3; p = .04) and greater body weight at the time of EST (57.5 ± 19.1 vs. 40.9 ± 15.6 kg; p = .03). Conclusion An abnormal blood pressure response following exercise is associated with smaller DTA:LCA ratios at the time of repair and increased weight during follow-up suggesting that patients with these factors warrant close observation.

Background Although single ventricle physiology and cyanosis are known risk factors for neurodevelopmental delay (NDD), the impacts of isolated coarctation (Iso CoA) repair or arch reconstruction (AR) are less understood. Methods The Vineland Adaptive Behavior Scales, third edition, prospectively evaluated children without a genetic syndrome. An overall composite score, normalized to age and sex, was generated from individual domain scores. NDD was defined as a composite or domain score at least 1 SD less than the established mean. Iso CoA was repaired using a left thoracotomy, whereas AR was performed using a sternotomy and cardiopulmonary bypass. Children with a structurally normal heart and without previous surgery were used as control patients. Results Of 60 children, 50 required neonatal repair (12 for Iso CoA, 38 for AR), and 10 were control patients. From the entire cohort of children who required neonatal coarctation repair (Iso CoA + AR) composite (93.9 ± 15.9 vs 105.0 ± 7.4; P = .004) and all domain scores were significantly lower than control patients. NDD was present in 25 of 50 children after repair and in 0 of 10 control patients (P = .003). Similarly, the prevalence of NDD was significantly greater after Iso CoA repair (58.3% vs 0%; P = .005) and AR (47.3% vs 0%; P = .007) than in the control population, but no significant difference was found between the Iso CoA and AR groups (P = .74). Conclusions In this small cohort, half of the neonates who required either Iso CoA repair or AR exhibit NDD at an intermediate-term follow-up.

Background:Infants with cyanotic congenital heart disease demonstrate wide fluctuations in hemoglobin (Hb), oxygen saturation, and cardiac output following palliation. Methemoglobin (Met-Hb), the product of Hb oxidation, may represent a compensatory mechanism during hypoxia and may be utilized as a biomarker.Methods:Arterial and venous Met-Hb levels were obtained from infants requiring palliation. The primary outcome was to describe the relationship between Met-Hb and other indices of tissue oxygenation (venous saturation, estimated arteriovenous oxygen difference [Est AV-Diff], and lactate). Secondary outcomes were to determine the impact of elevated Met-Hb levels ≥1.0% and the effect of red blood cell (RBC) transfusion on Met-Hb levels.Results:Fifty infants and 465 Met-Hb values were studied. Venous Met-Hb levels were significantly higher than arterial levels (venous: 0.84% ± 0.36% vs arterial: 0.45% ± 0.18%; P < .001). Venous Met-Hb demonstrated a significant inverse relationship with venous oxygen saturation ( R = −0.6; P < .001) and Hb ( R = −0.3, P < .001) and a direct relationship with the Est AV-Diff ( R = 0.3, P < .001). A total of 129 (29.6%) venous Met-Hb values were elevated (≥1.0%) and were associated with significantly lower Hb and venous saturation levels and higher Est AV-Diff and lactate levels. Methemoglobin levels decreased significantly following 65 RBC transfusions (0.94 ± 0.40 vs 0.77 ± 0.34; P < .001). Linear mixed models demonstrated that higher venous Met-Hb levels were associated with lower measures of tissue oxygenation and not related to any preoperative clinical differences.Conclusion:Methemoglobin may be a clinically useful marker of tissue oxygenation in infants following surgical palliation.

Cell saver blood is typically washed with normal saline (NS); however, recent studies have reported decreased red blood cell hemolysis and increased platelet function when a more physiologic washing solution, such as Plasma-Lyte A (PL-A) is used. We evaluated the in vitro and in vivo effects of NS compared to PL-A as washing solutions for cell saver blood in pediatric cardiac surgery. Cell saver blood was re-infused for up to 24 hours post-collection. Laboratory and clinical data were collected from infants receiving cell saver washed with either NS (n = 20) or PL-A (n = 21). Compositions of the cell saver blood were compared between groups at 5 in vitro time points and in vivo patient blood at 24 hours post-bypass. Although there were differences in in vitro laboratory values between groups; 24 hours post-bypass, in vivo results were similar. Our data supports 24-hour reinfusion of cell saver washed with either NS versus PL-A in pediatric cardiac surgery patients, and provides data on the differences in cell saver composition to guide future studies.

Central venous catheter (CVC) related venous thrombosis (VT) after pediatric cardiac surgery increases morbidity and mortality. Although VT prevention using low-dose anticoagulation therapy has proven ineffective, anticoagulation therapy using high-dose enoxaparin to achieve a therapeutic anti-Xa level has not been studied. We hypothesized that high-dose enoxaparin would reduce VT after pediatric cardiac surgery.Enoxaparin was administered to infants aged less than 150 days when postoperative CVC duration was anticipated to extend beyond 5 days. The primary outcome was the rate of VT, reexploration for bleeding, and postoperative red blood cell transfusions per 1000 CVC days.From 2012 to 2019, 157 infants were treated with enoxaparin. Infants were divided into two groups: (1) subtherapeutic (n = 51), in which therapeutic anti-Xa level (0.5 to 1.0 IU/mL) was not achieved; and (2) therapeutic (n = 106), in which therapeutic anti-Xa level was achieved. Baseline demographics demonstrated a lower age at operation in the therapeutic group. The subtherapeutic group had a higher VT rate per 1000 CVC days (8.2) compared with the therapeutic group (2.6; P = .005). Reexploration for bleeding was similar between groups. The number of postoperative red blood cell transfusions per 1000 CVC days was significantly greater in the subtherapeutic group (109.4 vs 81.6; P = .008). Multivariate analysis demonstrated that higher median anti-Xa levels reduced the risk of VT (odds ratio 0.02; 95% confidence interval, 0.001 to 0.63; P = .02).These data suggest that enoxaparin treatment resulting in a therapeutic anti-Xa level reduces postoperative CVC-associated VT without increasing bleeding complications.

