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Background: Congenital heart disease (CHD) is associated with abnormal fetal brain development, a phenomenon that may be related to decreased cerebral oxygen delivery in utero. We used an artificial womb model to test the hypothesis that decreasing fetal oxygen delivery would reproduce physiologic changes identified in fetuses with CHD., Methods: Experimental (hypoxemic) fetal lambs (mean gestational age, 111 ± 3 days; n = 4) and control animals (112 days; n = 5) were maintained in the artificial womb for a mean of 22 ± 6 days. Oxygen delivery was reduced to 15.6 ± 1.0 mL/kg/min in the hypoxemia animals versus 21.6 ± 2.0 mL/kg/min in the control animals. Blood chemistry analysis and sonographic evaluation were performed daily. An additional control group (n = 7) was maintained in utero and harvested for analysis at gestational age 134 ± 4 days., Results: Physiologic variables were monitored continuously, and no statistical differences between the groups were identified. Fetal oxygen delivery and arterial partial pressure of oxygen were remarkably lower in the experimental group longitudinally. Increased umbilical artery and decreased middle cerebral artery resistance resulted in a lower cerebral to umbilical resistance ratio, similar to the brain sparing effect observed in human fetuses with CHD. Experimental brains were smaller than control brains in relation to the calvarium on magnetic resonance., Conclusions: Sustained hypoxemia in fetal sheep leads to altered cerebrovascular resistances and loss of brain mass, similar to human fetuses with CHD. This unique model provides opportunities to investigate the pathologic process underlying CHD-associated brain dysmaturity and to evaluate potential fetal neuroprotective therapies., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Industrial chemicals are increasingly recognized as potential developmental neurotoxicants. Di(2-ethylhexyl) phthalate (DEHP), used to impart flexibility and temperature tolerance to polyvinylchloride, and bisphenol A (BPA), used to manufacture polycarbonate, are commonly present in medical devices. The magnitude of exposure in neonates during hospitalization for cardiac operations is unknown., Methods: We quantified urinary concentrations of DEHP metabolites and BPA preoperatively and postoperatively in neonates undergoing cardiac operations and their mothers. Urinary concentrations of these biomarkers reflect recent exposures (half-lives are approximately 6 to 24 hours). Biomarker concentrations in mothers' and infants' preoperative and postoperative samples were compared., Results: Operations were performed in 18 infants (mean age, 5 ± 4 [SD] days). The maternal sample was obtained on postpartum day 4 ± 4. The preoperative urine sample was obtained on day-of-life 4 ± 2 and the postoperative sample on day-of-life 6 ± 4. Mean maternal concentrations for DEHP metabolites and BPA were at the 50th percentile for females in the United States general population. Infant preoperative concentrations of 1 DEHP metabolite and BPA were significantly higher than maternal concentrations. Postoperative concentrations for all DEHP metabolites were significantly greater than preoperative concentrations., Conclusions: There is considerable perioperative exposure to DEHP and BPA for neonates undergoing cardiac operations. Infant concentrations for both BPA and DEHP metabolites were significantly higher than maternal concentrations, consistent with the infant's exposure to medical devices. Further study is needed to determine the potential role of these suspect neurotoxicants in the etiology of neurodevelopmental disability after cardiac operations., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Remote ischemic preconditioning (RIPC) is a mechanism to protect tissues from injury during ischemia and reperfusion. We investigated the neuroprotective effects of RIPC in neonates undergoing cardiac surgery., Methods: The outcome was white matter injury (WMI), assessed by the change in volume of WMI from preoperative to postoperative brain magnetic resonance imaging (MRI). Patients were randomized to RIPC or SHAM. RIPC was induced prior to cardiopulmonary bypass by four 5-minute cycles of blood pressure cuff inflation to produce ischemia of the lower extremity. For patients randomized to SHAM, the cuff was positioned, but not inflated., Results: The study included 67 patients, with 33 randomized to RIPC and 34 randomized to SHAM. Preoperative and postoperative MRIs were available in 50 patients, including 26 of the 33 RIPC patients and 24 of the 34 SHAM patients. There were no differences in baseline and operative characteristics for either the overall study group or the group with evaluable MRIs. WMI was identified in 28% of the patients preoperatively and in 62% postoperatively. There was no difference in the prevalence of WMI by treatment group (p > 0.5). RIPC patients had an average change in WMI of 600 mL 3 , and SHAM subjects had an average WMI change of 107 mL 3 . There was no significant difference in the mean value of WMI change between patients who received RIPC and those who received SHAM treatments (p = 0.178)., Conclusions: In this randomized, blinded clinical trial, there was no evidence that use of RIPC provides neuroprotection in neonates undergoing repair of congenital heart defects with cardiopulmonary bypass., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Calls for public reporting of outcomes in congenital heart surgery have led to several different reporting schemes, including a star rating system and benchmark procedure-specific mortality data tables. Important unanswered questions remain about the optimal format and content of public reporting of congenital heart surgery outcomes., Methods: In conjunction with three parent advocacy groups, we developed a questionnaire to gauge parents' attitudes regarding the format and content of an "optimal" public reporting scheme. Parents were solicited for participation through email lists of members of parent advocacy groups and from a cohort of parents whose children had undergone an STS benchmark procedure of the Society of Thoracic Surgeons at the Children's Hospital of Philadelphia after January 1, 2007., Results: The 1,297 responses received provided complete data for analysis. Nearly all the participants were mothers of children with congenital heart disease, and most were white. About half of the children were diagnosed prenatally, and 63% underwent initial repair of the defect in the neonatal period. Parents identified survival statistics, surgeon-specific experience, and complication rates as most important. Presented with three display formats for mortality rates, most parents (89%) identified a numeric procedure-based approach as the best format, and more than half identified the hospital star rating system as the worst format., Conclusions: Parents of children with congenital heart disease identify survival statistics, surgeon-specific experience, and complication rates as the most important outcome measures to report publicly. Additionally, parents preferred mortality data to be presented in a procedure-specific format using a numeric procedure-based approach, as opposed to the star rating system., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The use of administrative data for surgical site infection (SSI) surveillance leads to inaccurate reporting of SSI rates [1]. A quality improvement (QI) initiative was conducted linking clinical registry and administrative databases to improve reporting and reduce the incidence of SSI [2]., Methods: At our institution, The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and infection surveillance database (ISD) were linked to the enterprise data warehouse containing electronic health record (EHR) billing data. A data visualization tool was created to (1) use the STS-CHSD for case ascertainment, (2) resolve discrepancies between the databases, and (3) assess impact of QI initiatives, including wound alert reports, bedside reviews, prevention bundles, and billing coder education., Results: Over the 24-month study period, 1,715 surgical cases were ascertained according to the STS-CHSD clinical criteria, with 23 SSIs identified through the STS-CHSD, 20 SSIs identified through the ISD, and 32 SSIs identified through the billing database. The rolling 12-month STS-CHSD SSI rate decreased from 2.73% (21 of 769 as of January 2013) to 1.11% (9 of 813 as of December 2014). Thirty reporting discrepancies were reviewed to ensure accuracy. Workflow changes facilitated communication and improved adjudication of suspected SSIs. Billing coder education increased coding accuracy and narrowed variation between the 3 SSI sources. The data visualization tool demonstrated temporal relationships between QI initiatives and SSI rate reductions., Conclusions: Linkage of registry and infection control surveillance data with the EHR improves SSI surveillance. The visualization tool and workflow changes facilitated communication, SSI adjudication, and assessment of the QI initiatives. Implementation of these initiatives was associated with decreased SSI rates., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The use of a right ventricle-to-pulmonary artery (RV-PA) conduit for stage 1 palliation of hypoplastic left heart syndrome is common. A prospective randomized multiinstitutional study revealed that approximately 40% of those receiving this shunt required intervention on the shunt or pulmonary arteries, or both. A technique has been developed to help ameliorate proximal conduit stenosis. We present a new technique to improve distal conduit stenosis and decrease anastomotic bleeding from this site. The technique involves dunking a segment of the shunt into the pulmonary arteries and suture placement in the shunt rings and not in the polytetrafluoroethylene (PTFE)., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Mitral valve (MV) abnormalities may adversely impact outcomes after biventricular repair of critical left-side heart obstruction., Methods: Neonates with left-side heart obstruction and MV abnormality (anatomically abnormal or hypoplastic but otherwise normal [Z-score -1.5 to -4]) who underwent biventricular repair from December 2005 to December 2012 were included. Institutional selection criteria determined suitability for biventricular repair. Primary outcomes (death, univentricular conversion, pulmonary hypertension) and secondary outcomes (reinterventions) were compared between hypoplastic and anatomically abnormal MV groups. Freedom from reintervention was determined by Kaplan-Meier analysis., Results: There were 55 patients with hypoplastic MV (n = 35) or anatomically abnormal MV (n = 20). Initial interventions included arch repair (n = 45, with ventricular septal defect closure in 7), balloon aortic valvuloplasty (n = 8), and Ross-Konno operation (n = 2). None had initial MV intervention. Follow-up was available on 46 patients (84%) at a median of 25 months. Primary outcome occurred in 6 patients (13%): 2 deaths, 1 univentricular conversion, and 3 pulmonary hypertension. Twenty-six patients (57%) underwent 36 follow-up interventions, often for arch obstruction (n = 26). All follow-up MV interventions (n = 4) occurred in patients with anatomically abnormal MV. Median reintervention-free time was 7.3 months. Freedom from reintervention at 6, 9, and 12 months was 65%, 38%, and 27%, respectively. Neither MV size nor anatomy was associated with primary outcome., Conclusions: Using our selection criteria, most neonates with left-side heart obstruction and MV abnormalities who underwent biventricular repair had reasonable survival with complex reintervention needs in early follow-up. The MV reintervention occurred in patients with abnormal anatomy, not hypoplasia., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Single-ventricle patients undergoing surgical reconstruction experience a high rate of brain injury. Incidental findings on preoperative brain scans may result in safety considerations involving hemorrhage extension during cardiopulmonary bypass that result in surgical postponement., Methods: Single-ventricle patients were studied with brain scans immediately preoperatively, as part of a National Institutes of Health study, and were reviewed by neuroradiology immediately before cardiopulmonary bypass., Results: Of 144 consecutive patients recruited into the project, 33 were studied before stage I (3.7±1.8 days), 34 before bidirectional Glenn (5.8±0.5 months), and 67 before Fontan (3.3±1.1 years) operations. Six operations (4.5%), 2 before stage I, 3 before bidirectional Glenn, and 1 before Fontan, were postponed because of concerning findings on brain magnetic resonance imaging. Five were due to unexpected incidental findings of acute intracranial hemorrhage, and 1 was due to diffuse cerebellar cytotoxic edema; none who proceeded to operation had these lesions. Prematurity and genetic syndromes were not present in any patients with a postponed operation. Four of 4 before bidirectional Glenn/Fontan with surgical delays had hypoplastic left heart syndrome compared with 44 of 97 who did not (p=0.048). After observation and follow-up, all eventually had successful operations with bypass., Conclusions: Preoperative brain magnetic resonance imaging performed in children with single ventricles disclosed injuries in 4.5% leading to surgical delay; hemorrhagic lesions were most common and raised concerns for extension during the operation. The true risk of progression and need for delay of the operation due to heparinization associated with these lesions remains uncertain., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Survival after cardiac surgery in infancy requires adaptive responses from oxidative stress management and vascular regulation pathways. We tested the hypothesis that genetic variation in these pathways influences postoperative survival in nonsyndromic congenital heart disease children., Methods: This is an analysis of a cohort of nonsyndromic congenital heart disease patients who underwent cardiac surgery with cardiopulmonary bypass before 6 months of age (n=422). Six single nucleotide polymorphisms (SNPs) in six genes involved in oxidative stress and vascular response pathways, identified through a priori literature search, were tested for effects on transplant-free survival. Survival curves, adjusting for confounding covariates, were calculated using the Cox proportional hazard models., Results: Long-term survival was strongly associated with vascular endothelial growth factor A gene SNP rs833069 (p=7.03×10(-4)) and superoxide dismutase 2 gene SNP rs2758331 (p=0.019). To test for joint effects of the two SNPs on transplant-free survival, the genotypes were grouped to form a risk score reflecting the cumulative number of risk alleles (0 to 4 alleles per patient). A higher risk score based on the VEGFA and SOD2 SNP genotypes was associated with worse transplant-free survival (p=3.02×10(-4)) after confounder adjustment. The total burden of risk alleles was additive; subjects with the highest risk score of 4 (n=59 subjects, 14.2% of the cohort) had a total covariate-adjusted hazard ratio of 15.64 for worse transplant-free survival., Conclusions: After cardiac surgery, infants who are homozygous for the high-risk alleles for both the VEGFA and SOD2 SNPs have an approximately 16-fold increased risk of death or heart transplant, suggesting that genetic variants are important modifiers of survival after surgery for congenital heart disease., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The National Healthcare Safety Network (NHSN) is a safety surveillance system managed by the Centers for Disease Control and Prevention that monitors procedure specific rates of surgical site infections (SSIs). At our institution, SSI data is collected and reported by three different methods: (1) the NHSN database with reporting to the Centers for Disease Control and Prevention; (2) the hospital billing database with reporting to payers; and (3) The Society of Thoracic Surgeons Congenital Heart Surgery Database. A quality improvement initiative was undertaken to better understand issues with SSI reporting and to evaluate the effect of different data sources on annual SSI rates., Methods: Annual cardiac surgery procedure volumes for all three data sources were compared. All episodes of SSI identified in any data source were reviewed and adjudicated using NHSN SSI criteria, and the effect on SSI rates was evaluated., Results: From January 1, 2008, to December 31, 2011, 2,474 cardiac procedures were performed and reported to The Society of Thoracic Surgeons Congenital Heart Surgery Database. Billing data identified 1,865 cardiac surgery procedures using the 63 CARD International Classification of Diseases-Ninth Revision codes from the NHSN inclusion criteria. Only 1,425 procedures were targeted for NHSN surveillance using the NHSN's CARD operative procedure group in the same period. Procedures identified for NHSN surveillance annually underestimated the number of cardiac operations performed by 17% to 71%. As a result, annual SSI rates potentially differed by 12% to 270%., Conclusions: The NHSN CARD surveillance guidelines for SSI fail to identify all pediatric cardiac surgical procedures. Failure to target all at-risk procedures leads to inaccurate reporting of SSI rates largely based on identifying the denominator. Inaccurate recording of SSI data has implications for public reporting, benchmarking of outcomes, and denial of payment. Use of The Society of Thoracic Surgeons Congenital Heart Surgery Database as the gold standard to identify procedures for surveillance will lead to more accurate reporting of SSI rates., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Hypoplastic left ventricle with scimitar syndrome is a rare combination of anatomic lesions. Five patients with this anatomy have been followed up at our institution. Four of these patients are alive: 2 after heart transplant, 1 underwent a Norwood operation, and 1 has survived staged palliation, culminating in a Fontan operation., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Coronary ostial atresia is a rare anomaly, most frequently managed with coronary artery bypass grafting. This condition is often misdiagnosed as anomalous left coronary artery from the pulmonary artery until the time of surgical intervention, leaving the surgeon little time to contemplate the best operative technique., Methods: In this single-center experience, three cases of congenital atresia of the left main coronary artery presenting during childhood were surgically managed with homograft patch ostioplasty., Results: Antegrade left coronary artery blood flow was successfully established in all three cases without the use of a bypass graft. Two children survived and are clinically well; the third had visibly necrotic myocardium at the time of surgical ostioplasty and did not survive to hospital discharge., Conclusions: Surgical ostioplasty with a homograft patch should be considered as an alternative to bypass grafting for coronary ostial atresia in children., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The purpose of this study was to assess deep hypothermic circulatory arrest (DHCA) as a modifier of neurodevelopmental (ND) outcomes in preschool children after cardiac surgery in infancy for repair of congenital heart defects (CHD)., Methods: This is a planned analysis of infants enrolled in a prospective study of apolipoprotein E polymorphisms and ND outcome after cardiac surgery. The effect of DHCA was assessed in patients with single or biventricular CHD without aortic arch obstruction. Neurodevelopmental assessment at 4 years of age included cognition, language, attention, impulsivity, executive function, social competence, and visual-motor and fine-motor skills. Patient and procedural variables were evaluated in univariate and multivariate models., Results: Neurodevelopmental testing was completed in 238 of 307 eligible patients (78%). Deep hypothermic circulatory arrest was used at the discretion of the surgeon at least once in 92 infants (38.6%) with a median cumulative duration of 36 minutes (range, 1 to 132 minutes). By univariate analysis, DHCA patients were more likely to have single-ventricle CHD (p = 0.013), lower socioeconomic status (p < 0.001), a higher incidence of preoperative ventilation (p < 0.001), and were younger and smaller at the first surgery (p < 0.001). By multivariate analysis, use of DHCA was not predictive of worse performance for any ND outcome., Conclusions: In this cohort of children undergoing repair of CHD in infancy, patients who underwent DHCA had risk factors associated with worse ND outcomes. Despite these, use of DHCA for repair of single-ventricle and biventricular CHD without aortic arch obstruction was not predictive of worse performance for any ND domain tested at 4 years of age., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Intraoperative transesophageal echocardiography (IOTEE) is well established as a monitoring tool during ventricular septal defect (VSD) repair to ensure complete closure of the defect. Residual shunts detected by IOTEE are common. The predictive value of IOTEE findings on the long-term course of residual shunts is not well documented, especially in regard to the need for reoperation or bacterial endocarditis prophylaxis. The objective of this study is to determine the predictive value of intraoperative IOTEE diagnosis of residual VSDs and therefore delineating the natural history of these findings., Methods: Retrospective review of IOTEE reports of 690 consecutive patients with VSD (isolated or part of a complex lesion) was undertaken. Those were compared with transthoracic echocardiographic reports of these patients before their discharge from the hospital, and the most recent transthoracic echocardiographic examination. Positive and negative predictive values, sensitivity, and specificity of such diagnoses were then calculated from predischarge and from follow-up transthoracic echocardiographic data., Results: There were 260 of 690 patients with a residual VSD on IOTEE; 24 required repeat cardiopulmonary bypass for complete closure. There were 573 patients with predischarge transthoracic echocardiographic examination; 296 had residual VSDs (125 not detected by IOTEE), and 13 defects required reoperation during the same hospitalization, 5 of which were detected by IOTEE. The positive and negative predictive values were 78% and 65%, respectively. Follow-up transthoracic echocardiographic examination of 383 local patients showed residual VSD in 57 (37 not detected by IOTEE), with positive and negative predictive values of 15% and 83%, respectively., Conclusions: Although IOTEE is sensitive enough to detect residual VSD shunts in many patients (37% of this cohort), the majority of these defects are trivial and resolve spontaneously, with a positive predictive value of only 15% on follow-up transthoracic echocardiographic examination and a rare need for reoperation., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The prevalence of perioperative stroke in infants undergoing operations for congenital heart disease has not been well described. The objectives of this study were to determine the prevalence of stroke as assessed by postoperative brain magnetic resonance imaging (MRI), characterize the neuroanatomic features of focal ischemic injury, and identify risk factors for its development., Methods: Brain MRI was performed in 122 infants 3 to 14 days after cardiac operation with cardiopulmonary bypass, with or without deep hypothermic circulatory arrest. Preoperative, intraoperative, and postoperative data were collected. Risk factors were tested by logistic regression for univariate and multivariate associations with stroke., Results: Stroke was identified in 12 of 122 patients (10%). Strokes were preoperative in 6 patients and possibly intraoperative or postoperative in the other 6 patients, and were clinically silent except in 1 patient who had clinical seizures. Arterial-occlusive and watershed infarcts were identified with equal distribution in both hemispheres. Multivariate analysis identified lower birth weight, preoperative intubation, lower intraoperative hematocrit, and higher blood pressure at admission to the cardiac intensive care unit postoperatively as significant factors associated with stroke. Prematurity, younger age at operation, duration of cardiopulmonary bypass, and use of deep hypothermic circulatory arrest were not significantly associated with stroke., Conclusions: The prevalence of stroke in infants undergoing operations for congenital heart disease was 10%, half of which occurred preoperatively. Most were clinically silent and undetected without neuroimaging. Mechanisms included thromboembolism and hypoperfusion, with patient-specific, procedure-specific, and postoperative contributions to increased risk.

