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The most common long-term complication following arterial switch is supravalvar neopulmonary stenosis. Balloon dilatation has not been effective in treating this problem since the stenosis is often found at multiple levels which will require complex surgical interventions. These include patch augmentation of the supravalvar apparatus, patch augmentation of the distal main and branch pulmonary arteries, and subvalvar muscular resection as needed. The surgical considerations to avoid and treat supravalvar neopulmonary stenosis are: (1) prevention at the time of arterial switch by the use of a pantaloon autologous pericardial patch and (2) application of appropriate surgical interventions at secondary operations for acquired right ventricular outflow tract obstruction. The purpose of this article is to illustrate the optimal methods to achieve these goals.

Systemic-to-pulmonary shunt failure is a potentially catastrophic complication. We analyzed a large multicenter clinical registry to describe the prevalence and evaluate risk factors.Infants (aged ≤365 days) undergoing shunt operations (systemic artery-to-pulmonary artery or systemic ventricle-to-pulmonary artery) in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) from 2010 to 2015 were included. Multivariable logistic regression was used to evaluate risk factors for in-hospital shunt failure. Model covariates included patient characteristics, preoperative factors, procedural factors including shunt type, and center effects. Centers with more than 15% missing data for key covariates were excluded.Shunt operations were performed in 9,172 infants (118 centers). In-hospital shunt failure occurred in 674 (7.3%). In multivariable analysis, risk factors for in-hospital shunt failure included lower weight at operation (odds ratio [OR], 1.35; p = 0.001), preoperative hypercoagulable state (OR, 2.47; p = 0.031), and the presence of any other STS-CHSD preoperative risk factors (OR, 1.24; p = 0.038). Shunt failure was less likely with a systemic ventricle-to-pulmonary artery shunt than a systemic artery-to-pulmonary artery shunt (OR, 0.65; p = 0.020). Neither cardiopulmonary bypass nor single-ventricle diagnosis was a risk factor for shunt failure. Patients with in-hospital shunt failure had significantly higher rates of operative mortality (31.9% vs 11.1%, p 0.001) and major morbidity (84.4% vs 29.4%, p0.001), and longer median postoperative length of stay among survivors (45 vs 22 days, p0.001).In-hospital shunt failure is common, and associated mortality risk is high. These data highlight at-risk patients and procedural cohorts that warrant expectant surveillance and may benefit from enhanced antithrombotic prophylaxis or other management strategies to reduce shunt failure. These findings may inform planning of future clinical trials.

Biykem Bozkurt, MD, PhD, FACC, FAHA, Chair H. Vernon Anderson, MD, FACC, FAHA Garth N. Graham, MD, FACC Hani Jneid, MD, FACC, FAHA Gail K. Jones, MD David Kao, MD, FAHA Michael Kutcher, MD, FACC Leo Lopez, MD, FACC Gregory Marcus, MD, FACC, FAHA Jennifer Rymer, MD James E. Tcheng, MD

The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%).

In pediatric patients with connective tissue disorders (CTDs), early cardiac presentation often involves severe mitral regurgitation (MR) associated with severe bileaflet prolapse and, less frequently, aortic root enlargement. We adopted a simplified repair to address MR and prevent systolic anterior motion (SAM) in this unique group of patients.Retrospective review of clinical and echocardiographic data of all pediatric patients (age 18 years) with CTD and MR undergoing simplified repair at 3 institutions (2000-2014).Eighteen children who underwent surgery for severe MR and bileaflet prolapse were identified. All were treated with ring annuloplasty and Alfieri edge-to-edge repair. Median age and weight were 8.2 years (range, 0.4-17.2 years) and 24.9 kg (5.6-63.3 kg), respectively. Median left ventricular end diastolic dimension median z score was 4.9 (2.1-11.9). One patient died (5.6%), and there were no other major complications. Among survivors, 94.4% had mild regurgitation or less, with no stenosis or SAM at median clinical follow-up of 2.4 years (range, 0-13.9 years). Median left ventricular end-diastolic dimension z score regressed to 1.3 (-0.5 to 4.3).In pediatric patients with CTD and severe MR, a simplified approach is associated with intermediate-term competence, absence of SAM or significant stenosis, and regression of left ventricular enlargement.

Robert M. Sade, MD The ethical bedrock of contemporary health care is informed consent. We cannot touch others without their permission to do so. The flip side of the informed consent coin is informed refusal. Patients or their surrogate decision makers can refuse care at any time, even if the proffered treatment is life-saving. The refusal can be stated in advance in the form of limitations on future care. Sometimes limits of this kind can seem to an attending physician to be unwise or even foolish, but if they remain after efforts to change them through persuasion, they cannot be disregarded. The following vignette describes just such a case, in which the surgeon has to make a choice of how to respond. The Case of the Inflexible Wife Eighty-year old M.D. Baker was being treated for diabetes, hypertension, and chronic kidney disease with a creatinine of 2.5. When he developed chest pain, a CT scan demonstrated a type A aortic dissection with a false lumen extending into the left common carotid artery and proximal descending aorta. Mr. Baker became confused and was intubated and transferred to the university hospital. On arrival, he is sedated but moving all four extremities. He needs urgent surgery and the cardiac surgeon, Dr. Solomon, discusses plans for ascending aortic replacement with Baker’s family. The patient's wife is his health care agent under his health care durable power of attorney; she says that the patient "would like everything done," but also that "he had lived a good life, doesn’t want CPR, and doesn’t want to be on a respirator." Dr. Solomon tells Mrs. Baker that this is a high risk operation and that complications such as kidney failure and prolonged mechanical ventilation are reasonably likely. She says, "OK, but if he’s not better in a week, we will withdraw support — including dialysis." On further discussion, her position remains adamant. Should the surgeon accept these conditions and do the operation?

Previous analyses of The Society of Thoracic Surgeons (STS) Adult Cardiac Surgery Database have demonstrated a reduction over time of risk-adjusted operative mortality after coronary artery bypass grafting. The STS Congenital Heart Surgery Database (STS CHSD) was queried to assess multiinstitutional trends over time in discharge mortality and postoperative length of stay (PLOS).Since 2009, operations in the STS CHSD have been classified according to STAT (The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) Congenital Heart Surgery Mortality Categories. The five STAT Mortality Categories were chosen to be optimal with respect to minimizing variation within categories and maximizing variation between categories. For this study, all index cardiac operations from 1998 to 2014, inclusive, were grouped by STAT Mortality Category (exclusions: patent ductus arteriosus ligation in patients weighing less than or equal to 2.5 kg and operations that could not be assigned to a STAT Mortality Category). End points were discharge mortality and PLOS in survivors for the entire period and for 4-year epochs. The Cochran-Armitage trend test was used to test the null hypothesis that the mortality was the same across epochs, by STAT Mortality Category.The analysis encompassed 202,895 index operations at 118 centers. The number of centers participating in STS CHSD increased in each epoch. Overall discharge mortality was 3.4% (6,959 of 202,895) for 1998 to 2014 and 3.1% (2,308 of 75,337) for 2011 to 2014. Statistically significant improvement in discharge mortality was seen in STAT Mortality Categories 2, 3, 4, and 5 (p values for STAT Mortality Categories 1 through 5 are 0.060,0.001, 0.015,0.001, and0.001, respectively). PLOS in survivors was relatively unchanged over the same time intervals. Sensitivity analyses reveal that the finding of declining risk-stratified rates of discharge mortality over time is not simply attributable to the addition of more centers to the cohort over time.This 16-year analysis of STS CHSD reveals declining discharge mortality over time, especially for more complex operations.