Supplemental Digital Content is available in the text., Background: De novo genic and copy number variants are enriched in patients with congenital heart disease, particularly those with extra-cardiac anomalies. The impact of de novo damaging variants on outcomes following cardiac repair is unknown. Methods: We studied 2517 patients with congenital heart disease who had undergone whole-exome sequencing as part of the CHD GENES study (Congenital Heart Disease Genetic Network). Results: Two hundred ninety-four patients (11.7%) had clinically significant de novo variants. Patients with de novo damaging variants were 2.4 times more likely to have extra-cardiac anomalies (P=5.63×10−12). In 1268 patients (50.4%) who had surgical data available and underwent open-heart surgery exclusive of heart transplantation as their first operation, we analyzed transplant-free survival following the first operation. Median follow-up was 2.65 years. De novo variants were associated with worse transplant-free survival (hazard ratio, 3.51; P=5.33×10−04) and longer times to final extubation (hazard ratio, 0.74; P=0.005). As de novo variants had a significant interaction with extra-cardiac anomalies for transplant-free survival (P=0.003), de novo variants conveyed no additional risk for transplant-free survival for patients with these anomalies (adjusted hazard ratio, 1.96; P=0.06). By contrast, de novo variants in patients without extra-cardiac anomalies were associated with worse transplant-free survival during follow-up (hazard ratio, 11.21; P=1.61×10−05) than that of patients with no de novo variants. Using agnostic machine-learning algorithms, we identified de novo copy number variants at 15q25.2 and 15q11.2 as being associated with worse transplant-free survival and 15q25.2, 22q11.21, and 3p25.2 as being associated with prolonged time to final extubation. Conclusions: In patients with congenital heart disease undergoing open-heart surgery, de novo variants were associated with worse transplant-free survival and longer times on the ventilator. De novo variants were most strongly associated with adverse outcomes among patients without extra-cardiac anomalies, suggesting a benefit for preoperative genetic testing even when genetic abnormalities are not suspected during routine clinical practice. Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT01196182.

Infant mortality after cardiac surgery is multifactorial, but can be related to center and surgeon case volume. One method to increase case volume is to consolidate or regionalize pediatric cardiac surgical care. We evaluated in-hospital and 5-year outcomes in three separate pediatric cardiac surgical programs before and after they formed one regional consortium. Infants (

Repair of truncus arteriosus often requires early right ventricular outflow tract (RVOT) reoperation. Using a modified repair, the branch pulmonary arteries are left in situ, which may avoid earlier RVOT reoperation. We hypothesized that our modified repair for type I and II truncus arteriosus would extend the time to RVOT reoperation. Infants with truncus arteriosus were divided into 2 groups: (1) traditional technique where the branch pulmonary arteries are excised from the truncal root, or (2) modified repair where the branch pulmonary arteries are left in situ and septated from the truncal root. Regardless of the approach, a bioprosthetic conduit or homograft was used to establish right ventricular to pulmonary artery continuity. Follow-up pulmonary artery angiograms were used to assess for branch pulmonary artery stenosis. From 54 infants (modified repair: 33, traditional technique: 21), there were no significant differences in age at repair, gender, or type of truncus arteriosus. With 100% follow-up, use of the modified repair resulted in a lower rate of branch pulmonary artery stenosis, and greater freedom from surgical branch pulmonary arterioplasty. Five- and 10-year freedom from RVOT reoperation (5 years: modified-81.5% vs traditional-30.5%, P = 0.004; 10 years: modified-53.3% vs traditional-30.5%, P = 0.01) favored the modified repair. Cox regression analysis demonstrated that the modified repair was associated with an independently lower risk for RVOT reoperation (hazard ratio: 0.08, confidence interval: 0.01, 0.75, P = 0.02). Thus, maintaining the branch pulmonary artery architecture resulted in greater freedom from RVOT reoperation.

Postoperative electroencephalograms (EEGs) can identify seizure activity and neurologic dysfunction in high-risk neonates requiring cardiac surgical procedures. Although intraoperative EEG monitoring is uncommon, variations in cerebral blood flow and temperature during antegrade cerebral perfusion (ACP) can manifest as cortical asynchrony during EEG monitoring. We hypothesized that intraoperative EEG cortical asynchrony would identify neonates at risk for abnormal postoperative EEG tracings.Neonates requiring ACP for cardiac repair or palliation had continuous baseline, intraoperative, and postoperative hemodynamic and EEG monitoring. Synchronous and asynchronous cortical bursts were quantified during (1) cooling before ACP, (2) ACP, and (3) rewarming. Asynchronous bursts were defined as interhemispheric variations in electrical voltage or frequency. Neonates were divided into 2 groups, those with and without an abnormal postoperative EEG, which was defined as either persistent asynchrony for more than 2 hours or seizure activity on EEG.Among 40 neonates, 296 asynchronous bursts were noted, most commonly during rewarming. Eight (20%) neonates had an abnormal postoperative EEG (seizure activity, n = 3; persistent asynchrony, n = 5). Baseline demographics and intraoperative hemodynamics were similar between the groups. However, the total number of intraoperative asynchronous bursts was greater in neonates with an abnormal postoperative EEG (17 [11, IQR:24] vs 3 [IQR:1, 7]; P.001). Multivariate analysis confirmed that the number of asynchronous bursts was independently associated with an abnormal postoperative EEG (odds ratio,1.35; confidence interval,:1.10, 1.65; P = .004).Neonates with a greater number of intraoperative asynchronous cortical bursts had an abnormal postoperative EEG.

Background There are minimal circulatory support options for patients with a failing Fontan. The Heartmate II (HMII) left ventricular assist device (Thoratec, Bedford, MA) in its packaged state cannot augment caval/pulmonary arterial blood flow. Aim We hypothesized that a modified HMII pump could augment caval and pulmonary arterial blood flow. Methods A bifurcated ringed Gore-Tex graft (W. L. Gore & Associates, Flagstaff, AZ) was sewn to the HMII inflow, and the outflow graft transected and tapered from 16 mm to 8 mm in diameter. In three sheep, the inflow and outflow grafts were anastomosed end-to-side to both cava and the pulmonary artery. Results Following baseline measurements, the pump speed was increased to 8000 revolutions per minute (RPMs). Compared to baseline, at 8000 RPMs, there were no significant differences in mean arterial, central venous, or pulmonary arterial pressure. However, there was a significant decrease in right ventricular diastolic diameter (3.1 ± 0.1 vs. 1.8 ± 0.2 cm, R = 0.6, p = 0.02) and similarly a decrease in pulmonary arterial pulse pressure (8.5 ± 2.1 vs. 2.1 ± 2.9 mmHg, p = 0.01). As pump speed increased, there was a corresponding increase in pump flow and power, with a decrease in pulsatility index. Conclusions These findings suggest that the HMII may be modified to provide caval/pulmonary circulatory support for the failing Fontan circulation.