Background: To our knowledge, late electrophysiologic outcomes after the Ross procedure have not been described. The purpose of this study was to assess rhythm and conduction disturbances at midterm follow-up after the Ross procedure., Methods: A cross-sectional analysis of Ross procedure survivors (January 1, 1995 to December 31, 2005) followed at our institution was performed, including resting and 24-hour ambulatory electrocardiography (Holter monitoring). Rhythm and conduction disturbances were described, and predictors of arrhythmia were identified., Results: Of 64 eligible patients, 47 (71%) participated. Median age at surgery was 8.7 years (age range, 34 days to 34 years). Twenty-five patients (53%) had isolated aortic valve disease and 22 (47%) had complex left-sided heart disease. At median follow-up of 8.9 years (range, 2.6-11.1 years), 46 patients (98%) exhibited sinus rhythm. Sinus node dysfunction (SND), defined as a pause of 2 seconds or longer or bradycardia for age, was present in 7 patients (15%). Complete heart block requiring a pacemaker occurred in 2 patients (4%). Ventricular tachycardia (VT) was present in 7 patients (15%), including nonsustained VT in 5 patients on Holter monitoring, and sustained VT in 2 patients requiring defibrillator placement. In multivariate analysis, concurrent arch repair at the time of the Ross operation (p = 0.04), longer cross-clamp time at the time of Ross operation (p = 0.04), and right ventricular outflow tract obstruction on follow-up echocardiogram (p = 0.03) were associated with SND. Longer cross-clamp time (p = 0.03) was also associated with VT, along with older age at surgery (p = 0.06 for trend)., Conclusions: At midterm follow-up after the Ross procedure, rhythm and conduction disturbances occur in one third of patients, including SND in 15%, atrioventricular block in 4%, and VT in 15%. Routine surveillance for late arrhythmias after the Ross procedure is warranted.

