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The success of the radiofrequency catheter ablation procedure for most types of supraventricular and ventricular tachycardia, particularly in young patients, largely eliminated the role of surgical therapy of arrhythmias. However, there remains a subset of arrhythmia patients in whom the catheter approach has not been successful and types of arrhythmias with high recurrence rates following initially successful catheter ablation procedures where surgery can provide more definitive therapy. In addition, the concepts of ablation therapy can be successfully incorporated into the concomitant repair of complex congenital heart disease, resulting in single-stage therapy for structural and rhythm abnormalities. Prospectively, knowledge of the role of anatomic barriers as substrates for future reentrant arrhythmia circuits provides the opportunity to alter these circuits prophylactically at the time of initial surgical repair of congenital heart disease in an attempt to avoid the late development of tachycardia. This article describes our experience during the past decade with 71 patients undergoing arrhythmia surgery using this approach.

An International Nomenclature for Congenital Heart Surgery was officially adopted at the Annual Meeting of the EACTS in Glasgow, UK on September 6, 1999. This nomenclature was achieved following 1 year's work of the International Nomenclature and Data Base Committee for Congenital Heart Surgery of the Society of Thoracic Surgeons. This international group included members from the STS, AATS, AHA and EACTS and associated surgeons and cardiologists from United States, Canada, Australia and Europe. The Nomenclature includes a minimal data set of 21 items and lists of 150 diagnoses, 200 procedures, 32 complications and 28 extra cardiac anomalies and preoperative risk factors. It will serve as a basis for the Pediatric European Cardiac Surgical Registry (http://www.pediatric. ecsur.org). The outcome of such an International Nomenclature represents an important event for the medical community in charge of treating patients with congenital heart diseases. It will allow scientific exchanges on an international scale and promote multicenter evaluation of congenital heart surgery. Nevertheless, this Nomenclature is only the first step. Further collection of validated data at the Pediatric ECSUR Data Base requires ethical belief, time consumption and financial resources. Comparison of results, according to pathologies, across centers and countries will help define, in the future, official European standards of Quality of Care available for health care organizations, public scrutiny and governmental agencies.

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.

This discussion of arrhythmia terminology attempts to classify rhythm disorders for which surgical therapy may be necessary. 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, for the purpose of establishing a unified reporting system. Efforts were made to include all relevant nomenclature categories, using synonyms where appropriate. Extant surgical ablative procedures, detailed methods of pacemaker insertion, and AICD technology are discussed. A comprehensive database set is presented that 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 will lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

The extant nomenclature for patent ductus arteriosus (PDA), coarctation of the aorta (CoAo), and interrupted aortic arch (IAA) 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. PDA is subclassified by origin, insertion, and patient weight. CoAo is subclassified into isolated CoAo, CoAo with ventricular septal defect, and CoAo with complex intracardiac anomalies. IAA is subclassified into anatomic types A, B, and C based on the location of the interruption. 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 coronary artery 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. The seven major categories of coronary artery anomalies are: anomalous pulmonary artery origins of the coronaries, anomalous aortic origins of the coronaries, congenital atresia of the left main coronary artery, coronary artery fistulas, coronary artery bridging, coronary aneurysms, and coronary stenosis. 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 International Congenital Heart Surgery Nomenclature and Database Project was organized for the purpose of standardizing nomenclature and reporting strategies that would establish the foundations for an international database. Worldwide representatives met for a series of conferences, at which time, issues of nomenclature were discussed and debated. Authors were chosen to review the various congenital heart diagnoses and reflect the mediated debate that followed. Manuscripts were prepared that reviewed the appropriate extant nomenclature, made recommendations for an inclusive rather than an exclusive method of reporting, and determined a hierarchical database scheme that would allow several levels of reporting based on the data input. This manuscript outlines two datasets for an international congenital heart surgery database, a minimum dataset and a comprehensive dataset. The comprehensive dataset includes all the imagined variables, in a hierarchical scheme, which are detailed enough to generate risk stratification analyses. The minimum dataset will include data points that would create an essential dataset, which would be mandatory for data sharing and would lend itself to basic interpretation of trends. The minimum dataset has four drop-down menus for short lists of: (1) noncardiac abnormalities/general preoperative risk factors, (2) diagnoses, (3) procedures, and (4) complications, from which clinicians can choose for entry into the minimum dataset. There was universal agreement for these datasets and short lists by the assembled members of the Society of Thoracic Surgeons-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. The datasets and short lists were also unanimously approved by the Congenital Heart Surgery Committee of The European Association for Cardiothoracic Surgery and adopted by the European Congenital Heart Surgeons Foundation.