Background Atrial arrhythmias and progressive circulatory failure frequently develop in patients with a Fontan circulation. Improvement of flow dynamics and revision of the arrhythmia substrate may improve outcomes in selected patients. We sought to determine intermediate-term outcomes after Fontan conversion with arrhythmia operations and identify characteristics associated with decreased transplant-free survival. Methods The first 140 Fontan conversions with arrhythmia operations at a single institution were analyzed for predictors of cardiac death or transplant and incidence of arrhythmia recurrence. Results The median age at the Fontan conversion operation was 23.2 years (range, 2.6 to 47.3 years). Preoperative arrhythmias were present in 136 patients: right atrial tachycardia in 48 patients, left atrial tachycardia in 21, and atrial fibrillation in 67. Freedom from cardiac death or transplant was 90% at 5 years, 84% at 10 years, and 66% at 15 years. The median age at the last follow-up among survivors was 32 years (range, 15 to 61 years). By multivariable analysis, risk factors for cardiac death or heart transplantation were a right or indeterminate ventricular morphology, cardiopulmonary bypass time exceeding 240 minutes, ascites, protein-losing enteropathy, or a biatrial arrhythmia operation at the time of conversion. Freedom from recurrence of atrial tachycardia was 77% at 10 years. Among 67 patients with atrial fibrillation undergoing biatrial arrhythmia operations, none had recurrent atrial fibrillation. Conclusions Freedom from cardiac death or transplant for patients undergoing Fontan conversion with an arrhythmia operation is 84% at 10 years. The effects of atrial arrhythmia operations are durable in most patients. These outcomes may serve as useful benchmarks for alternative management strategies.

Adults with anomalous left coronary artery from the pulmonary artery typically have multiple venous and arterial collateral arteries surrounding the artery, making precise ligation imperative. Most physicians delay mitral valve repair until left ventricular function improves allowing for reperfusion through a 2-coronary system reimplanting the anomalous left coronary artery from the pulmonary artery into the aorta.

Specific congenital heart anomalies significantly increase the risk for late atrial arrhythmias, raising the question whether prophylactic arrhythmia operations should be incorporated into reparative open heart procedures. Currently no consensus exists regarding standard prophylactic arrhythmia procedures. Questions remain concerning the arrhythmia-specific lesions to perform, energy sources to use, need for atrial appendectomy, and choosing a right, left, or biatrial Maze procedure. These considerations are important because prophylactic arrhythmia procedures are performed without knowing if the patient will actually experience an arrhythmia. This review identifies congenital defects with a risk for the development of atrial arrhythmias and proposes standardizing lesion sets for prophylactic arrhythmia operations.

In this descriptive analysis, data in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database pertaining to patients who underwent reoperative cardiac surgery were analyzed. Practice patterns and outcomes are described. Reoperative cardiac surgery for congenital heart disease is common, with one third of index operations in the database occurring subsequent to prior cardiothoracic operation(s) performed on cardiopulmonary bypass. This analysis suggests that a history of previous cardiac surgery does not independently confer a significant incremental risk of operative mortality, but that patients with greater number of previous operations appear to be at higher risk.

In the end, ethical principles of autonomy (respect for the individual), beneficence (doing good for the patient), non-maleficence (avoiding harm), and justice (treat all patients fairly) support the patient in doing what she wants to do with her own life. However, thoracic surgical societies are under no obligation to facilitate this practice, given the potential negative consequences to our health care structure, our culture, and our economic well-being.

was affirmed in a significant number of patients who tolerated the persistent effects of right ventricular volume overload for many years. In a significant number of postoperative patients, however, long-term complications of pulmonary regurgitation, residual ventricular septal defects (VSD), distal pulmonary artery stenosis, right ventricular dysfunction, atrial or ventricular arrhythmias, and exercise intolerance 2-5 required further operative intervention to address the residual defects, perform concomitant arrhythmia surgery, and pulmonary valve insertion.

Background We evaluated outcomes for groups of risk-stratified operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database to provide contemporary benchmarks and examine variation between centers. Methods Patients undergoing surgery from 2005 to 2009 were included. Centers with more than 10% missing data were excluded. Discharge mortality and postoperative length of stay (PLOS) among patients discharged alive were calculated for groups of risk-stratified operations using the five Society of Thoracic Surgeons–European Association for Cardio-Thoracic Surgery Congenital Heart Surgery mortality categories (STAT Mortality Categories). Power for analyzing between-center differences in outcome was determined for each STAT Mortality Category. Variation was evaluated using funnel plots and Bayesian hierarchical modeling. Results In this analysis of risk-stratified operations, 58,506 index operations at 73 centers were included. Overall discharge mortality (interquartile range among programs with more than 10 cases) was as follows: STAT Category 1=0.55% (0% to 1.0%), STAT Category 2=1.7% (1.0% to 2.2%), STAT Category 3=2.6% (1.1% to 4.4%), STAT Category 4=8.0% (6.3% to 11.1%), and STAT Category 5=18.4% (13.9% to 27.9%). Funnel plots with 95% prediction limits revealed the number of centers characterized as outliers by STAT Mortality Categories was as follows: Category 1=3 (4.1%), Category 2=1 (1.4%), Category 3=7 (9.7%), Category 4=13 (17.8%), and Category 5=13 (18.6%). Between-center variation in PLOS was analyzed for all STAT Categories and was greatest for STAT Category 5 operations. Conclusions This analysis documents contemporary benchmarks for risk-stratified pediatric cardiac surgical operations grouped by STAT Mortality Categories and the range of outcomes among centers. Variation was greatest for the more complex operations. These data may aid in the design and planning of quality assessment and quality improvement initiatives.

Background We evaluated outcomes for common operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSDB) to provide contemporary benchmarks and examine variation between centers. Methods Patients undergoing surgery from 2005 to 2009 were included. Centers with greater than 10% missing data were excluded. Discharge mortality and postoperative length of stay (PLOS) among patients discharged alive were calculated for 8 benchmark operations of varying complexity. Power for analyzing between-center variation in outcome was determined for each operation. Variation was evaluated using funnel plots and Bayesian hierarchical modeling. Results Eighteen thousand three hundred seventy-five index operations at 74 centers were included in the analysis of 8 benchmark operations. Overall discharge mortality was: ventricular septal defect (VSD) repair=0.6% (range, 0% to 5.1%), tetralogy of Fallot (TOF) repair=1.1% (range, 0% to 16.7%), complete atrioventricular canal repair (AVC) = 2.2% (range, 0% to 20%), arterial switch operation (ASO) = 2.9% (range, 0% to 50%), ASO + VSD=7.0% (range, 0% to 100%), Fontan operation=1.3% (range, 0% to 9.1%), truncus arteriosus repair=10.9% (0% to 100%), and Norwood procedure=19.3% (range, 0% to 100%). Funnel plots revealed that the number of centers characterized as outliers were VSD=0, TOF=0, AVC=1, ASO=3, ASO + VSD=1, Fontan operation=0, truncus arteriosus repair=4, and Norwood procedure=11. Power calculations showed that statistically meaningful comparisons of mortality rates between centers could be made only for the Norwood procedure, for which the Bayesian-estimated range (95% probability interval) after risk-adjustment was 7.0% (3.7% to 10.3%) to 41.6% (30.6% to 57.2%). Between-center variation in PLOS was analyzed for all operations and was larger for more complex operations. Conclusions This analysis documents contemporary benchmarks for common pediatric cardiac surgical operations and the range of outcomes among centers. Variation was most prominent for the more complex operations. These data may aid in quality assessment and quality improvement initiatives.