Background: Circulating levels of atrial natriuretic peptide and brain natriuretic peptide (BNP) are elevated in patients with cyanotic congenital heart disease and associated with the severity of ventricular dysfunction. We evaluated the effect of chronic hypoxemia on left ventricle pro-atrial natriuretic peptide and pro-BNP, the cytoplasmic precursors of the plasma hormones., Methods: Forty newborn piglets were randomized to placement of a pulmonary artery to left atrium shunt to create hypoxemia or sham thoracotomy. Animals were studied at 1 or 2 weeks after the procedure (four groups, n = 10 per group). Arterial oxygen tension and hematocrit were obtained. Left ventricular shortening fraction was measured by echocardiography. Left ventricular tissue was harvested and cytoplasm was extracted. Pro-BNP levels were determined by Western blot analysis. Pro-atrial natriuretic peptide levels were determined using enzyme-linked immunosorbent assay., Results: Significant differences among treatment groups were observed for arterial oxygen tension (p < 0.001) and hematocrit (p < 0.001). Pairwise comparisons indicated lower arterial oxygen tension and higher hematocrit for hypoxemic piglets compared with control piglets at 1 and 2 weeks. Left ventricular shortening fraction was not decreased in the hypoxemic animals at any time (p = 0.638). Left ventricular pro-atrial natriuretic peptide decreased in hypoxemic piglets (p = 0.029), whereas left ventricular pro-BNP increased in hypoxemic piglets at 2 weeks (p = 0.002)., Conclusions: Chronic hypoxemia alone, even in the absence of cardiac dysfunction, is sufficient to increase ventricular levels of pro-BNP. This finding may have implications for the interpretation of BNP levels in the clinical management of patients with cyanotic congenital heart disease.