Background: The goals following pulmonary valve replacement (PVR) are to optimize right ventricular hemodynamics and minimize the need for subsequent reoperations on the right ventricular outflow tract. We hypothesized PVR using a xenograft valved conduit would result in superior freedom from reoperation with sustained improvement in right ventricular chamber dimensions. Methods: Xenograft valved conduits placed in patients aged >16 years were reviewed from 2000 to 2010 to allow for a 5-year minimum follow-up. Preoperative, one-year, and the most recent echocardiograms quantified right ventricular chamber dimensions, corresponding Z scores, and prosthetic valve function. Magnetic resonance imaging (MRI) studies compared preoperative and follow-up right ventricular volumes. Results: A total of 100 patients underwent PVR at 24 (19-34) years. Freedom from reintervention was 100% at 10 years. At most recent follow-up, only one patient had greater than mild pulmonary insufficiency. The one-year (17.3 ± 7.2 mm Hg; P < .01) and most recent follow-up (18.6 ± 9.8 mm Hg; P < .01) Doppler-derived right ventricular outflow tract gradients remained significantly lower than preoperative measurements (36.7 ± 27.0 mm Hg). Similarly, right ventricular basal diameter, basal longitudinal diameter, and the corresponding Z scores remained lower at one year and follow-up from preoperative measurements. From 34 MRI studies, the right ventricular end-diastolic indexed volume (161.7 ± 58.5 vs 102.9 ± 38.3; P < .01) and pulmonary regurgitant fraction (38.0% ± 15.9% vs 0.8% ± 3.3%; P < .01) were significantly lower at 7.1 ± 3.4 years compared to the preoperative levels. Conclusion: Use of a xenograft valved conduit for PVR results in excellent freedom from reoperation with sustained improvement in right ventricular dimensions at an intermediate-term follow-up.

A 2-month-old male with dysmorphic facies, neonatal thrombocytopenia, left congenital cataract, and long bone abnormalities became hypotensive with ST depression on induction of anesthesia for congenital cataract repair. Echocardiogram demonstrated decreased left ventricular function (ejection fraction 46%), mild mitral valve regurgitation (MR), and regional wall motion abnormalities. The left coronary artery could not be visualized. Subsequent cardiac catheterization confirmed atresia of the left main coronary artery. The patient underwent cardiac surgery with coronary artery bypass grafting of the left internal mammary artery to the left anterior descending coronary artery. His postoperative course was uncomplicated, his ventricular function normalized, and he was discharged home. Over the next few months, he developed progressive, severe MR refractory to medical management. Repeat cardiac catheterization revealed stenosis of the right proximal coronary artery, raising concern for progressive coronary involvement. In addition to dysmorphic features and failure to thrive, there were profound developmental delays and a finding of vacuolated lymphocytes on blood smear, which led to a diagnosis of mucolipidosis II (Inclusion [I]-cell disease). The patient was referred for mitral valve replacement, which was successful; however, ongoing respiratory issues attributed to the progression of I-cell disease led to a prolonged hospitalization with placement of a tracheostomy. Reports of coronary anomalies in patients with I-cell disease are rare. An association between mucopolysaccharidosis syndromes and coronary artery abnormalities has been established and is supported by this case report, highlighting the importance of considering the potential for coronary artery involvement with I-cell disease and other similar storage diseases.

Background The optimal choice for pulmonary valve replacement (PVR) remains controversial. This study hypothesized that xenografts used for PVR would result in prolonged long-term survival and freedom from reoperation. Methods Children and adults with congenital heart disease requiring PVR using a xenograft from 1980 to 1985 were reviewed. In all cases, the xenograft valve was either sewn or manufactured into a Dacron conduit, and the conduit was sewn to the pulmonary artery bifurcation. Clinical data were analyzed, and survival and freedom from reoperation were determined using Kaplan-Meier analysis. Results Twenty-four patients received a xenograft for PVR at 14.6 ± 5.6 years. Conduit size ranged from 21 to 27 mm. Most patients received a Carpentier-Edwards valved conduit (n = 17), followed by a Hancock valved conduit (n = 5) and an Ionescu-Shiley valve sewn into a Dacron graft (n = 2). No perioperative deaths occurred. Reoperation was required mainly for pulmonary stenosis (72.7%), followed by pulmonary insufficiency (18.2%), or both stenosis and insufficiency (9%). Freedom from reoperation was 90%, 56%, 43%, and 14% at 10, 20, 25, and 30 years, respectively. At most recent follow-up the was only death, which was related to severe biventricular failure 25 years after conduit implant. Conclusions PVR using a xenograft valved conduit results in prolonged freedom from reoperation and excellent long-term survival. These data, which provide long-term follow-up information on xenograft valves after PVR.

Neonates with congenital heart disease are at risk for impaired neurodevelopment after cardiac surgery. We hypothesized that intraoperative EEG activity may provide insight into future neurodevelopmental outcomes.Neonates requiring surgery had continuous intraoperative EEG and hemodynamic monitoring. The level of EEG suppression was classified as either: slow and continuous; moderate burst suppression; severe burst suppression; or isoelectric (no brain activity for3 minutes). Follow-up neurodevelopmental outcomes were assessed using the Vineland Adaptive Behavior Scale II (Vineland-II).Twenty-one neonates requiring cardiac surgery developed a slow and continuous EEG pattern after general anesthesia. Ten neonates (48%) maintained continuous brain electrical activity with moderate burst suppression as the maximum level of EEG suppression. Eleven neonates (52%) developed severe burst suppression that progressed into an isoelectric state during the deep hypothermic period required for circulatory arrest. However, the duration of this state was significantly longer than circulatory arrest times (111.1 ± 50 vs 22.3 ± 17 minutes; P.001). At a mean follow-up at 5.6 ± 1.0 years, compared with neonates with continuous brain electrical activity, neonates who developed an isoelectric state had lower Vineland-II scores in communication. There was an inverse relationship between composite Vineland-II scores and duration of isoelectric activity (R = -0.75, P = .01). Of neonates who experienced an isoelectric state, durations of90 minutes were associated with the lowest Vineland-II scores (125.0 ± 2.6 vs 81.1 ± 12.7; P.01).The duration of cortical isoelectric states seems related to neurodevelopmental outcomes. Strategies using continuous EEG monitoring to minimize isoelectric states may be useful during complex congenital heart surgery.