Background The purpose of this study is to review our experience with late reoperations after the arterial switch operation (ASO) and to introduce reparative solutions adapted from previous techniques. Methods A retrospective study was performed on 23 patients who underwent late reoperations after ASO between 1983 and 2010. Eighteen patients were from our concomitantly reported cohort of 258 ASO patients and 5 came from distant referrals. Results Twenty-seven reoperations on 23 patients were performed for lesions relating to coronary arteries (9 procedures, 7 patients), the neoaortic root (12 procedures, 10 patients), and the right ventricular outflow tract (6 procedures, 6 patients). Four patients died: 1 from an exsanguinating gastric ulcer 4 years after prosthetic valve replacement; 1 from coronary occlusion one month postoperatively from an unroofed intramural left main coronary artery; and 2 after supravalvar pulmonary artery stenosis repair complicated by coexisting left ventricular dysfunction from the original ASO. Conclusions The ASO remains the treatment of choice for transposition of the great arteries and its variants. While the incidence of late reintervention is low, a subset of patients will require operations that extend the principles of myocardial revascularization, left ventricular outflow tract reconstruction, and relief of pulmonary stenosis.

Background At our institution, the arterial switch operation for transposition of the great arteries has transitioned from the Gore-Tex patch (W.L. Gore & Associates, Flagstaff, AZ) for pulmonary artery reconstruction to redundant pantaloon pericardial patch (RPPP). The (U-shaped) coronary artery button was used for coronary reimplantation. This study investigates overall mortality and factors for neopulmonary artery, neoaortic, and coronary artery surgical reintervention. Methods We performed a retrospective chart review of all patients who underwent arterial switch between 1983 and 2007. Our surgical database, operative reports, and cardiology clinic charts were reviewed. Time to event was plotted as Kaplan-Meier curves. Predictors of time-to-event were examined using Cox proportional hazard modeling. Results A total of 258 patients underwent arterial switch during the study. Mortality declined from 15% (era I: 1983 to 1990) to 11% (era II: 1991 to 1998) to 7% (era III: 1999 to 2007). Era III had a significantly later time to death compared with era I (hazard ratio [HR] 0.62, p = 0.04). The RPPP had a lower neopulmonary artery reintervention rate compared with Gore-Tex; 9 of 225 (4%) versus 3 of 21 (14%), p = 0.008. Complex anatomy increased risk for neopulmonary reintervention (HR 3.3, p = 0.03). Surgical reintervention rate for coronary arteries was 2%. Complex coronary anatomy (HR 17.9, p = 0.01) predicted coronary reintervention. Predictors of neoaortic reintervention were prior pulmonary artery band (HR 4.3, p = 0.03), complex anatomy (HR 3.5, p = 0.01), and coronary artery anatomy (HR 3.5, p = 0.04). Conclusions Arterial switch operation mortality has decreased. Conversion to RPPP reduced neopulmonary artery reintervention. The (U-shaped) coronary artery button technique is associated with low coronary reintervention rates. Complex coronary anatomy increases coronary and aortic reintervention. Prior pulmonary artery banding and complex anatomy increase aortic reintervention.

Background Anomalous aortic origin of the coronary artery (AAOCA) has been associated with coronary ischemia, myocardial infarction, and sudden death. Advances in echocardiography and computed tomography have identified at-risk patients. Treatment options include unroofing strategies in symptomatic and asymptomatic patients. We review our experience for efficacy and safety. Methods Between 1998 and 2008, we performed coronary unroofing in 22 patients with AAOCA without aortic commissural detachment. Of 7 patients with "left from right" AAOCA, 4 had chest pain only, 1 had syncope, 1 had myocardial infarction, and 1 was asymptomatic. Of 15 patients with "right from left" AAOCA, 11 had chest pain only, 4 had syncope, and none were without symptoms. Median age was 15 years (range, 5 to 54). Eight patients had concomitant procedures, most commonly patent foramen ovale closure. Results There were no deaths or complications. Mean cross-clamp time was 53 minutes. Mean length of stay was 4 days. Postoperative evaluation included echocardiography, computed tomography angiogram, stress thallium, stress echocardiography, and exercise stress test. In all patients, the repaired coronary was patent, with demonstrated flow. Mean follow-up was 17 months (range, 1 to 63). Conclusions Anomalous aortic origin of the coronary artery is emerging as an identifiable disease entity associated with symptoms or sudden death. We conclude that coronary unroofing is a safe and effective therapy for symptomatic patients. Surgery for asymptomatic patients has been more controversial, with growing advocacy for patients with "left from right" as opposed to "right from left" origins. Prospective studies will be required to answer these questions.

This article was prepared to summarize the points made in a debate that the first author (C.L.B.) had with Dr. Richard Jonas at the American Association for Thoracic Surgery 90th Annual Meeting. The topic of the debate was the optimal surgical approach for functional single-ventricle patients: extracardiac versus intra-atrial lateral tunnel Fontan. My role was to take the viewpoint that the extracardiac Fontan is better. This review summarizes our results at Children's Memorial Hospital (Chicago, IL) with 180 patients undergoing a primary Fontan procedure and 126 patients undergoing an extracardiac Fontan as part of a Fontan conversion. The world literature was reviewed on outcomes following the Fontan procedure, focusing on six main areas supporting the superiority of the extracardiac Fontan: hemodynamics, arrhythmias, applicability to complex anatomy, use of cardiopulmonary bypass, complications of fenestration and thromboembolism, and operative mortality. Based on this review, it is our conclusion that the extracardiac Fontan is the procedure of choice for patients with a functional single ventricle based on a very low operative mortality, a lower incidence of early and late arrhythmias, improved hemodynamics, fewer postoperative complications, and applicability to a wide variety of complex cardiac anatomy.

Background The Society of Thoracic Surgeons Database was queried to ascertain current trends in management of tetralogy of Fallot (TOF) and to determine the prevalence of various surgical techniques. Methods The study population (n = 3059 operations) was all index operations in 2002–2007, age 0–18 years with Primary Diagnosis of TOF, and Primary Procedure TOF repair or palliation. Patients with Pulmonary Atresia, Absent Pulmonary Valve, and Atrioventricular Canal were excluded. Results 294 patients had initial palliation, including 178 neonates. 2534 patients had repair of TOF as the initial operation (primary repair), including 154 neonates. 217 patients had repair of TOF after prior palliation. Of patients who had primary repair (n = 2534), 975 had repair at 3 to 6 months, 614 at 6 months to 1 year, 492 at 1 to 3 months, and 154 at 0 to 30 days. Of patients who had repair following prior palliation (n = 217), 65 had repair in the first 6 months of life, 111 at 6 months to 1 year, and only 41 (18.9%) at more than 1 year of age. Type of repair: Of 2534 primary repairs, 581 (23%) had no ventriculotomy, 571 (23%) had nontransanular patch, 1329 (52%) had transanular patch, and 53 (2%) had right ventricle to pulmonary artery conduits. Of repairs after prior palliation (n = 217), 20 (9%) had no ventriculotomy, 30 (14%) had nontransanular patch, 144 (66%) had transanular patch, and 24 (11%) had conduits. Discharge mortality (95% confidence interval; CI) was 22 of 294 (7.5%; CI: 4.7%–11.1%) for initial palliation, 33 of 2534 (1.3%; CI: 0.9%–1.8%) for primary repair, and 2 of 217 (0.9%; CI: 0.1%–3.3%) for secondary repair. For neonates, discharge mortality was 11 of 178 (6.2%; CI: 3.1%–10.8%) for palliation and 12 of 154 (7.8%; CI: 4.1%–13.2%) for primary repair. Conclusions Primary repair in the first year of life is the most prevalent strategy. Despite contemporary awareness of the late consequences of pulmonary insufficiency, ventriculotomy with transanular patch remains the most prevalent technique, both for primary repair and for repair following palliation.