Background: Hyperglycemia has been associated with worse outcome after traumatic brain injury and cardiac surgery in adults. It is not known whether postoperative hyperglycemia results in worse neurodevelopmental outcome after infant cardiac surgery., Methods: Secondary analysis of postoperative glucose levels was performed in infants younger than 6 months of age enrolled in a prospective study of genetic polymorphisms and neurodevelopmental outcomes who were undergoing repair of two-ventricle cardiac defects. Neurodevelopmental outcomes at 1 year of age were assessed with the Bayley Scales of Infant Development-II, yielding two indices: Mental Developmental Index and Psychomotor Developmental Index., Results: Surgical repair was performed in 247 infants with 1 in-hospital and 3 late deaths. Neurodevelopmental evaluation was performed in 188 of 243 (77%) survivors. Glucose levels at cardiac intensive care unit admission and during the first 48 postoperative hours were available for 180 of 188 patients. Mean admission glucose was 328 +/- 106 mg/dL; maximum glucose was 340 +/- 109 mg/dL. At least one glucose was greater than 200 mg/dL in 160 of 180 patients, and 49 of 180 patients (27%) had a glucose greater than 400 mg/dL. Only 1 patient had a glucose less than 50 mg/dL. Female sex (p = 0.02), but no other patient or operative variable, was associated with higher glucose levels. Mean Mental Developmental Index and Psychomotor Developmental Index were 90.6 +/- 14.9 and 81.6 +/- 17.2, respectively. Hyperglycemia was not associated with lower Mental Developmental Index and Psychomotor Developmental Index scores for the entire cohort or for neonates alone., Conclusions: Hyperglycemia is common early after infant cardiac surgery, but is not associated with worse neurodevelopmental outcome at 1 year of age.

Background: Despite recent improvements in survival of patients with hypoplastic left heart syndrome, those with severe atrial level restriction continue to face higher rates of mortality. We sought to assess the impact of prenatal diagnosis and immediate intervention on the outcome of this high-risk population., Methods: We performed a retrospective review of patients with hypoplastic left heart syndrome and restriction at the atrial level born between 1997 and 2006. Patients with an intact atrial septum or an interatrial communication less than 2 mm by color Doppler flow mapping on initial postnatal study, or evidence of obstruction to left atrial egress on fetal echocardiogram, were included. Patients were subdivided into two groups based on severity of obstruction., Results: Thirty-eight patients met inclusion criteria; 16 had the most severe form of anatomic obstruction (group 1), and 22 had a lesser degree of obstruction (group 2). Thirty-day cumulative survival for all was 68%: 38% for group 1 and 94% for group 2 (p = 0.001). Twenty-six of the 38 patients (68%) were prenatally diagnosed. Prenatal diagnosis did not improve initial hospital survival or cumulative survival for either group. Eight patients had progression of atrial level restriction from the first fetal study to the first postnatal study. Seven patients prenatally diagnosed with severe restriction underwent planned cesarean section followed by immediate surgical or catheter-based intervention, with only 2 survivors (28%)., Conclusions: Hypoplastic left heart syndrome with an intact atrial septum is a highly lethal condition despite prenatal diagnosis and immediate intervention at birth. Fetal intervention should be considered for these high-risk fetuses.

Background: Avoidance of cardiopulmonary bypass (CPB) and aortic cross-clamping during the Fontan procedure has been advocated to improve outcomes. We continue to use CPB with aortic cross-clamping for the Fontan procedure., Methods: We performed a review of patients undergoing the Fontan procedure between January 1, 2000 and December 31, 2004., Results: The Fontan procedure was performed in 160 patients. The median age was 2.2 years (range, 1.0 to 29.1 years). Hypoplastic left heart syndrome or a variant was present in 114 patients (71%), and heterotaxy was present in 19 (12%). CPB and modified ultrafiltration were used in all patients. Aortic cross-clamping was used in 154 (96%) of 160 patients and deep hypothermic circulatory arrest (DHCA) in 132 (83%). A lateral tunnel Fontan was performed in 69 patients (43%) and an extracardiac Fontan in 91 (57%). A fenestration was created in 144 patients (90%). Two patients died. Freedom from death or takedown was 98% (157/160). Median duration of pleural drainage was 2 days (range, 1 to 44 days) and was more than 14 days in 16 patients. Median duration of hospitalization was 6 days (range, 3 to 55 days). The small number of deaths precluded assessment of risk factors for mortality. By multivariable analysis, risk factors for pleural drainage longer than 3 days were extracardiac connection (p < 0.001) and increasing mean pulmonary artery pressure before the Fontan procedure (p = 0.033). By multivariable analysis, risk factors for hospitalization for more than 7 days were extracardiac connection (p = 0.003), increasing duration of total support (CPB and DHCA, p = 0.027), and decreasing systemic oxygen saturation before the Fontan procedure (p = 0.048)., Conclusions: The Fontan procedure can be performed using CPB and aortic cross-clamping with low morbidity and mortality.

Background: After utilizing separate congenital databases in the early 1990s, the Society of Thoracic Surgeons (STS) and the European Association for Cardio-Thoracic Surgery (EACTS) collaborated on several joint database initiatives., Methods: In 1998, the joint EACTS-STS International Congenital Heart Surgery Nomenclature and Database Project Committee was created and a common nomenclature and common core minimum database dataset were adopted and published by the STS and the EACTS. In 1999, the joint EACTS-STS Aristotle Committee was created and the Aristotle Score was adopted and published as a method to provide complexity adjustment for congenital heart surgery. Collaborative efforts involving the EACTS and STS are underway to develop mechanisms to verify data completeness and accuracy., Results: Since 1998, this nomenclature, database, and methodology of complexity adjustment have been used by both the STS and EACTS to analyze outcomes of over 40,000 patients. A huge amount of data have been generated which allow comparison of practice patterns and outcomes analysis between Europe and North America. The aggregate data from the first 5 years of data collection not only make for interesting comparison but also allow examination of regional difference in practice patterns. For example, in the EACTS, out of 4,273 neonates, 885 (20.7%) underwent arterial switch procedures and 297 (6.95%) underwent Norwood stage 1 procedures. In the STS, out of 3,988 neonates, 472 (11.8%) underwent arterial switch procedures and 575 (14.4%) underwent Norwood stage 1 procedures., Conclusions: This analysis of the EACTS-STS multi-institutional outcomes database confirms that in both Europe and North America, case complexity and mortality is highest among neonates, then infants, and then children. Regional differences in practice patterns are demonstrated, with the overall goal being the continued upgrade in the quality of surgery for congenital heart disease worldwide.