Adverse events following tetralogy of Fallot repair include arrhythmia, reoperation, and death. While limiting the right ventriculotomy mitigates these events over the short and intermediate term, the impact over the long term is unknown. Children requiring tetralogy of Fallot repair were divided into 2 groups: extended right ventriculotomy vs limited right ventriculotomy with transatrial ventricular septal defect closure. Follow-up office ECGs and Holter monitoring, exercise stress tests, and echocardiograms were obtained in the first, second, and third postoperative decades. The primary outcome was cumulative events defined as reoperation, arrhythmia, or death. The extended and limited right ventriculotomy techniques were used in 21 and 17 children, respectively. Cumulative survival was 93.6% at 30 years and was not different between groups. While 10-year (42.9% vs 5.9%, P = 0.02) event rates favored the limited ventriculotomy technique, there were no significant differences at 20 or 25 years. Cox proportional hazard analysis demonstrated that the limited ventriculotomy technique was independently associated with lower events at 10 years (hazard ratio 0.03, 95% confidence interval 0.0016, 0.5697, P = 0.01). The limited ventriculotomy group had greater exercise capacity in the second decade but not the third. Right ventricular end-diastolic diameter Z score was not different at 10 or 20 years, but was significantly smaller in the limited ventriculotomy group at 30 years (5.55 ± 1.69 vs 4.14 ± 0.63, P = 0.03). Limiting the right ventriculotomy during tetralogy of Fallot repair limits 10-year events, improves exercise capacity at 20 years, and attenuates late right ventricular dilation.

Although all coronary anatomy in D-transposition of the great arteries may be "switchable," there are reports in the literature of early and late coronary obstruction following the arterial switch operation. The Damus-Kaye-Stansel procedure does not risk injury to the coronary arteries, and unlike the atrial switch, commits the left ventricle to the systemic circulation. We present a series of four neonates over 22 years with D-transposition of the great arteries and a concomitant coronary artery anomaly precluding arterial switch that were repaired using a Damus-Kaye-Stansel procedure and right ventricle to pulmonary artery conduit.

Background Aortic coarctation (CoA) with concomitant aortic arch hypoplasia (AAH) is associated with an increased risk of hypertension after surgical repair. The differentiation of CoA with or without AAH may be critical to delineate the ideal surgical approach that best ameliorates postoperative hypertension. Since 2000, we have defined CoA with AAH when the diameter of the distal transverse aortic arch is equal to or less than the diameter of the left carotid artery. We hypothesized that, based on our definition, aortic tissue from infants having CoA with AAH would demonstrate distinct genetic expression patterns as compared with infants having CoA alone. Methods From 6 infants (AAH, 3; CoA, 3), an Affymetrix 1.0 genome array identified genes in the coarctation/arch region that were differentially expressed between infants having CoA with AAH versus CoA alone. Reverse transcription polymerase chain reaction validated genetic differences from a cohort of 21 infants (CoA with AAH, 10; CoA, 11). To evaluate the clinical outcomes based on our definition of CoA with AAH, we reviewed infants repaired using this algorithm from 2000 to 2010. Results Microarray data demonstrated genes differentially expressed between groups. Reverse transcription polymerase chain reaction confirmed that CoA with AAH was associated with an increased expression of genes involved in cardiac and vascular development and growth, including hepsin, fibroblast growth factor-18, and T-box 2. The clinical outcomes of 79 infants (AAH, 26; CoA, 53) demonstrated that 90.1% were free of hypertension at 13 years when managed with this surgical strategy. Conclusions These findings provide evidence that the ratio of the diameter of the distal transverse arch to the left carotid artery may be helpful to identify CoA with AAH and, when used to delineate the surgical approach, may minimize hypertension.

Left ventricular hypertrophy (LVH) frequently accompanies the progression of aortic valve disease in children. The extent of LVH regression following surgical relief of aortic valve disease in children has not been clearly elucidated. We hypothesized that significant regression of LVH will occur in children following the Ross procedure.We examined LVH over time in children18 years of age who underwent the Ross procedure. Left ventricular mass index (LVMI) and corresponding z scores were calculated based on height, age and gender. Left ventricular hypertrophy was defined as an LVMI of39 g/m(2.7) and a z score of1.6.Twenty-five children underwent the Ross procedure. The left ventricular mass increased proportionally with the growth of the child from baseline to the latest follow-up at 7.3 ± 2.9 years (121.1 ± 81.5 vs 133.1 ± 79.8 g, P = 0.4). However, 96% (24/25) of children demonstrated LVMI regression from baseline. Mean LVMI decreased from 70.8 ± 31.2 to 41.8 ± 16.6 g/m(2.7) (P0.001). Similarly, LVMI z scores decreased from 2.2 ± 1.2 to 0.2 ± 1.9 (P0.001). Freedom from LVH was 83% at 10 years. Examination of LVMI and z scores over time demonstrated that the largest decrease occurred after the first year, with continued gradual decline over 10 years of follow-up.The Ross procedure is effective in reversing LVH in children with aortic valve disease.

Background Despite repair, a significant proportion of patients with coarctation of the aorta (CoA) present with late hypertension. Increased gene expression of aortic wall collagen and vascular smooth muscle cell markers occurs in the presence of hypertension. Before repair, a patent ductus arteriosus (PDA) limits hypertension proximal to the coarctation. We hypothesize that preoperative collagen and vascular smooth muscle expression from the aortic arch in children is variable, depending on the presence or absence of a PDA. Methods We analyzed the expression patterns of collagen and vascular smooth muscle cell markers in 25 children with CoA using a quantitative polymerase chain reaction. Aortic arch tissue proximal to the CoA was normalized to descending aortic tissue distal to the coarctation. Collagen-I, transforming growth factor-β, elastin, and calponin were analyzed. Results At repair, 19 patients were aged younger than 3 months (14 with a PDA, 5 with a ligamentum arteriosum), and the remaining 6 were older than 1 year. There was no difference in age or weight between infants with or without a PDA. Infants without a PDA had the greatest difference in collagen-I expression compared with infants with a PDA (7.0 ± 1.6-fold vs 0.8 ± 1.1-fold, p = 0.01). Expression of transforming growth factor-β (4.3 ± 1.4 vs 2.6 ± 2.3, p = 0.01) and calponin (3.7 ± 0.7 vs 0.6 ± 1.1, p = 0.05) was lower from infants with vs without a PDA. Conclusions Our findings provide evidence of preoperative changes in the aortic arch before repair, particularly in the absence of a PDA.