Hypoplastic left heart syndrome may coexist with noncardiac congenital defects or genetic syndromes. We explored the impact of such lesions on outcomes after staged univentricular palliation.Society of Thoracic Surgeons database 2002 to 2006: Children diagnosed with hypoplastic left heart syndrome who underwent stage 1 Norwood (n = 1,236), stage 2 superior cavopulmonary anastamosis (n = 702) or stage 3 Fontan (n = 553) procedures were studied. In-hospital mortality, postoperative complications, and length of stay were compared at each stage between those with and without noncardiac-genetic defects. Congenital Heart Surgeons' Society database 1994 to 2001: All 703 infants enrolled in the Congenital Heart Surgeons' Society critical left ventricular outflow tract obstruction study who underwent primary stage 1 palliation were reviewed. The impact of noncardiac defects-syndromes on survival was explored using multivariable parametric models with bootstrap bagging.Society of Thoracic Surgeons database: Stage 1 in-hospital mortality (26% vs 20%, p = 0.04) and mean postoperative length of stay (42 versus 31 days, p0.0001) were greater, and postoperative complications significantly more prevalent in infants with noncardiac-genetic defects. Congenital Heart Surgeons' Society database: Noncardiac-genetic defects were present in 55 (8%). Early hazard for death after Norwood was significantly worse in infants with noncardiac defects-syndromes (p = 0.008). Chromosomal defects (n = 14) were highly unfavorable: the early risk of death was doubled (10-year survival 25 +/- 9% vs 54 +/- 2%, p = 0.005). Turner syndrome accounted for the majority of chromosomal defects in this population (11 of 14, 79%). Mode of death was rarely attributable to the noncardiac-genetic defect.Survival in hypoplastic left heart syndrome is strongly influenced by the presence of noncardiac abnormalities. Strategies to improve mortality in infants with noncardiac abnormalities should be explored. Presence of chromosomal defects, especially Turner syndrome, should enter decision-management options for parents and physicians.

Objective Analysis of congenital heart surgery results requires a reliable method of estimating the risk of adverse outcomes. Two major systems in current use are based on projections of risk or complexity that were predominantly subjectively derived. Our goal was to create an objective, empirically based index that can be used to identify the statistically estimated risk of in-hospital mortality by procedure and to group procedures into risk categories. Methods Mortality risk was estimated for 148 types of operative procedures using data from 77,294 operations entered into the European Association for Cardiothoracic Surgery (EACTS) Congenital Heart Surgery Database (33,360 operations) and the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (43,934 patients) between 2002 and 2007. Procedure-specific mortality rate estimates were calculated using a Bayesian model that adjusted for small denominators. Each procedure was assigned a numeric score (the STS–EACTS Congenital Heart Surgery Mortality Score [2009]) ranging from 0.1 to 5.0 based on the estimated mortality rate. Procedures were also sorted by increasing risk and grouped into 5 categories (the STS–EACTS Congenital Heart Surgery Mortality Categories [2009]) that were chosen to be optimal with respect to minimizing within-category variation and maximizing between-category variation. Model performance was subsequently assessed in an independent validation sample (n = 27,700) and compared with 2 existing methods: Risk Adjustment for Congenital Heart Surgery (RACHS-1) categories and Aristotle Basis Complexity scores. Results Estimated mortality rates ranged across procedure types from 0.3% (atrial septal defect repair with patch) to 29.8% (truncus plus interrupted aortic arch repair). The proposed STS–EACTS score and STS–EACTS categories demonstrated good discrimination for predicting mortality in the validation sample (C-index = 0.784 and 0.773, respectively). For procedures with more than 40 occurrences, the Pearson correlation coefficient between a procedure's STS–EACTS score and its actual mortality rate in the validation sample was 0.80. In the subset of procedures for which RACHS-1 and Aristotle Basic Complexity scores are defined, discrimination was highest for the STS–EACTS score (C-index = 0.787), followed by STS–EACTS categories (C-index = 0.778), RACHS-1 categories (C-index = 0.745), and Aristotle Basic Complexity scores (C-index = 0.687). When patient covariates were added to each model, the C-index improved: STS–EACTS score (C-index = 0.816), STS–EACTS categories (C-index = 0.812), RACHS-1 categories (C-index = 0.802), and Aristotle Basic Complexity scores (C-index = 0.795). Conclusion The proposed risk scores and categories have a high degree of discrimination for predicting mortality and represent an improvement over existing consensus-based methods. Risk models incorporating these measures may be used to compare mortality outcomes across institutions with differing case mixes.

Background Infants with congenital tracheal stenosis may also have unilateral lung agenesis or severe lung hypoplasia. The purpose of this review is to evaluate our results with these patients and compare their presentations and outcomes to those of tracheal stenosis patients with two lungs. Methods Our database was queried for patients undergoing tracheal stenosis repair since 1982. Patients were divided into two groups based on pulmonary anatomy of single lung (SL = unilateral lung agenesis or severe hypoplasia) or two lungs (BL = bilateral lungs) and analyzed to compare presentation and outcomes. Results From 1982 to 2008, 71 patients had tracheal stenosis repair. Bilateral lungs were present in 60 patients; 9 patients had an absent (4) or severely hypoplastic (5) right lung, and 2 patients had an absent left lung (SL = 11). Age at repair was similar between groups; median age 0.42 years in the SL group (mean 0.80 ± 1.0 years) versus 0.37 years in the BL group (mean 0.91 ± 2.1 years, p = not significant [ns]). In the SL group 8 of 11 (73%) were intubated preoperatively versus 15 of 60 (25%) in the BL group ( p = 0.004). In the SL group 4 of 11 (36%) patients had pulmonary artery sling versus 20 of 60 (33%) of BL patients ( p = ns). In the SL group 2 of 11 (18%) versus 14 of 60 (23%) in the BL group had intracardiac anomalies requiring simultaneous repair ( p = ns). Procedures included pericardial tracheoplasty (2 vs 26), tracheal autograft (4 vs 16), slide tracheoplasty (3 vs 8), and tracheal resection (2 vs 10). Overall mortality (operative and late) was 2 of 11 (18%) SL versus 10 of 60 (17%) BL ( p = ns). Median postoperative length of stay was 43 days SL (mean 48.6 ± 40) versus 30 days BL (mean 52.2 ± 65) ( p = ns). The incidence of postoperative tracheostomy (SL group) was 0 of 3 for slide tracheoplasty and 5 of 8 for the other techniques ( p = 0.12). Conclusions Despite the increased severity of pathology and increased critical presentation of tracheal stenosis patients with unilateral lung agenesis or severe hypoplasia, outcome measures of mortality and length of stay were similar to patients with two lungs. The incidence of associated pulmonary artery sling (1 of 3) and intracardiac anomalies (1 of 4) was similar. Unilateral lung agenesis or severe hypoplasia should not preclude operative repair of tracheal stenosis. Slide tracheoplasty is our current procedure of choice for these infants.