Background: Recent reports advocate that a right ventricular to pulmonary artery (RV-PA) conduit improves outcome after the stage I reconstruction., Methods: We retrospectively compared the outcomes of all neonates who underwent a stage I reconstruction between January 1, 2002, and October 1, 2004, with use of the RV-PA conduit and modified Blalock-Taussig shunt (mBTS) interspersed over this time period., Results: In all, 149 infants underwent a stage I reconstruction (95 mBTS, 54 RV-PA) for hypoplastic left heart syndrome (HLHS) or variants. There was a preference for the RV-PA conduit in patients with aortic atresia (mBTS 30% versus RV-PA 67%, p < 0.01). There was no difference in surgical mortality (mBTS 14% versus RV-PA 17%, p = 0.67), time to extubation (mBTS 4.5 +/- 4.8 days versus RV-PA 3.9 +/- 3.5 days, p = 0.47), or length of hospital stay (mBTS 25 +/- 29 days versus RV-PA 21 +/- 23 days, p = 0.52). There was an increased incidence of shunt reinterventions in the patients with the RV-PA conduit (mBTS 17% versus RV-PA 32%, p = 0.04). Patients with RV-PA conduit returned earlier for stage II reconstruction (mBTS 6.5 +/- 2.5 months versus RV-PA 5.6 +/- 1.7 months, p = 0.05). There was no difference in overall mortality (mBTS 32% versus RV-PA 30%, p = 0.45) with a median duration of follow-up of 18 +/- 8 months., Conclusions: Comparing shunt strategies (mBTS versus RV-PA) over the same time period, we found no difference in outcome. These data support the need for a larger prospective, randomized trial.

Background: The analysis of the second harvest (1998-2001) of the Society of Thoracic Surgeons Congenital Heart Surgery Database included the first attempt by the STS to apply a complexity-adjustment method to evaluate congenital heart surgery results., Methods: This data harvest represents the first STS multiinstitutional experience with software utilizing the international nomenclature and database specifications adopted by the STS and the European Association for Cardio-Thoracic Surgery (April 2000 Annals of Thoracic Surgery) and the first STS Congenital Database Report incorporating a methodology facilitating complexity adjustment. This methodology, allowing for complexity adjustment, gives each operation a basic complexity score (1.5 to 15) and level (1 to 4) based upon the work of the EACTS-STS Aristotle Committee, a panel of 50 expert surgeons. The complexity scoring, based on the primary procedure (from the EACTS-STS International Nomenclature Procedures Short List), estimates complexity through three factors: mortality potential, morbidity potential, and technical difficulty., Results: This STS harvest includes data from 16 centers reporting 12,787 cases, with discharge mortality known for 10,246 cases. The basic complexity score has been applied to the outcomes analysis of these cases and a new equation has been proposed to evaluate one aspect of performance: Aristotle Performance Index = Outcome x Complexity = (Survival) x (Mean Complexity Score), Conclusions: The complexity analysis represents a basic complexity-adjustment method to evaluate surgical results. Complexity is a constant precise value for a given patient at a given point in time; performance varies between centers. Future STS congenital data harvests will incorporate a second step, the Comprehensive Aristotle Score, utilizing additional patient specific complexity modifiers to allow a more precise complexity adjustment.

Background: We have previously reported that the outcome of infants with functional single ventricle and total anomalous pulmonary venous connection is poor relative to that of other single ventricle patients. Younger age at initial operation and obstructed total anomalous pulmonary venous connection were found to be risk factors for mortality. A review of our recent experience was undertaken to determine whether results in these patients are improving., Methods: Medical records of 18 patients admitted after 1997 were reviewed (group B) and compared with the previous group of 73 patients admitted between 1984 and 1997 (group A). Data were analyzed using a Cox proportional hazards model., Results: Median age at first operation was the same for both groups. The incidence of obstructed total anomalous pulmonary venous connection was not significantly different between groups (29% versus 33%, p = 0.70). Early survival is significantly improved for group B compared with group A (p = 0.015). Only group and younger age at initial operation were found to be risk factors for mortality. In the current group, 5 patients have undergone superior cavopulmonary connection with one death, 3 have undergone Fontan completion with no deaths, and heart or heart-lung transplantation was performed in 5 patients with two deaths., Conclusions: Early survival in patients with single ventricle and total anomalous pulmonary venous connection has improved significantly in recent years, but intermediate survival is still approximately 50%. Selective management using staged reconstructive surgery and transplantation may result in improved survival. Further study may identify risk factors for mortality and improve selection of appropriate patients for each therapy.

Background: Postpericardiotomy syndrome (PPS) occurs in 10% to 50% of pediatric patients after cardiac surgery. The incidence and outcome of PPS after permanent pacemaker implantation in children is not described., Methods: A retrospective analysis was performed of all pediatric patients who underwent isolated placement of a pacemaker between January 1984 and December 2002. Patients who underwent congenital heart surgery at the time of pacemaker implantation were excluded. PPS was diagnosed on the basis of clinical symptoms with echocardiographic confirmation of a pericardial effusion., Results: Four hundred and forty-three pacemakers (237 epicardial, 206 transvenous) were implanted in 370 patients (median age 10 years, range 2 months to 24 years). Eight (2%) episodes of PPS (6 epicardial, 2 transvenous) occurred in 7 patients. The median time from implantation to PPS was 12.5 days (range 8 to 22 days). Six (75%) episodes followed primary pacemaker implantation, two occurred after subsequent lead revision. Three patients were initially treated with medical therapy (1 nonsteroidal agents, 2 steroids), and 1 required subsequent pericardiocentesis. Five patients underwent initial pericardiocentesis followed by medication. One patient had echocardiographic recurrence of a pericardial effusion 3 weeks after a nonsteroidal taper, with resolution after nonsteroidal agents were reinitiated. One patient required a pericardial window for a persistent effusion. No pacemaker was explanted., Conclusions: PPS occurred in 2% of children undergoing isolated pacemaker implantation of both epicardial and transvenous systems. PPS is usually managed successfully with medical therapy. Patients with medical treatment failure were successfully treated with pericardiocentesis or the surgical creation of a pericardial window.