Regionalization of pediatric cardiac surgical care varies between and within states. In most geographic regions, at least some neonates with critical heart disease are transferred from their birth hospital to a different hospital for surgery. The impact of neonatal transfer for surgery, particularly over a considerable distance (>10 miles), has been largely unexplored. We sought to examine the impact of transferring neonates for cardiac surgery. We queried the New York State Cardiac Surgery database (2005-2014) from a single institution to identify neonates born within the cardiac surgery center and those transferred for surgery. Outcomes were compared between groups, with subgroup analysis of neonates with single ventricle anatomy. 113 surgical neonates were born at the cardiac surgery center, and 268 were transferred to the cardiac surgery center. Median transfer distance was 91 (IQR 73, 94) miles. Age at operation and the need for preoperative ventilation were significantly lower in neonates born at the cardiac surgery center. In addition, single ventricle anatomy was more prevalent among those born at the cardiac surgery center (48.7 vs. 31.3%; p = 0.001). However, postoperative outcomes were the same-30-day survival was similar across groups (birth: 89% vs. transfer: 90%; p = 0.7), and for those with single ventricle palliation (birth: 81% vs. transfer: 81%; p = 0.9). Within our regionalized network, we found no difference in 30-day survival between neonates either born or transferred to a cardiac surgery center, which supports the use of a regionalized network of hospitals to the care of children with congenital heart disease.

The article by Naimo and colleagues (outcomes of truncus arteriosus repair in children: 35 years of experience from a single center) provides the initial glimpse at long-term outcomes following truncus arteriosus repair. Although survival has improved over the past decade, the morbidity resulting from subsequent re-operations now comprises a significant area for improvement in the ensuing decade.

In order to identify differentially expressed genes that are specific to the ductus arteriosus, 18 candidate genes were evaluated in matched ductus arteriosus and aortic samples from infants with coarctation of the aorta. The cell specificity of the gene's promoters was assessed by performing transient transfection studies in primary cells derived from several patients. Segments of ductus arteriosus and aorta were isolated from infants requiring repair for coarctation of the aorta and used for mRNA quantitation and culturing of cells. Differences in expression were determined by quantitative PCR using the ΔΔCt method. Promoter regions of six of these genes were cloned into luciferase reporter plasmids for transient transfection studies in matched human ductus arteriosus and aorta cells. Transcription factor AP-2b and phospholipase A2 were significantly up-regulated in ductus arteriosus compared to aorta in whole tissues and cultured cells, respectively. In transient transfection experiments, Angiotensin II type 1 receptor and Prostaglandin E receptor 4 promoters consistently gave higher expression in matched ductus arteriosus versus aorta cells from multiple patients. Taken together, these results demonstrate that several genes are differentially expressed in ductus arteriosus and that their promoters may be used to drive ductus arteriosus-enriched transgene expression.

Circulatory arrest (CA) is traditionally utilized during the repair of total anomalous pulmonary venous return (TAPVR). Since 2005, we have exclusively repaired all types of TAPVR using continuous cardiopulmonary bypass. We present our technique using continuous cardiopulmonary bypass throughout the duration of the repair, by temporarily occluding the vertical vein and placing a pump sucker within the pulmonary venous confluence. This technique has been used on 29 consecutive patients and resulted in limited morbidity and absence of pulmonary vein stenosis from most recent follow-up.

Background The optimal hemoglobin for infants after cardiac operation is unknown. Red blood cells (RBCs) are commonly transfused to maintain high hemoglobin concentrations in the absence of a clinical indication. We hypothesized that infants can be managed with a postoperative conservative RBC transfusion strategy, resulting in lower daily hemoglobin concentrations, without evidence of impaired oxygen delivery (ie, lactate, arteriovenous oxygen difference [avO 2 diff]), or adverse clinical outcomes. Methods Infants weighing 10 kg or less undergoing biventricular repair or palliative (nonseptated) operation were randomly assigned to either a postoperative conservative or liberal transfusion strategy. Conservative group strategy was RBC transfusion for a hemoglobin less than 7.0 g/dL for biventricular repairs or less than 9.0 g/dL for palliative procedures plus a clinical indication. Liberal group strategy was RBC transfusion for hemoglobin less than 9.5 g/dL for biventricular repairs or less than 12 g/dL for palliative procedures regardless of clinical indication. Results After the operation of 162 infants (82 conservative [53 biventricular, 29 palliative], 80 liberal [52 biventricular, 28 palliative]), including 12 Norwood procedures (6 conservative, 6 liberal), daily hemoglobin concentrations were significantly lower within the conservative group than the liberal group by postoperative day 1 and remained lower for more than 10 days. The percentage of patients requiring a RBC transfusion, number of transfusions, and volume of transfusions were all significantly lower within the conservative group. Despite lower hemoglobin concentrations within the conservative group, lactate, avO 2 diff, and clinical outcomes were similar. Conclusions Infants undergoing cardiac operation can be managed with a conservative RBC transfusion strategy. Clinical indications should help guide the decision for RBC transfusion even in this uniquely vulnerable population. Larger multicenter trials are needed to confirm these results, and focus on the highest risk patients would be of great interest.

Objective Right ventricular outflow tract (RVOT) reconstruction necessitates frequent reoperation. To understand the early outcomes, we analyzed our results to provide the intra- and postoperative morbidity and mortality. We hypothesized that multiple previous sternotomies do not influence the morbidity, mortality, or survival. Design We performed a retrospective review of patients who underwent reoperative RVOT reconstruction at the University of Rochester Medical Center and SUNY Upstate Medical Center from January 1, 2000 to December 31, 2009. Patients were divided into three groups based upon the number of previous sternotomies: Group 1 with one, Group 2 with two, and Group 3 with three or more previous sternotomies. Results 220 patients had reoperative RVOT reconstruction, 103 in Group 1, 71 in Group 2, and 46 in Group 3. There was no difference in the percentage of inadvertent cardiotomy between groups (Group 1: 2%, Group 2: 1%, Group 3: 2%; P =.9) The number of previous sternotomies had no effect upon infection, arrhythmia, or the percentage of patients who received a red blood cell transfusion (Group 1: 56%, Group 2: 49% Group 3: 43%; P =.3). Perioperative mortality for the entire group was 3/220 (1.4%), with no difference between groups. At a mean follow-up of 39 months, there was a survival of 98% for Groups 1 and 3 and 97% for Group 2 (P =.7). Conclusion Reoperative RVOT reconstruction can safely be performed with limited morbidity and mortality. The number of previous sternotomies does not influence the rate of cardiotomy, red blood cell transfusion, or early outcome.