The purpose of this study was to compare the modified single-patch technique to the two-patch technique for infants with complete atrioventricular canal (CAVC) defects.Between January 2000 and June 2006, 55 infants underwent CAVC repair. Twenty-six patients had a modified single-patch technique; 29 patients had a two-patch technique. Trisomy 21 was present in 23 of 26 and 26 of 29 patients (p = not significant [ns]). Mean age was 4.4 +/- 1.3 months (single-patch) versus 5.5 +/- 1.9 months (two-patch, p0.02). Mean weight was 4.74 +/- 0.92 versus 5.28 +/- 1.67 kilograms (p = ns).There was one death in the modified single-patch group (postoperative day 130, liver failure) and no deaths in the two-patch group. Cross-clamp times and cardiopulmonary bypass times were shorter in the modified single-patch group (97.3 +/- 19.9 vs 123.3 +/- 28.2 minutes, p0.0003; 128 +/- 25 vs 157 +/- 37, p0.03). Rastelli classification was type A (18 vs 14), B (1 vs 0), and C (7 vs 15). Mean size of the ventricular septal defect as assessed by transesophageal echocardiogram was 9 +/- 2 mm, (single-patch) versus 10 +/- 3 mm (two-patch) (p = ns). Median postoperative length of stay did not differ (10 vs 8 days). There was no difference in the degree of postoperative left or right AV valve insufficiency as assessed by serial echocardiography. One patient (4%) required reoperation for mitral insufficiency in the modified single-patch versus three patients in the two-patch group (10%, p = ns). There were no patients with third degree atrioventricular block or that required reoperation for residual VSD in the modified single-patch group. There was one patient with third-degree AV block that required a pacemaker and one patient who had reoperation for a residual ventricular septal defect in the two-patch group (p = ns). No patient in either group required reoperation for left ventricular outflow tract obstruction.The modified single-patch technique produced results comparable with the two-patch technique in younger patients with similarly sized ventricular septal defects. Furthermore, the modified single-patch technique was performed with significantly shorter cross-clamp and cardiopulmonary bypass times.

ObjectiveAprotinin, a serine protease inhibitor, decreases transfusion requirements and inflammatory response after cardiopulmonary bypass. This study was done to determine whether aprotinin is associated with adverse outcomes, particularly mortality and acute kidney failure, in pediatric patients (

Background The evolving operative strategy and course of 111 consecutive Fontan conversions with arrhythmia surgery and pacemaker therapy were reviewed to identify risk factors for poor outcome. Methods Since 1994, 111 patients (mean age 22.5 ± 7.9 years) underwent Fontan conversion with arrhythmia surgery. The series was divided into three time periods: (1) 1994 to 1996 (initial isthmus ablation, n=9, group I); (2) 1996 to 2003 (early modified right atrial maze and Cox-maze III, n=51, group II); and (3) 2003 to 2006 (recent modifications of the modified right atrial maze and left atrial Cox-maze III for both atrial fibrillation and left atrial reentry tachycardia, n=51, group III). Results There were one early (0.9%) and six late deaths (5.4%); six patients required cardiac transplantation (5.4%). Two late deaths occurred after transplantation. Renal failure requiring dialysis occurred in four patients (3.6%). Mean hospital stay was 13.7 ± 12.1 days. Mean cross-clamp time was 70.8 ± 41.6 minutes. Four risk factors for death or transplantation were identified: presence of a right or ambiguous ventricle, preoperative protein-losing enteropathy, preoperative moderate-to-severe atrioventricular valve regurgitation, and long (>239 minutes) cardiopulmonary bypass time. In intergroup comparisons (groups I and II versus group III), three trends were noted: increased incidence of concomitant surgical repairs ( p = 0.03), older patients ( p = 0.01), and increased incidence of left atrial reentry tachycardia and atrial fibrillation ( p = 0.04). Late recurrence of atrial tachycardia ensued in 15 of 111 (13.5%); 8 of 51 in group II (15.7%) and 4 of 51 in group III (7.8) ( p = 0.3). Conclusions Fontan conversion with arrhythmia surgery is safe and efficacious. Based on improved results and evolving surgical techniques, selection criteria can be more clearly defined.

Paraplegia is a devastating complication of coarctation of the aorta (COA) repair. Since 1990 we have used left atrium-to-descending aorta cardiopulmonary bypass (CPB) for COA repair in patients with inadequate collaterals. We reviewed the results with that strategy and compared this CPB group with COA repairs in which CPB was not used to see whether there was any increase in morbidity or delay in recovery.From 1990 to 2006, 11 patients with COA were identified to have inadequate collaterals based on preoperative examination and intraoperative arterial monitoring and test clamp. Left thoracotomy with left atrium-to-descending aorta CPB was used in all. Age ranged from 4.2 to 17.4 years (mean, 8.7 +/- 4.6 years). Two were reoperations for recurrent COA, 3 patients had four prior transcatheter balloon dilatations. One patient had aberrant origin of the right subclavian artery. Operative techniques included resection with extended end-to-end anastomosis (n = 6), interposition graft (n = 4), and patch repair (n = 1). During the same period 71 patients older than 1 year of age had COA repair without CPB. Age ranged from 1.1 to 46.1 years (mean, 7.6 +/- 7.1 years; p = 0.6).Preoperative imaging of CPB patients demonstrated absence of collaterals (n = 7), possible collaterals (n = 2), small collaterals (n = 1), and anomalous origin of the right subclavian artery (n = 1). Preoperative arm leg gradient in CPB patients was 36.0 +/- 9.0 mm Hg versus 49.9 +/- 15 mm Hg in non-CPB patients (p0.01). Mean distal femoral artery pressure with aortic test clamp was 34.3 +/- 4.8 mm Hg in CPB patients versus 49.8 +/- 12.4 mm Hg in non-CPB patients (p0.01). Mean CPB flow was 53% +/- 7.3% of calculated total flow. Cardiopulmonary bypass time ranged from 17 to 46 minutes (mean, 27.5 +/- 9.7 minutes). Aortic clamp time in CPB patients ranged from 15 to 33 minutes (mean, 21.6 +/- 6.3 minutes). In the non-CPB group aortic clamp time ranged from 10 to 50 minutes (mean, 23.4 +/- 7.5 minutes; p = 0.5). In the CPB group length of stay ranged from 3 to 7 days (mean, 4.9 +/- 1.3 days), and in the non-CPB group length of stay ranged from 3 to 12 days (mean, 4.7 +/- 1.4 days; p = 0.5). No patient had a neurologic complication. There were no other major complications in the CPB group (eg, bleeding, recurrent laryngeal nerve injury, re-COA).Preoperative imaging and a lower arm-to-leg gradient in this series of COA patients suggested inadequate collateral circulation with the potential need for CPB. A femoral artery pressure of less than 45 mm Hg during test clamp was used as an indication for partial CPB. The use of left atrium-to-descending aorta CPB with just over 50% calculated total flow protected the spinal cord in a safe and expeditious fashion. Use of left heart bypass did not affect morbidity or recovery time as compared with COA repair in non-CPB patients.