Background: Epicardial pacemakers are often required in children and young adults who cannot undergo a transvenous system because of patient size, vascular barriers, or significant residual intracardiac shunts. Prophylactic epicardial pacing leads, placed at the time of concomitant congenital heart surgery, may reduce a late thoracotomy or sternotomy. The efficacy of prophylactic epicardial leads in the pediatric population is unknown., Methods: A retrospective review of the cardiovascular surgery and pacemaker databases at The Children's Hospital of Philadelphia identified all patients less than or equal to 21 years of age, who underwent placement of an epicardial pacing lead between January 1, 1990 and December 31, 2002. Prophylactic epicardial pacing leads placed at the time of a concomitant congenital heart procedure were compared to standard epicardial leads that were connected to a simultaneous programable generator. Pacing and sensing threshold data were obtained in prophylactic epicardial leads at the time of lead retrieval and 6 month follow-up and compared to standard epicardial pacing leads., Results: Twenty-two (13 ventricular, 9 atrial) prophylactic epicardial pacing leads were retrieved in 13 patients at a median of 252 days (7 days to 3.98 years) from the time of initial implant and compared to 256 (164 ventricular, 92 atrial) standard epicardial leads placed in 142 patients. Nineteen (86%) prophylactic epicardial leads had acceptable pacing and sensing thresholds at lead retrieval. Only 1 patient with atrial and ventricular leads had poor pacing and sensing at retrieval and required a redo-sternotomy for placement of new atrial and ventricular epicardial pacing leads. For the remaining atrial (n = 7) and ventricular (n = 12) prophylactic epicardial leads, there was no significant difference in pacing (atrial, 1.59 +/- 1.1 microJ; ventricular, 1.98 +/- 1.9 microJ) or sensing (atrial, 3.6 +/- 1.8 mV; ventricular, 13.8 +/- 4.4 mV) compared to standard pacing (atrial, 2.1 +/- 1.8 microJ; ventricular, 1.9 +/- 3.4 microJ) and sensing (atrial, 3.3 +/- 1.7 mV; ventricular, 11.3 +/- 5.3 mV) epicardial leads. Six-month follow-up pacing and sensing thresholds were not significantly different between the prophylactic and standard epicardial pacing leads., Conclusions: Prophylactic epicardial pacing leads can be successfully placed and retrieved in a subset of children and young adults who will likely require pacing at a later date. Prophylactic leads have comparable pacing and sensing qualities at lead retrieval and short-term follow-up compared to standard epicardial leads. Consideration for prophylactic epicardial pacing leads will likely reduce the need for a late thoracotomy or sternotomy.

Background: Junctional ectopic tachycardia (JET) occurs commonly after pediatric cardiac operation. The cause of JET is thought to be the result of an injury to the conduction system during the procedure and may be perpetuated by hemodynamic disturbances or postoperative electrolyte disturbances, namely hypomagnesemia. The purpose of this study was to determine perioperative risk factors for the development of JET., Methods: Telemetry for each patient admitted to the cardiac intensive care unit from December 1997 through November 1998 for postoperative cardiac surgical care was examined daily for postoperative JET. A nested case-cohort analysis of 33 patients who experienced JET from 594 consecutively monitored patients who underwent cardiac operation was performed. Univariate and multivariate analyses were conducted to determine factors associated with the occurrence of JET., Results: The age range of patients with JET was 1 day to 10.5 years (median, 1.8 months). Univariate analysis revealed that dopamine or milrinone use postoperatively, longer cardiopulmonary bypass times, and younger age were associated with JET. Multivariate modeling elicited that dopamine use postoperatively (odds ratio, 6.2; p = 0.01) and age less than 6 months (odds ratio, 4.0; p = 0.02) were associated with JET. Only 13 (39%) of the patients with JET received therapeutic interventions., Conclusions: Junctional ectopic tachycardia occurred in 33 (5.6%) of 594 patients who underwent cardiac operation during the study period. Postoperative dopamine use and younger age were associated with JET. It may be speculated that dopamine should be discontinued in the presence of postoperative JET.

Background: Early outcomes after repair of total anomalous pulmonary venous connection continue to improve; however, little information is available concerning long-term functional status and quality of life., Methods: One hundred patients underwent isolated total anomalous pulmonary venous connection repair at The Children's Hospital of Philadelphia between 1983 and 2001. Medical records were reviewed and a standardized questionnaire was administered to guardians of survivors., Results: Median age at repair was 15.5 days (range, 1 to 563 days). Overall hospital mortality was 14%, decreasing from 19% before 1995 to 5% after 1995. At 15 years after repair, actuarial survival was 84% and freedom from late death or reintervention for hospital survivors was 85%. At a median follow-up of 5.9 years (range, 0 to 17.7 years) 64% of guardians described their child's overall health as excellent, 27% good, 9% fair, and 0% poor. With regard to school performance, 40% of children were characterized as above average, 29% average, 4% below average, and 27% were in special education classes or had repeated grades. By multivariable logistic regression, the presence of associated chromosomal or noncardiac syndromes and pulmonary venous obstruction were found to be significant factors with regard to parental assessment of both overall health and school performance., Conclusions: The majority of children who undergo isolated total anomalous pulmonary venous connection repair can expect an excellent long-term functional outcome. Factors present before operation, such as pulmonary venous obstruction and associated anomalies, can influence overall health and school performance in the long term.

Background: It is not known whether atrioventricular valve regurgitation (AVVR) can improve in some patients after the bidirectional cavopulmonary anastomosis (BCPA) without undertaking concomitant valvuloplasty., Methods: We retrospectively reviewed our experience with patients who underwent the BCPA between June 1989 and June 2000 with specific attention to those patients with moderate or severe AVVR. The degree of AVVR was determined by color Doppler echocardiography. During the study period there were 576 patients who underwent BCPA at a median age of 6.8 months (range, 1.3 months to 4.8 years). The majority of subjects (66%) had a tricuspid valve as the systemic atrioventricular valve. Significant (moderate or severe) AVVR before BCPA was noted in 36 of 576 patients (6%) and was significantly more prevalent (8 of 52 patients, 15%; p = 0.01) in patients with a common atrioventricular canal., Results: Of the 36 subjects with moderate or severe AVVR, 8 had interventions (6 valvuloplasty, 2 aortic arch dilation) at BCPA. There was 1 hospital death (2.8%). Of the remaining 27 survivors who did not undergo additional interventions at BCPA, improvement in AVVR (mild or none) was noted in 6 (22%) at intermediate follow-up (median, 2 years; range, 0.5 to 3.1 years). The presence of significant AVVR before BCPA was not significantly associated with hospital survival or intermediate-term freedom from death or transplantation., Conclusions: We conclude that AVVR improves in some patients after BCPA and that valvuloplasty is not justified in all patients with moderate preoperative AVVR.

Background: In congenital heart disease (CHD), neurologic abnormalities suggestive of hypoxia-ischemia are often apparent before cardiac surgery. To evaluate preoperative cerebral oxygenation, this study determined cerebral O2 saturation (ScO2) in CHD and healthy children., Methods: Ninety-one CHD and 19 healthy children aged less than 7 years were studied before surgical or radiologic procedures. Arterial saturation (SaO2) and ScO2 were measured by pulse-oximetry and near infrared cerebral oximetry. Cerebral O2 extraction (CEO2) was calculated (SaO2-ScO2). SaO2, ScO2, and CEO2 were compared among diagnoses. Multivariable regression was performed between ScO2 and clinical variables., Results: In healthy subjects, ScO2 (68%+/-10%) and CEO2 (30%+/-11%) were similar to patients with ventricular septal defect, aortic coarctation, and single ventricle after Fontan operation. ScO2 was significantly decreased in patients with patent ductus arteriosus (53%+/-8%), tetralogy of Fallot (57%+/-12%), hypoplastic left heart syndrome (46%+/-8%), pulmonary atresia (38%+/-6%), and single ventricle after aortopulmonary shunt (50%+/-7%), or bidirectional Glenn operation (43%+/-6%). CEO2 was significantly different only in patent ductus arteriosus (46%+/-8%) and hypoplastic left heart syndrome (38%+/-12%). In multivariable regression, only SaO2 was related to ScO2 (R = 0.63, p < 0.001)., Conclusions: Cerebral oxygenation in CHD varies with anatomy and arterial saturation, and in some patients, it is very low compared with healthy subjects.