The New York State Department of Health developed a Pediatric Cardiac Surgery Reporting System in 1991 that contains detailed information on demographics, diagnoses, procedures, comorbidities, complications, and discharge information for every pediatric cardiac surgery patient in the state. The Department and the Congenital Cardiac Services Subcommittee of the Department's Cardiac Advisory Committee have used data from this system to assess, assure, and improve quality of care and to generate public reports on an ongoing basis. Two reports (one covering 3years and the other covering 4years) have been published, and a third report, comprising the years 2006–2009, will be released shortly. These reports contain information on patient diagnoses, patient severity groups, and risk-adjusted in-hospital mortality rates for all hospitals that perform pediatric cardiac surgery. The goal of the DOH and the CAC is to improve the quality of cardiac care in NYS. Providing the hospitals and cardiac surgeons in NYS with data about their own outcomes for these procedures allows them to examine the quality of the care they provide and to identify areas that need improvement. These data are fed back to providers on an ongoing basis in addition to in the published reports. The overall results of this program of ongoing review show that significant progress is being made. The volume of surgeries and the number of hospitals where they are performed have both decreased dramatically in the years since NYS first began reporting risk-adjusted outcomes for pediatric cardiac surgery. In 1997, 16 hospitals performed 1749 pediatric cardiac surgeries. By 2009, there were only 10 hospitals performing 1304 surgeries. The average number of surgeries per hospital in 1997 was 109 compared to 139 in 2010. Many factors, including increased use of catheter-based therapies, may have contributed to the overall decline in the number of surgeries. The overall mortality rate for pediatric cardiac surgery dropped from 4.08% in 2002 to 2005 period to a mortality rate of 3.35% in 2006 to 2009 period with no major changes in overall patient risk, although part of this decrease may also be due to improvements in the field in general. A multivariable analysis demonstrated that a dedicated pediatric cardiac ICU proved to be a significant independent predictor of mortality. The methods for assessing patients' risk of in-hospital mortality have changed with each successive report, and this study describes the current method and the risk factors used in the method. The Department's other initiatives for improving quality, including consolidation of services, linking processes and structures of care to outcomes, and other regulatory actions, are also described.

Objective. The goal of aortic coarctation repair is laminar aortic blood flow resulting in a negative or absent arm:leg blood pressure (BP) gradient. Despite satisfactory relief of coarctation, associated arch hypoplasia can result in residual obstruction and postoperative upper body hypertension. Intervention. We devised a surgical strategy to create a tension-free anastomosis with a diameter as large as both the adjacent proximal and distal aorta using a radically extended end-to-end anastomosis via sternotomy and/or thoracotomy. Sternotomy is chosen when there is significant transverse arch hypoplasia defined as a distal transverse arch ≤ diameter of the left carotid artery, presence of a common brachiocephalic trunk, or coexisting intracardiac lesion requiring repair. Thoracotomy is used in all other cases. Results. From 2000 to 2008, 95 consecutive patients were repaired using this approach, 35 with sternotomy and 60 with thoracotomy. At a mean follow-up of 50 ± 23 months, mean systolic BP was 94 ± 10 mm Hg, and 84% of patients had no residual arm:leg BP gradient. Mean arm:leg BP gradient was not statistically different between groups (−8.5 ± 15 sternotomy and −7.0 ± 10 mm Hg thoracotomy, P= .7). With Doppler echocardiography, 96% of patients demonstrated normal early diastolic reversal of blood flow in the descending thoracic aorta. Conclusions. For aortic coarctation repair in infancy, a strategy designed to directly address aortic arch hypoplasia results in excellent intermediate-term results with normal BP, physiologic arm:leg BP relationship, and near normal descending aortic blood flow velocities by Doppler.

Objective: To examine the impact of a restrictive vs. liberal transfusion strategy on arterial lactate and oxygen content differences in children with single-ventricle physiology post cavopulmonary connection. Children with single-ventricle physiology are routinely transfused postoperatively to increase systemic oxygen delivery, and transfusion thresholds in this population have not been studied. Design: Prospective, randomized, controlled, clinical trial. Setting: Pediatric cardiac intensive care unit in a teaching hospital. Patients: Infants and children (n 60) with variations of singleventricle physiology presenting for cavopulmonary connection. Interventions: Subjects were randomized to a restrictive (hemoglobin of 13.0 g/dL) transfusion strategy for 48 hrs post operation. Primary outcome measures were mean and peak arterial lactate. Secondary end points were arteriovenous (C(a-v)O2) and arteriocerebral oxygen content (C(a-c)O2) differences and clinical outcomes. Measurements and Main Results: A total of 30 children were in each group. There were no significant preoperative differences. Mean hemoglobin in the restrictive and liberal groups were 11 1.3 g/dL and 13.9 0.5 g/dL, respectively (p < .01). No differences in mean (1.4 0.5 mmol/L [Restrictive] vs. 1.4 0.4 mmol/L [Liberal]) or peak (3.1 1.5 mmol/L [Restrictive] vs. 3.2 1.3 mmol/L [Liberal]) lactate between groups were found. Mean number of red blood cell transfusions were 0.43 0.6 and 2.1 1.2 (p < .01), and donor exposure was 1.2 0.7 and 2.4 1.1 to (p < .01), for each group, respectively. No differences were found in C(a-v)O2, C(a-c)O2, or clinical outcome measures. Conclusion: Children with single-ventricle physiology do not benefit from a liberal transfusion strategy after cavopulmonary connection. A restrictive red blood cell transfusion strategy decreases the number of transfusions, donor exposures, and potential risks in these children. Larger studies with clinical outcome measures are needed to determine the transfusion threshold for children post cardiac repair or palliation for congenital heart disease. (Pediatr Crit Care Med 2011; 12: 39‐45)

Background Repair of tetralogy of Fallot (ToF) using a transannular patch can result in severe pulmonary insufficiency (PI) and subsequent right ventricular (RV) dilation. Use of a Dacron (Maquet Cardiovascular LLC, Wayne, NJ) limited transannular patch with nominal pulmonary annular expansion (LTAP) attempts to limit PI. We sought to evaluate the degree of PI and RV dilation resulting from a LTAP or annular sparing (AS) approach. Methods Infants less than 1 year of age undergoing ToF repair between 2000 and 2010 were divided into 2 groups: LTAP and AS RV outflow tract patch. Echocardiograms were used to determine RV dimensions and corresponding Z-values. Results From 94 infants, 48 required a LTAP and 46 required an AS patch. The preoperative pulmonary valve annulus Z-value was significantly smaller in the LTAP versus AS group (–2.7 ± 1.4 versus –0.9 ± 1.5; p 0.001). Mean follow-up was obtained at 7.9 ± 3.4 years. Ten-year freedom from severe pulmonary insufficiency was 78.5% versus 93.2% ( p = 0.3) in the LTAP and AS groups, respectively. There was no significant difference in the diameter of the RV base Z-value between groups (LTAP: 0.9 ± 0.8 versus AS: 0.0 ± 2.3; p = 0.1). Further, the freedom from reoperation at 10 years was also not significantly different between the LTAP and AS groups (95.6% versus 91.8%; p = 0.5). Conclusions When required, a LTAP results in a similar change in RV chamber size and rate of reoperation at an intermediate-term follow-up.