The most concrete and universal outcome measure used in databases, whether governmental, professional society, research, or third-party payer, is operative mortality. To assure congruous data entry by multiple users of The Society of Thoracic Surgeons and the European Association for Cardiothoracic Surgery congenital heart surgery databases, operative mortality must be clearly defined. Traditionally, operative mortality has been defined as any death, regardless of cause, occurring (1) within 30 days after surgery in or out of the hospital, and (2) after 30 days during the same hospitalization subsequent to the operation. Differing hospital practices result in problems in use of the latter part of the definition (eg, the pediatric hospital that provides longer-term care will have higher mortality rates than one which transfers patients to another institution for such care). In addition, because of the significant number of pediatric multiple operation hospitalizations, issues of assignment of mortality to a specific operation within the hospitalization, calculation of operative mortality rates (operation based vs patient admission based), and discharge other than to home must be addressed and defined. We propose refinements to the definition of operative mortality which specifically meet the needs of our professional societies' multi-institutional registry databases, and at the same time are relevant and appropriate with respect to the goals and purposes of administrative databases, government agencies, and the general public.

Our surgical strategy for repair of tetralogy of Fallot has focused on preserving the pulmonary valve. The purpose of this review was to identify pulmonary valve characteristics that mark the limits of this strategy.From 1997 through 2004, 102 consecutive patients underwent repair of tetralogy of Fallot at a median age of 5.9 months. Twenty-five patients had a prior shunt. Eighty-two patients (80%) had pulmonary valve-sparing procedures, predominantly through a transatrial and transpulmonary approach (n = 52). Twenty patients had a transannular patch (20%). Intraoperative measurements included the pulmonary valve annulus size and the postoperative pressure ratio between the right and left ventricles.Eighty of 85 (94%) patients with z-score greater than -4 had a pulmonary valve-sparing procedure compared with 2 of 17 patients (12%) with pulmonary valve annulus z-scores less than -4 (p0.0001). All patients with a tricuspid pulmonary valve (n = 26) had a pulmonary valve-sparing procedure compared with 56 of 76 (74%) patients with a bicuspid pulmonary valve (p = 0.0016). Five patients with initial pulmonary valve-sparing operations required reoperation for residual stenoses; 4 pulmonary valve-sparing right ventricular outflow tract resections and 1 transannular patch. The only death occurred after reoperation elsewhere. Three of 9 patients (33%) who had a postoperative pressure ratio between the right and left ventricles greater than 0.7 after their initial pulmonary valve-sparing procedure required reoperation compared with 2 of 73 with postoperative pressure ratio between the right and left ventricles less than 0.7 (3%; p = 0.008). Fifteen of 25 patients (60%) with prior shunts had pulmonary valve-sparing procedures.A pulmonary valve-sparing approach to the repair of tetralogy of Fallot was applied successfully in 80% of patients. Significant markers for success were a measured pulmonary annulus z-score of -4 or larger, a tricuspid pulmonary valve, and a postoperative pressure ratio between the right and left ventricles less than 0.7.

Objectives We sought to determine the effect of cardiopulmonary bypass and surgical intervention on the natriuretic hormone system in children and to assess whether such changes are associated with morbidity. Methods At 6 perioperative time points in 25 patients, plasma levels of atrial natriuretic peptide, brain natriuretic peptide, and guanosine 3′, 5′-monophosphate were measured, and the biologic activity of the natriuretic hormone system was quantified. Relationships were sought between changes in brain natriuretic peptide levels, biologic activity, and a number of morbidity indicators. Results There was a significant change in atrial natriuretic peptide levels ( P = .037), brain natriuretic peptide levels ( P = .001), and biologic activity of the natriuretic hormone system ( P = .009) over the first 4 time points in the study. Atrial natriuretic peptide levels transiently decreased from baseline to 12 hours after surgical intervention. Compared with baseline values, brain natriuretic peptide levels were increased at 12 hours after surgical intervention and on postoperative day 1. The increase in brain natriuretic peptide levels from baseline to 12 hours after surgical intervention was associated with cardiopulmonary bypass time ( r s = 0.4, P = .047). The biologic activity transiently decreased from baseline to intensive care unit admission but was not associated with any morbidity indicators. Conclusions Increased postoperative brain natriuretic peptide levels are associated with longer bypass times. The biologic activity of the natriuretic hormone system is transiently impaired. Larger studies should investigate brain natriuretic peptide as a predictor of postoperative morbidity and the potential for natriuretic hormone infusions to improve postoperative hemodynamics and urine output.

Background Chronic inflammation and angiogenesis have been implicated in the pathogenesis of both cardiac allograft vasculopathy (CAV) and age-related vasculopathy. Because concurrent atherosclerosis does not complicate assessment of CAV in children, we sought to characterize the spectrum of coronary lesions in this population and determine whether inflammatory infiltrates and angiogenesis correlate with severity of CAV. Methods In 18 pediatric heart specimens CAV was graded 1 to 4 (none to severe). Each case was assigned to either: Group I, no inflammation; Group II, perivascular inflammation; or Group III, perivascular and neointimal inflammation. Inflammatory infiltrates were immunophenotyped using anti-CD3, anti-CD20 and HAM 56. Angiogenesis was assessed by determining microvascular density (MVD) in 5 high-power fields (HPFs) per section. Results CAV was evident in 94% of cases, and inflammation in 61%. Cases with neointimal inflammation had significantly more severe CAV compared with cases without inflammation (2.7 ± 0.16 vs 1.9 ± 0.2, p = 0.002). MVD was significantly higher in both inflammation groups (Groups II and III) compared with Group I (4.1 ± 0.5 per HPF and 5.9 ± 0.5 vs 3.1 ± 0.7, p = 0.018 and p = 0.002) and correlated with the degree of CAV ( p = 0.007). The perivascular infiltrates (Group II, n = 5) contained lymphocytes, macrophages and plasma cells, and 67% of neointimal infiltrates (Group III, n = 6) also contained eosinophils. Conclusions CAV in children is more common than previously reported. Our data indicate that CAV is often associated with inflammation and that adventitial angiogenesis correlated with the severity of CAV.

Objective Vascular remodeling, often accelerated after cardiovascular procedures, may result in stenosis or aneurysm formation. The bone-associated protein osteopontin has been suggested to be involved in vascular remodeling, yet the effect of locally applied osteopontin in an acute vascular injury model of aortic calcification has not been examined. Methods Vascular healing of rabbit thoracic aortas treated locally with recombinant osteopontin (dose: 1 μg; n = 16) or albumin (control, n = 16) after acute injury created by end-to-end anastomosis was evaluated. Matrix metalloproteinase-2 level was quantified by gelatin zymography. Proliferation of smooth muscle cells was detected by immunostaining for proliferative cell nuclear antigen. Results Osteopontin-treated aortas showed significantly diminished vascular remodeling with less calcification ( P = .001) and reduced anastomotic luminal stenosis (4% vs 28%, P = .002) compared with controls 2 months postsurgery. Moreover, osteopontin-treated aortas revealed a thickened adventitia with increased fibrosis ( P = .006). Matrix metalloproteinase-2 level was up-regulated 2-fold with osteopontin treatment compared with control at 1 week, returning to baseline by 1 month. Staining for proliferation cell nuclear antigen disclosed an increase in proliferation cell nuclear antigen-positive smooth muscle cells in the media of osteopontin-treated aortas at 1 week, normalizing by 1 month. Conclusions These data suggest a beneficial effect of locally applied osteopontin after acute injury possibly by altering matrix metalloproteinase-2 activity and smooth muscle cell proliferation. Brief application of osteopontin may effectively enhance vascular healing by reducing calcification and thus maintaining luminal integrity. The role of the osteopontin-related increase in adventitial fibrosis on vascular healing has to be explored.