Background: Significant atrioventricular valve (AVV) insufficiency has been associated with increased mortality and morbidity in patients with single ventricle. Although many patients can be managed with valvuloplasty alone, some patients require AVV replacement. The optimal timing, outcome, and risk factors for AVV replacement in this population have not been described., Methods: We retrospectively reviewed our experience with AVV replacement in patients with single ventricle from January 1984 to August 2000. Outcome variables included mortality and valve-related complications., Results: Seventeen patients required AVV replacement. Prosthetic valve types included: St. Jude's valve in 14, Bjork-Shiley in 1, Hall-Kaster in 1, and Carpentier-Edwards in 1. Valve size ranged from 17 to 33 mm, Median age at valve replacement was 3.0 years (range 7 days to 17.3 years). Of the 16 subjects with normal atrioventricular conduction preoperatively, 7 (44%) developed postoperative complete heart block. Hospital mortality was 29%. Hospital mortality decreased significantly from 56% in 1984 to 1993 to no deaths from 1994 to 2000 (p = 0.03). Younger age (less than 2 years) at operation was also a risk factor for hospital mortality (p = 0.03). There were four late deaths in this series and 1 patient underwent heart transplantation. Of the surviving patients, none has required replacement of the prosthetic valve. No patients have had cerebrovascular accident subsequent to AVV replacement. Functional status is New York Heart Association functional class I in 5, class II in 1, and Class III in 1., Conclusions: Atrioventricular valve replacement can be performed in patients with single ventricle with acceptable morbidity and mortality. The development of postoperative complete heart block is common. Survival after AVV replacement has improved in recent years, and intervention before patients develop ventricular dysfunction and atrial arrhythmias may further improve outcome.

Background: Thrombus formation is common after a Fontan operation. We investigated the frequency and location of thrombus in our population of children based on the type of Fontan operation performed., Methods and Results: Between January 1987 and January 1999, 592 patients underwent echocardiography after Fontan operation and 52 (8.8%) had intracardiac thrombus. Median age at Fontan operation was 1.9 years (range 0.8 to 35.1). Freedom from thrombus was 92%, 90%, 84% and 82% at 1, 3, 8, and 10 years after Fontan operation, respectively. There was no difference in freedom from thrombus, based on type of operation (atriopulmonary vs. lateral tunnel) or presence of fenestration. Thrombus was detected in the systemic venous atrium in 26 (48%), in the pulmonary venous atrium in 22 (44%), in both atria in 1 (2%), in the hypoplastic left ventricular cavity in 2 (8%), and in the ligated pulmonary artery stump in 1 (2%)., Conclusions: Thrombus formation occurs with equal frequency in all types of modifications and is seen in the pulmonary, as well as the systemic venous atria. Our study suggests that thrombus formation is inherent to the physiology after Fontan operation and is not related to the type of modification performed.

We describe a simple technique for the implantation of left atrial epicardial pacing leads in children with congenital heart disease who have undergone multiple operations. The pulmonary veins are exposed to reveal the pulmonary venous to atrial confluence using a left thoracotomy. A pacemaker lead is secured to the posterior left atrium inferior to the lower pulmonary vein. This approach provides a reliable site for atrial lead placement without the need for extensive dissection.

Background: Although operative mortality for reconstructive surgery for hypoplastic left heart syndrome continues to improve, nonoperative mortality, especially in the first year of life, remains relatively high. A number of patients who are thought to be clinically well at hospital discharge die unexpectedly. The goal of the present study was to determine the incidence of and risk factors for unexpected death in patients with hypoplastic left heart syndrome., Methods: Retrospectively, we determined the incidence of unexpected death among 536 patients with hypoplastic left heart syndrome who were discharged to home after stage I surgical procedure. To identify potential risk factors, a nested case-control analysis was undertaken., Results: Unexpected death occurred in 22 of 536 patients (4.1%) discharged to home after stage I surgical procedure. The median age at unexpected death was 79 days (range, 25 to 227 days). Seizures preceded cardiac arrest in 2 patients, and ventricular arrhythmias were documented in 3 additional patients during attempted resuscitation. Autopsy studies were performed in 12 patients and identified residual lesions that may have contributed to death in 2 patients. In multivariate analysis documented perioperative arrhythmia and earlier year of stage I surgical procedure were associated with an increased risk for unexpected death (p = 0.03 and p = 0.04, respectively). There were 4 additional patients who had unexpected death after subsequent cavopulmonary operation at a median age of 1.6 years (range, 0.9 to 3.8 years)., Conclusions: Unexpected death occurred in more than 4% of patients with hypoplastic left heart syndrome who were discharged to home after stage I surgical procedure and was most common in the first several months of life. Factors that may contribute to unexpected death include residual lesions, arrhythmias, and neurologic events, although in the majority of cases the cause remains largely unknown.

A case of bilateral sequential lung transplantation for anhidrotic ectodermal dysplasia is presented. The patient was a 16-year-old male with end-stage lung disease secondary to chronic severe respiratory infection. Although a relatively rare disease, the common association of fatal pulmonary compromise in those affected with this disorder warrants consideration of lung transplantation as a viable therapeutic option.

The extant nomenclature for end-stage lung disease is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories, using synonyms where appropriate. Indications for lung transplantation are coded under a broad category called pulmonary failure. The proposed hierarchical scheme also allows classification of complications of lung transplantation under a category called status post lung transplant. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented, which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

The extant nomenclature for systemic venous anomalies is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. This nomenclature system classifies systemic venous anomalies into two primary groups by venous segment: (1) systemic venous anomalies, superior vena cava; and (2) systemic venous anomalies, inferior vena cava. Subsets are clearly defined and categorized. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Cardiac transplantation provides the best option for neonates with congenital heart disease that is not amenable to surgical repair or palliation. The scarcity of suitable organs for this group has resulted in prolonged waiting times; many infants die awaiting transplantation. We present the case of a newborn with severe Ebstein's anomaly and low cardiac output who was supported with extracorporeal membrane oxygenation for 1,126 hours, until an appropriate organ became available.

Background: Complex congenital heart disease (CHD) often necessitates the use of epicardial pacing. Adequate right atrial (RA) sensing and pacing thresholds are often difficult to obtain due to suture line scarring and RA dilatation. The purpose of this study was to evaluate the placement of left atrial (LA) epicardial leads in children., Methods: Patient demographics, pacing, and sensing data of atrial pacing systems implanted between January 1994 and January 1997 were collected., Results: Forty-nine pacing systems were implanted: 14 LA epicardial, 19 RA epicardial, and 16 transvenous in the right atrium. Lead impedance, current, and energy were similar in the two epicardial groups throughout the study. Energy thresholds (ET) were lower in the LA than RA at 6 months, and 1 and 2 years (p < 0.05). Analysis of post-Fontan patients performed alone revealed a lower ET in the LA as compared with the RA. Pacing and sensing parameters from transvenous leads are presented for relative comparison., Conclusions: Transvenous leads are most efficient but often contraindicated in complex CHD. LA leads offer lower energy thresholds than RA leads with similar sensing parameters.

Background: Large-volume hemoptysis during cardiopulmonary bypass is an infrequent, but life-threatening event. Rapid airway clearance and control are the primary prerequisites for successful management., Methods: The cases of 3 patients with different sources of exsanguinating hemoptysis during cardiopulmonary bypass managed initially with rigid bronchoscopy were reviewed., Results: In all patients, airway control was rapidly established and weaning from cardiopulmonary bypass CPB was accomplished. Two patients survived the operative procedure. The other patient died in the operating room of unremitting bilateral pulmonary hemorrhage., Conclusions: Major hemoptysis during cardiopulmonary bypass is best dealt with initially by rapid airway control and cessation of bypass in an expeditious manner. An algorithm for suggested management is provided. The rigid bronchoscope is the optimal tool for initial management and it should always be available. Definitive treatment is determined by the cause and the persistence of hemorrhage once these maneuvers have been performed.

Background: Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression., Methods: Since January 1, 1984, 28 patients with TOF/ APV have undergone complete repair (median age 11 days, range 1 day to 16 years)., Results: Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/ posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04)., Conclusions: Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.