Thrombotic events cause significant morbidity and mortality in children who undergo surgery for complex congenital cardiac disease. We prospectively evaluated the incidence of thrombosis and examined preoperative and postoperative laboratory tests of coagulation and inflammation in neonates experiencing initial surgical palliation for variations of single ventricle physiology.Neonates (30 days) requiring initial surgical palliation were studied. All subjects received aspirin from postoperative day 1 onward. Thromboses were diagnosed by serial transthoracic echocardiograms, vascular imaging, and interstage cardiac catheterizations according to predefined criteria.Twenty-two neonates, age 1 to 11 days (mean 4 +/- 2.5) were studied. Follow-up ranged from three hours to 18 months (median, 212 days). Eight infants died. Four of the 14 subjects who survived (28%), and one of the eight who died (12.5%), had evidence of thrombosis identified over a range of four hours to nine months postoperatively (median 14 days). When compared with reference values established in healthy children, preoperative subject hematocrit (Hct), platelet count, factors II, V, VII, VIII, and X, antithrombin, protein C, and soluble CD40 ligand measures were significantly lower, and the prothrombin time and partial thromboplastin time were significantly higher. Postoperative C reactive protein (CRP) was significantly higher, and Hct and platelet count significantly lower, than preoperative values. Thrombotic events were significantly related to high preoperative CRP (p = 0.02).Thrombotic complications occur frequently in neonates undergoing initial palliative surgery, suggesting that aspirin therapy alone may constitute inadequate protection. Elevated preoperative CRP appears to be associated with increased thrombotic risk.

Traumatic aortic rupture due to blunt trauma in the pediatric population is rare. The management of this unusual injury has largely been extrapolated from the adult literature and is evolving. Open surgical repair is the accepted treatment; however, endograft repair is a promising alternative, which can serve as a definitive or bridging technique in select patients who are high-risk surgical candidates. The authors report the successful deployment of an endograft limb to correct a traumatic pseudoaneurysm of the aorta in a high-risk pediatric patient.

Introduction: The mortality of infants requiring cardiac surgery is related to center and surgeon case volume. One solution for increasing case volume is to regionalize pediatric cardiac surgical care. Hypothesis: We hypothesized that the regionalization of pediatric cardiac surgical care from two medical centers would lower mortality, rate of re-operation, and subsequent health care costs. Methods: Infants undergoing a biventricular repair were divided into two groups: pre-regionalization when operations were performed in two separate hospitals (1991-2000) and post-regionalization when all operations were performed in one regional hospital (2001-2010). Operative survival, midterm mortality, need for re-operation, and subsequent healthcare costs were compared between the groups. Results: There were 508 infants in the pre-regionalization group, and 479 infants in the post-regionalization group. There were no significant differences in pre-operative age, weight, or gender between groups. Pre-regionalization operative survival was significantly lower than the state average. Post-regionalization operative survival was significantly greater than pre-regionalization and was similar to the state average (Figure 1). Five and 10 year survival remained significantly greater in the post-regionalization group (5 year: 96.8% vs 90.6%, 10 year: 96.8% vs. 89.9%, p

Most commonly, hearts have either a single arterial trunk or two arterial trunks; however, rare reports exist of a “tritruncal” heart, a heart with three outflow tracts. Here, we present one of the first reports of successful surgical palliation of a triple-outlet right ventricle.

Background: Antifibrinolytic medications such as ε-aminocaproic acid (EACA) are used in pediatric heart surgery to decrease surgical bleeding and transfusion. Dosing schemes for neonates are often based on adult regimens, or are simply empiric, in part due to the lack of neonatal pharmacokinetic information. The authors sought to determine the pharmacokinetics of EACA in neonates undergoing cardiac surgery and to devise a dosing regimen for this population. Methods: Ten neonates undergoing cardiac surgery with cardiopulmonary bypass were given EACA according to standard practice, and blood was drawn at 10 time points to determine drug concentrations. Time-concentration profiles were analyzed using nonlinear mixed effects models. Parameter estimates (standardized to a 70-kg person) were used to develop a dosing regimen intended to maintain a target concentration shown to inhibit fibrinolysis in neonatal plasma (50 mg/l). Results: Pharmacokinetics were described using a two-compartment model plus an additional compartment for the cardiopulmonary bypass pump. First-order elimination was described with a clearance of 5.07 l/h × (WT/70)0.75. Simulation showed a dosing regimen with a loading dose of 40 mg/kg and an infusion of 30 mg · kg−1 · h−1, with a pump prime concentration of 100 mg/l maintained plasma concentrations above 50 mg/l in 90% of neonates during cardiopulmonary bypass surgery. Conclusions: EACA clearance, expressed using allometry, is reduced in neonates compared with older children and adults. Loading dose and infusion dose are approximately half those required in children and adults.

Objective Although the arterial switch operation has been performed with very low mortality and morbidity in large pediatric cardiac surgical centers, we sought to determine if similar arterial switch results could be achieved in two medium-sized pediatric cardiac surgical centers that share one full-time surgeon who implements a comprehensive management protocol for both centers. Methods Patients with D-transposition of the great arteries undergoing arterial switch operations at two medium-sized pediatric cardiac surgery centers were evaluated for 30-day mortality and morbidity during three time periods: Period 1, before protocol was implemented at either center (Center 1: 11/1982–8/1995, Center 2: 3/1992–11/1999); Period 2, protocol implemented only at Center 1 (9/1995–11/1999); Period 3, protocol implemented at both centers with regional sharing of one surgeon (12/1999–1/2003). Results Ninety-six consecutive patients undergoing an arterial switch operation were reviewed. Mortality data were available for all cases. Mortality was 0% and significantly lower in Periods 2 and 3 versus Period 1 ( p p p Conclusions Outstanding surgical results for the arterial switch operation, similar to those in the largest pediatric cardiac surgical centers, are obtainable in two medium-sized pediatric cardiac surgery centers that share one full-time congenital heart surgeon. This model may be applicable in many other regions of the United States, where several medium-sized pediatric cardiac centers are within acceptable driving distance.