Our profession is based on ethical behavior, which extends far beyond the rule of law. In this Festschrift for Hiram C. Polk, the ethical forces that shape a career in surgery-including their impact on the timely issue of medical errors, truth-telling, and disclosure-are discussed. The philosophical underpinnings (as addressed by Kant and Aristotle, among others) that may guide the surgeon are considered and discussed with particular emphasis on their relevance to the practice of surgery. The roles that courage, self-evaluation, and teaching play in our practices are reviewed as are new initiatives for improved outcomes for our patients-our ultimate professional goal.

Stellar outcomes have been achieved for patent ductus arteriosus (PDA), atrial septal defect (ASD), and ventricular septal defect (VSD) repairs by using the traditional surgical methods. Over the last decade, minimally invasive procedures have been introduced that promise excellent results with an improved cosmetic appearance, shorter rehabilitation period, less pain, and decreased hospital cost. We reviewed various minimally invasive procedures that are used in PDA, ASD, and VSD to assess their safety and efficacy. These techniques use limited approaches to the heart (partial sternotomies, transxiphoid, anterolateral thoracotomy, and mini-thoracotomy) that reduce the surgeon℉s access and control of the cardiac structures. Cannulation sites for the establishment of cardiopulmonary bypass have been altered. Minimally invasive therapies for congenital heart surgery cannot be adopted until evidence-based data has proven them to be equal or better than the traditional procedure.

Background Transcatheter radiofrequency ablation to treat supraventricular and ventricular arrhythmias has supplanted routine surgical ablative therapy and redefined its role. A small population of arrhythmia patients now requires surgical ablation: those who have failed catheter ablation, patients with concomitant congenital heart disease in association with arrhythmias, those with atrial fibrillation and very young patients for whom transcatheter techniques are prohibitive because of small size, cyanosis or distorted anatomy. Methods From July 1992 through August 2003, 133 patients underwent arrhythmia surgery at Children's Memorial Hospital, 50% (67/133) in association with Fontan conversion (FC), 22% (28/133) with concomitant initial Fontan (IF) procedure and 28% (38/133) for various arrhythmias (MISC) in patients with (36/38, 95%) or without (2/38, 5%) associated structural heart disease. Mean age at surgery in the FC group was 20±7.6 years (median 19 years), and in the IF group and the MISC group, mean ages were 8.1±8.9 (median 4.2) years and 16.4±10.9 (median 11.3) years, respectively. Results There were three operative (3/133, 2.6%; 1 FC, 2 MISC) and three late deaths (2 FC, 1 MISC). Four patients in the FC group had progressive ventricular failure and underwent successful cardiac transplantation. Follow-up data are available for non-transplant, surviving patients and reveal 11 incidences of persistent arrhythmia recurrence and 2 new-onset arrhythmias. Five of the 11 recurrences occurred early in our series of FC patients, when isthmus block interruption of arrhythmia foci was performed. Four additional recurrences occurred later in the FC series, two post-maze and two post-Cox-maze III. In the MISC group, there were two recurrences. Atrial reentry tachycardia (ART) recurred in a patient with no structural heart disease and accessory connection-mediated tachycardia recurred in a child who underwent concomitant initial Fontan. Two patients had ventricular tachycardia inducible at postoperative studies (2/7, 29%), but no clinical recurrence. Two new-onset tachycardias occurred, one child developed ART post-surgical ablation of accessory connections and one patient with inducible ventricular tachycardia developed ART 5 years postoperatively. Conclusion Variations in atrial and ventricular anatomy that may limit the catheter approach can be addressed surgically. Patient size or anatomic complexity should not be limiting factors in the combined surgical arrhythmia approach. Incorporation of arrhythmia therapy into planned surgical revision should be considered.

Background The development of transplant coronary artery disease is associated with neovascularization in the thickened neointima. We previously reported that captopril inhibits neointimal proliferation in a rat allograft model. We postulated that angiogenic inducers are upregulated post-transplantation and captopril ameliorates transplant coronary artery disease by suppressing the angiogenic activity of coronaries. Methods Animals received no treatment or captopril (50 mg/kg/day). Allograft hearts were analyzed at post-transplantation Days 0, 14, and 21 and angiogenic inducer, plasma platelet-activating factor, determined. The conditioned media from coronaries and myocardium were tested for vascular endothelial growth factor, thrombospondin-1 and angiogenic activity using an endothelial migration assay and rat corneal neovascularization assay. Results The captopril-treated group had reduced plasma platelet-activating factor and coronary media revealed earlier upregulation of thrombospondin-1 secretion, diminished vascular endothelial growth factor and no angiogenic activity. At Day 0, the coronary and myocardial conditioned medium had inhibitory activity due to thrombospondin-1, and circulating levels of platelet-activating factor were negligible. By 21 days post-transplantation, plasma platelet-activating factor was elevated and the conditioned medium from untreated coronaries had significantly higher angiogenic activity due to increased vascular endothelial growth factor whereas the myocardium remained non-angiogenic. Conclusions After transplantation, coronary vessels switch to an angiogenic phenotype and vascular endothelial growth factor contributes to the high angiogenic activity, possibly exacerbated by high circulating levels of platelet-activating factor. The ability of captopril to modulate angiogenic mediators and maintain the allograft coronary to its normal anti-angiogenic phenotype may be one mechanism by which it suppresses transplant coronary artery disease.

The aim of the Aristotle project was to develop a new method of evaluation of quality of care in congenital heart surgery based on the complexity of the surgical procedures. Involving a panel of expert surgeons, the project started in 1999 and included 50 pediatric surgeons from 23 countries representing International Scientific Societies. The complexity was based on the procedures as defined by the Society of Thoracic Surgeons (STS)/European Association for Cadiothoracic Surgery (EACTS) International Nomenclature and was undertaken in two steps: The first step was establishing the Basic Score, which adjusts only the complexity of the procedures and is based on three factors: the potential for mortality, the potential for morbidity, and the anticipated technical difficulty. The second step was the development of the Comprehensive Score, which further adjusts the complexity according to the specific patient characteristics. The Aristotle score allows precise scoring of the complexity for 145 congenital heart surgery procedures. One interesting concept coming out of this study is that complexity is a constant and precise value for a given patient regardless of the center where he is operated. The Aristotle method allows proposing the following equation of quality of care: Complexity FN Outcome = Performance. The Aristotle score, electronically available, was introduced in the EACTS and STS databases. A validation process, designed to evaluate its predictive value, is being developed.

When biventricular repair of congenitally corrected transposition of the great arteries (ccTGA) is possible, complete anatomic (left ventricle to the aorta, right ventricle to the pulmonary artery, and associated atrial baffle operation) or physiologic (left ventricle to pulmonary artery, right ventricle to aorta) connections are complex and associated with significant long-term problems such as arrhythmias, subaortic obstruction, valvar dysfunction, ventricular-to-pulmonary artery conduit changes, and deteriorating ventricular function. Recent advances have supported physiologic, anatomic, and single-ventricle repairs. The purpose of this article is to review the natural history of ccTGA, compare and contrast the physiologic and anatomic repairs, and reintroduce a new concept of physiologic repair for patients with ccTGA, ventricular septal defect, and pulmonary stenosis using left ventricular unloading techniques via a bidirectional shunt.