Pulmonary arteriovenous malformation can occur in patients with functional single ventricle after a cavopulmonary connection. There is no effective medical treatment for this complication. Pulmonary arteriovenous malformations may regress over time after heart transplantation, but may be a source of significant perioperative morbidity. We report the case of a woman with single ventricle, ventricular dysfunction, and bilateral pulmonary arteriovenous malformations who had successful treatment of both cardiac and pulmonary failure with en bloc heart and right lung transplantation.

Background: The Ross procedure is performed for a variety of left ventricular outflow tract diseases in children. The preoperative hemodynamic burden of pressure or volume overload and associated ventricular hypertrophy can predispose to ventricular arrhythmias. Additional procedures performed with the Ross procedure (eg, Konno) may damage the conduction system., Methods: Between January 1995 and February 1997, the Ross procedure was performed in 42 patients, 31 (74%) of whom had 71 prior interventions. Concomitant procedures (n = 42 in 23 patients) included 17 annular-enlarging procedures. Screening was performed for perioperative conduction and rhythm abnormalities., Results: There was one postoperative death. Perioperative ventricular tachycardia occurred in 12 patients (29%), with 2 receiving antiarrhythmic medication for ventricular tachycardia at discharge. Transient complete heart block occurred in 3 patients, all of whom had concomitant procedures performed in the subaortic area; all patients were discharged in sinus rhythm and no patient received a permanent pacemaker., Conclusions: The Ross procedure can be performed successfully in children with complex cardiac disease with low mortality and perioperative morbidity. The incidence of perioperative ventricular tachycardia is high (29%), suggesting the need for vigilant perioperative monitoring and long-term surveillance.

Background: The mechanisms by which increased pulmonary blood flow results in pulmonary hypertension have not been determined., Methods: To determine if increased pulmonary blood flow produces endothelial dysfunction that precedes vascular remodeling and smooth muscle proliferation, neonatal swine (n = 12) (age, 6.1+/-0.5 days) underwent ligation of the left pulmonary artery (LPA) to increase blood flow to the right lung. At 12 weeks of age, endothelium-dependent vasodilatation was assessed by acetylcholine infusion and endothelium-independent vasodilatation by inhaled nitric oxide (NO) in the LPA group and age-matched controls (CON) (n = 11)., Results: Mean pulmonary artery pressure was 24.1+/-3.0 mm Hg in the LPA group and 20.8+/-1.9 mm Hg in the CON group (p < 0.1). Pulmonary vascular resistance was 13.2+/-2.2 Wood units in the LPA group and 5.8+/-0.8 Wood units in the CON group (p = 0.001). Acute occlusion of the left pulmonary artery in the CON group increased pulmonary vascular resistance to 6.9+/-3.9 Wood units (p = 0.04). Administration of acetylcholine in the CON group after preconstriction with the thromboxane A2 analogue U46619 resulted in a 30.6%+/-5.4% decrease in pulmonary vascular resistance. In the LPA group, acetylcholine produced paradoxical vasoconstriction and a 15.4%+/-4.1% increase in pulmonary vascular resistance (p < 0.001 versus CON) indicating loss of endothelium-dependent vasodilatation. Nitric oxide decreased pulmonary vascular resistance by 41.9%+/-3.3% in the CON group and 30.8%+/-2.7% in the LPA group (p = 0.04 versus CON), indicating preserved endothelium-independent vasodilatation in both groups. Morphometric analysis was performed in 4 animals from each group. Medial wall thickness as percent of external diameter of small arteries (<100 microm) was the same in both groups (6.4%+/-0.4% in the LPA group versus 6.6% +/-0.4% in the CON animals; p > 0.1)., Conclusions: Increased pulmonary blood flow in immature animals produces endothelial cell dysfunction with loss of endothelium-dependent vasodilatation before the onset of pulmonary vascular remodeling. Subsequent smooth muscle proliferation may be mediated by endothelium-derived factors.

Background: Pediatric thoracic organ transplantation is associated with an increased risk of perioperative bleeding. Many of these patients are undergoing repeat surgical procedures and in general require cardiopulmonary bypass., Methods: This article reviews the efficacy and safety of the serine protease inhibitor aprotinin in improving hemostasis in pediatric transplantation., Results: A review of the literature and investigations from Children's Hospital of Philadelphia suggest that aprotinin is beneficial in pediatric lung transplantation: high-risk patients do as well as low-risk patients. Aprotinin also appears to be of benefit in redo heart transplantations, particularly in patients who have had previous sternotomy or previous transplantation. Repeat use of aprotinin appears to be safe and does reduce blood loss in retransplantation patients. Use in the pump prime and a maintenance dose of aprotinin may be the most effective protocol. At this time, however, it is uncertain whether aprotinin is valuable in primary heart transplantation in low-risk patients., Conclusions: Current practice at Children's Hospital of Philadelphia is to use aprotinin in all lung and heart-lung transplantations and in all redo transplantations: lung, heart-lung, and heart. The use of aprotinin in primary heart transplantations is limited to patients who have had previous sternotomies or thoracotomies.

An anomalous left brachiocephalic vein is an uncommon systemic venous anomaly, which usually has no clinical significance. We describe a case of tricuspid atresia with such an anomalous left brachiocephalic vein. The presence of this unusual venous anomaly had a number of implications in the surgical management of the tricuspid atresia.

Background: Superior cavopulmonary connection reduces the volume work of the single ventricle., Methods: To determine the effects of superior cavopulmonary connection on preload, wall stress (or afterload), and systolic ventricular function, we studied 9 patients before and after operation, and at hospital discharge. Using echocardiography, preload was estimated by the ventricular end-diastolic area, and wall stress was calculated at end-systole and peak-systole. Ventricular function was represented by rate-corrected velocity of circumferential fiber shortening and fractional area change divided by rate-corrected ejection time., Results: End-diastolic area and wall stress decreased postoperatively. Ventricular wall thickness increased with a concomitant decrease in cavity area. There was no change in mean blood pressure or heart rate or in rate-corrected velocity of circumferential fiber shortening or fractional area change divided by rate-corrected ejection time. These findings persisted at hospital discharge., Conclusions: In single ventricles, superior cavopulmonary correction results in an immediate decrease in preload and afterload. The decrease in afterload results primarily from alterations in ventricular geometry. Although no improvement in systolic function was noted, diminished work related to the reduction in loading conditions may have beneficial long-term effects on preserving myocardial performance.

Background: Modified ultrafiltration reduces the deleterious effects of cardiopulmonary bypass in children. Patients undergoing repair of single-ventricle cardiac anomalies may be particularly sensitive to these adverse effects, and benefit from the use of modified ultrafiltration., Methods: From January 1995 to June 1996, 120 consecutive cavopulmonary operations were performed at The Children's Hospital of Philadelphia. Procedures included lateral tunnel fenestrated Fontan (n = 50), extracardiac Fontan (n = 5), hemi-Fontan (n = 60), and bidirectional Glenn shunt (n = 5). Modified ultrafiltration was performed after cardiopulmonary bypass in 41 patients, and results were compared by t test with a control group of 79 patients in whom modified ultrafiltration was not used., Results: There was one death for an operative (30-day) mortality of 0.8%. Age, weight, diagnosis, ischemic arrest time, and cardiopulmonary bypass time were similar between the modified ultrafiltration and control groups. Postoperative blood use, chest tube output, the incidence of pleural and pericardial effusions, and hospital stay were all significantly decreased when modified ultrafiltration was used., Conclusions: By lowering the perioperative morbidity of staged cavopulmonary operations, modified ultrafiltration makes an important contribution to improving outcome after the correction of single-ventricle cardiac anomalies.