Sinus of Valsalva aneurysm (SVA) is a rare cardiac lesion especially in the western countries and older population. We report an unusual case of a 60-year-old Caucasian male with SVA, acute decompensation, and a pressurized prolapsed aortic leaflet cystic remnant via a small supracristal VSD causing recurrent right ventricular outflow tract obstruction following a Bentall procedure. doi: 10.1111/jocs.12219 (J Card Surg 2014;29:317–319)

A 5-month-old boy who had previously undergone a modified Norwood procedure for hypoplastic left heart syndrome presented for elective cardiac catheterization before undergoing the next stage of repair. The catheterization demonstrated mild stenosis of the right ventricle-to-pulmonary artery Sano conduit. The patient became hypoxic and febrile at the end of the procedure and died in the intensive care unit the next day. Postmortem examination demonstrated a vegetation containing Gram-positive cocci in the conduit and a right upper lobe lung abscess also containing Gram-positive cocci. The authors report the first case of Sano shunt endocarditis further complicated by septic pulmonary embolus and review similar infections in this population. Shunt infection may be an underrecognized cause of interstage mortality among patients undergoing a modified Norwood procedure.

Objectives: The pH of cardioplegic solutions is postulated to affect myocardial protection during neonatal hypothermic circulatory arrest. Neither optimization of cardioplegic pH nor its influence on intracellular pH during hypothermic circulatory arrest has been previously studied in vivo. Thus we examined the effects of the pH of cardioplegic solutions on postischemic cardiac function in vivo, including two possible operative mechanisms: (1) reduction in adenosine triphosphate use and depletion of high-energy phosphate stores or (2) reduction of H + flux during reperfusion, or both. Methods: Dynamic 31 P spectroscopy was used to measure rates of adenosine triphosphate use, high-energy phosphate depletion, cytosolic acidification during hypothermic circulatory arrest, and phosphocreatine repletion and realkalinization during reperfusion. Neonatal pigs in three groups (n = 8 each)—group A, acidic cardioplegia (pH = 6.8); group B, basic cardioplegia (pH = 7.8); and group N, no cardioplegia—underwent hypothermia at 20° C with 60 minutes of hypothermic cardioplegia followed by reperfusion. Results: Recoveries of peak elastance, stroke work, and diastolic stiffness were superior in group B. Indices of ischemic adenosine triphosphate use, initial phosphocreatine depletion rate, and τ, the exponential decay half-time, were not different among groups. Peak [H + ] in group A (end-ischemia) was significantly elevated over that of group B. The realkalinization rate was reduced in group B compared with that in groups A (p = 0.015) and N (p = 0.035), with no difference between groups A and N (p = 0.3). Cytosolic realkalinization rate was markedly reduced and the half-time of [H + ] decay was increased during reperfusion in group B. Conclusions: Superior postischemic cardiac function in group B is not related to alterations in ischemic adenosine triphosphate use or high-energy store depletion, but may be due to slowing in H + efflux during reperfusion, which should reduce Ca ++ and Na + influx. (J Thorac Cardiovasc Surg 1997;114:601-8)

Background During the surgical repair of infants with congenital cardiac defects, there can be periods of decreased cerebral blood flow, particularly during deep hypothermic circulatory arrest. As a result, these infants are at increased risk for seizures and long-term neurodevelopmental difficulties. Methods Thirty-two infants with congenital heart disease had continuous video-electroencephalographic (EEG) monitoring pre-, intra-, and postoperatively for 48 hours after surgery. Results For patients requiring deep hypothermic circulatory arrest (n = 17) the EEG pattern for all patients became suppressed and eventually isoelectric below 25°C. Two of the 32 infants had electrical seizures within the 48-hour monitoring period. Both required deep hypothermic circulatory arrest, and the burst pattern during recovery had rhythmic, sharp components that were high amplitude and often asynchronous between the hemispheres. The interval between the onset of seizure activity and initiation of the sharp burst pattern during surgery was 29 and 40 hours. This pattern was not observed during isoelectric recovery from infants who did not develop postoperative seizures. Conclusions The EEG in infants during deep hypothermic circulatory arrest displayed predictable changes. We identified an electroencephalographic pattern following the isoelectric period that may predict seizure development in the subsequent 48 hours.

Sinus of Valsalva aneurysm (SVA) is a rare cardiac lesion especially in the western countries and older population. We report an unusual case of a 60-year-old Caucasian male with SVA, acute decompensation, and a pressurized prolapsed aortic leaflet cystic remnant via a small supracristal VSD causing recurrent right ventricular outflow tract obstruction following a Bentall procedure

b r r m TROKE IS ONE OF the more common serious complications of cardiac surgery, with an estimated incidence of pproximately 0.8% to 5.2% in adults.1 The incidence of overt troke in congenital heart surgery is less well defined but is ikely in the same range.2 Typically, stroke during cardiac urgery is embolic in nature.1 Embolic material includes disupted arterial plaque and air from cardiac chambers or the ardiopulmonary bypass (CPB) pump. Less commonly, cereral injury may occur as a result of decreased global perfusion. hrombosis of cerebral vessels is not uncommon in congenital eart disease but virtually always involves the venous system.3 rterial thrombosis is rare after cardiac surgery because paients tend to be hypocoagulable after CPB, especially chilren.2 A case of arterial thrombosis after reoperative congenital eart surgery with an unusual etiology is described.

Background Providing unobstructed systemic blood flow is one goal of stage I palliation for hypoplastic left heart syndrome. Although clinically significant obstruction is defined when the arch gradient exceeds 15 mm Hg, any positive gradient constitutes obstruction, which may impair ventricular function. We sought to identify risk factors before stage I palliation that result in a positive neoaortic arch gradient before bidirectional Glenn. Methods Reviewed were 51 neonates who underwent stage I palliation and subsequent bidirectional cavopulmonary anastomosis procedures for hypoplastic left heart syndrome. Echocardiograms before stage I palliation were reviewed for aortic dimensions. Cardiac catheterization before bidirectional cavopulmonary anastomosis determined the aortic arch gradient and degree of right ventricular dysfunction. Results Of the 51 patients, 24 (47%) had a negative or absent arch gradient. Patients with a positive gradient had a median gradient of 5 mm Hg (range, 1 to 60 mm Hg). The diameter of the ascending aorta and proximal transverse arch indexed to the descending thoracic aorta (0.5 ± 0.2 vs 0.7 ± 0.4 and 0.4 ± 0.2 vs 0.6 ± 0.2 mm, p = 0.02 and p = 0.01) and lower birth weight (3.1 ± 0.5 vs 3.4 ± 0.4 kg, p = 0.03) were risk factors of a positive neoaortic arch gradient. Further, the degree of arch obstruction was inversely related to the degree of right ventricular function (odds ratio, 1.08; p Conclusions Lower birth weight and a smaller aortic diameter confer a higher risk of developing a positive neoaortic arch gradient, resulting in reduced right ventricular function.