Surgical arrhythmia therapy may be performed for patients failing the catheter ablation approach or incorporated into repair of complex congenital heart disease. Variations in atrial and ventricular anatomy that may limit the catheter approach can be directly addressed surgically assuring lesion depth and continuity of anatomic lines of block. Between July 1992 and August 2002, we performed arrhythmia surgery on 34 patients for refractory atrial (n = 29) or ventricular (n = 5) arrhythmias. Not included in this series are patients who had arrhythmia surgery during Fontan conversion. The majority of patients had various forms of complex congenital heart disease; two had structurally normal hearts. Median age at surgery was 13.0 years (range, 7 days to 45 years). Five patients were infants (mean age, 25 days). Twenty-two patients (65%) had an average of 2.8 previous cardiac procedures; all required resternotomy. Operative mortality was 5.9% (2 of 34 patients) because of low cardiac output in one patient following Mustard takedown and arterial switch operation and in one neonate with Ebstein's anomaly and pulmonary atresia. Ablative surgery for supraventricular tachycardia (atrial re-entry, automatic atrial, atrioventricular nodal re-entry, and atrial fibrillation) had a 93% success rate (25 of 27 patients). Clinical tachycardia recurred in two of 27 surviving patients (7%) with atrial arrhythmia, one after an arrhythmia-free interval of several years. Ventricular tachycardia was inducible postoperatively in two of three patients with ventricular arrhythmias and congenital heart disease. Patient size or anatomic complexity should not be limiting factors in the combined surgical arrhythmia approach. Because older patients undergoing surgical revision of prior surgical repairs of congenital heart disease are at increased risk for the later development of atrial arrhythmias, incorporation of arrhythmia therapy into any planned surgical revision should be routinely considered.

Late postoperative reentry tachycardia is a major source of morbidity and mortality after intracardiac repair of congenital heart disease. The incidence is greatest after the Fontan operation, but is significant after repair of other defects. Although medical, pacing, and ablation therapy each play a role in treating these tachyarrhythmias, none are completely effective. In addition, many of these patients require additional surgical procedures to improve hemodynamic abnormalities. We have combined intracardiac electrophysiologic studies with detailed mapping and a direct surgical approach using cryoablation to divide reentry circuits. Permanent atrial pacing is an important part of each surgical procedure. This extensive right atrial maze procedure has been highly effective in eliminating atrial reentry tachycardia.

Postoperative Fontan patients can develop hemodynamic abnormalities and refractory atrial arrhythmias resulting in significant morbidity and mortality. We present our experience with total cavopulmonary artery conversion and arrhythmia surgery. Between 1994 and 2001, 41 patients underwent total cavopulmonary artery conversion and arrhythmia surgery. Significant hemodynamic lesions were repaired concomitantly: aortic aneurysm (n=1), atrioventricular valve insufficiency (n=8), and pulmonary artery stenosis (n=9). Thirty-five patients were in New York Heart Association class III or IV. Mean age at original Fontan was 7.5+/-6.5 years, at Fontan conversion, 18.7+/-9.0 years. Arrhythmia surgery for atrial re-entry tachycardia evolved from isthmus cryoablation (n=10) to right-sided maze (n=17). Maze-Cox III was used for 14 patients with atrial fibrillation. Atrial (n=34) and dual chamber (n=5) pacemakers were placed. Mortality and reoperation for bleeding rates are 0%. Chest tubes were removed on postoperative day 9.0+/-6.0. Mean hospital stay was 11.8+/-6.6 days. Three patients required cardiac transplantation at 8 days, 9 months, and 33 months postoperatively. There was one long-term death from acute myocardial infarction 2 years postoperatively. For the entire series, arrhythmia recurrence is 12.2% (5/41). Only 9.8% of patients (4/41) receive chronic antiarrhythmic medications; these patients were among the first eight in the series. Most patients are in New York Heart Association I or II. Bruce protocol in 12 patients showed increased tolerance (P

Background/Purpose: Since 1996 a new procedure—the free tracheal autograft—has been used to repair long segment congenital tracheal stenosis (LSCTS). The purpose of this report is to examine the intermediate-term results of that technique. Methods: Between January 1996 and July 1999, 10 infants underwent repair of LSCTS using a free tracheal autograft. Age ranged from 10 days to 23 months (mean age, 6.6 months). Six infants had a pulmonary artery (PA) sling; 5 had intracardiac anomalies. On cardiopulmonary bypass (CPB) the trachea was incised anteriorly through the area of stenosis. The midportion of the stenotic trachea was excised, and an end-to-end anastomosis was made posteriorly. The excised tracheal segment was trimmed and sutured in place anteriorly as a free autograft. In 5 patients the autograft was not long enough, and the upper portion of the tracheal opening was patched with pericardium. Results: There was 1 death 26 days postoperatively in a child that had simultaneous repair of tetralogy of Fallot and required extracorporeal membrane oxygenation postoperatively for cardiac failure. The other 9 children are alive and well at 2 to 44 months postoperatively (mean follow-up, 24 months). One child had autograft dehiscence and required replacement of the autograft with an aortic homograft. Two children have tracheostomies at 6 and 36 months postoperatively. All children have had serial postoperative bronchoscopic examinations. Most recent bronchoscopies have shown widely patent tracheal lumina in all survivors. Conclusion: Intermediate-term follow-up of children with a free tracheal autograft continues to support our use of this technique as our procedure of choice for infants with LSCTS. J Pediatr Surg 35:813-819. Copyright © 2000 by W.B. Saunders Company.

A randomized, prospective, double-blind study of 29 children was performed to evaluate the hypothesis that dexamethasone administration prior to cardiopulmonary bypass would decrease the inflammatory mediator release and improve the postoperative clinical course.Fifteen children received dexamethasone (1 mg/kg intravenously) and 14 (controls) received saline solution 1 hour prior to CPB. Serial blood analyses for interleukin-6, tumor necrosis factor-alpha, complement component C3a, and absolute neutrophil count were performed. Postoperative variables evaluated included temperature, supplemental fluids, alveolar-arterial oxygen gradient, and days of mechanical ventilation.Dexamethasone caused an eightfold decrease in interleukin-6 levels and a greater than threefold decrease in tumor necrosis factor-alpha levels after CPB (p0.05). Complement component C3a and absolute neutrophil count were not affected by dexamethasone. The mean rectal temperature for the first 24 hours postoperatively was significantly lower in the group given dexamethasone than in the controls (37.2 degrees +/- 0.4 degrees C versus 37.7 degrees +/- 4 degrees C; p = 0.007). Dexamethasone-treated patients required less supplemental fluid during the first 48 hours (22 +/- 28 mL/kg versus 47 +/- 34 mL/kg; p = 0.04). Compared with controls, dexamethasone-treated children had significantly lower alveolar-arterial oxygen gradients during the first 24 hours (144 +/- 108 mm Hg versus 214 +/- 118 mm Hg; p = 0.02) and required less mechanical ventilation (median duration, 3 days versus 5 days; p = 0.02).Dexamethasone administration prior to CPB in children leads to a reduction in the postbypass inflammatory response as assessed by cytokine levels and clinical course.