Background . Late failure of the systemic right ventricle after atrial baffle procedures in patients with transposition of the great arteries poses significant management problems. We reviewed our experience with staged conversion to arterial switch operation (ASO) in these patients. Methods . Between 1984 and 1999, 11 patients underwent pulmonary artery band (PAB) to prepare the left ventricle for ASO conversion. One additional patient had subpulmonic stenosis and was naturally prepared. Mean age at the initial PAB was 12.2 ± 7 years (range, 1.9 to 23 years). Four patients underwent reoperation to tighten the PAB before ASO. Mean interval from PAB to ASO was 1.3 ± 0.9 years. Results . There was no mortality from PAB. Six patients had ASO conversion and 2 died. Recent surgical modifications at the time of ASO were used to prevent neoaortic valve insufficiency and to cryoablate atrial reentry tachycardia. Four patients developed biventricular failure after PAB and had orthotopic cardiac transplantation (OCT) 14 ± 10 months after PAB. The other 2 patients are still with PAB: 1 is awaiting ASO conversion and the other has insufficient left ventricular hypertrophy necessary for ASO conversion despite two preparatory PABs. Conclusions . A select group of patients with right ventricular failure after atrial baffle operations can undergo staged conversion to ASO with the opportunity for excellent long-term outcome. Surgical modifications at the time of ASO can address the problems of neoaortic insufficiency and persistent atrial arrhythmias. PAB may be a therapeutic endpoint in some patients not responding with adequate left ventricular hypertrophy. Those patients who develop biventricular failure after PAB will require cardiac transplantation.

Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle. Although the presence of aortic-mitral discontinuity and bilateral coni are important descriptors, they should not serve as absolute prerequisites for the diagnosis of DORV. The morphology of DORV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the great artery relationships to each other, the coronary artery anatomy, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), the tricuspid-pulmonary annular distance, and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the left ventricle to the systemic circulation by an intraventricular tunnel repair connecting the VSD to the systemic semilunar valve. This ideal surgical therapy is not always possible due to the presence of confounding anatomical barriers. A multitude of alternative surgical procedures has been devised to accommodate these more complex situations. A framework for the development of the DORV module for a pediatric cardiac surgical database is proposed.

Background: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. Methods: Thirty-one patients (19.9 ± 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. Results: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. Conclusions: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues. (J Thorac Cardiovasc Surg 2000;119:340-6)

A case of cardiac inflammatory pseudotumor with a unique presentation is reported. This especially rare case belongs to a subset of pseudotumor caused by an infectious etiology. Furthermore, it is unique in that the patient experienced unexplained sinus tachycardia, which resolved with resection of the tumor. This report also emphasizes the importance of multiple imaging methods combined with the usefulness of surgery for treatment and diagnosis.

Background . Pediatric coronary artery bypass (PCAB) has been recently employed for expanding indications to treat acquired, congenital, post arterial switch, and other iatrogenic pediatric coronary artery problems. Methods . Between 1987 and 1998, 3 infants and 13 children (n = 16, mean age 6.1 years, range 2 months–18 years) underwent one or more internal thoracic artery (ITA) to coronary artery (CA) bypass grafts for Kawasaki disease (n = 4), congenital lesions (n = 3), post arterial switch (n = 4), and other iatrogenic obstructions (n = 5). Proximal left main CA arterioplasty was performed concurrently with ITA-CA bypass in 4 patients. Results . Survival is 93.8%. All bypass grafts in surviving patients are patent 2 months–11 years postoperation. The 11 elective patients are well (NYHA I–II). The 5 emergent operations were performed in 2 infants and 3 adolescents who had poor ventricular function prior to ITA-CA bypass due to iatrogenic injuries in 3, congenital critical left main stenosis in 1, and intraoperative iatrogenic coronary injury in 1. The 3 adolescents fared worse, resulting in death in the first, cardiac transplantation in the second, and full recovery in the third. The 2 infants have steadily improving ventricular function. Conclusions . ITA-CA bypass can be successfully performed in infants and children for expanding elective and life-saving indications with excellent results. Poor preoperative ventricular function often persists, especially in those older children with iatrogenic injuries, and may result in death or cardiac transplantation.

This analysis summarizes the first report of the Society of Thoracic Surgeons National Congenital Heart Surgery Database Committee in association with Summit Medical Systems. Twenty-four centers joined the program at various dates of entry resulting in 18,894 enrolled patient records. This report compiled the relevant clinical features of 18 congenital heart categories over a 4-year period (1994-1997), which included 8,149 patient records. The data analyses are largely descriptive in character. Missing data points were described and not omitted in the analysis. Statistical analysis was not performed due to missing data points in some categories. Certain trends, however, could be identified and are discussed. The first Society of Thoracic Surgeons National Congenital Heart Surgery Database Report has succeeded in establishing a finite record that can be improved to establish universal national and international utility, risk stratification, and scholarly outcome analyses.

Accelerated coronary artery disease (CAD) is the leading cause of late mortality following cardiac transplantation. The vascular lesions are characterized by myointimal proliferation and perivascular mononuclear inflammatory infiltrates. Platelet-activating factor (PAF, 1-O-alkyl-2-acetyl-sn-glycero-3-phosphocholine) is a potent phospholipid mediator produced by inflammatory cells and activated endothelial cells. Angiotensin II is known to activate phospholipase A2, a critical enzyme in PAF synthesis. Using a rat heterotopic cardiac transplant model known to induce graft CAD, we previously reported that chronic administration of captopril, an angiotensin converting enzyme inhibitor, reduces intimal proliferation and maintains luminal patency. The purpose of the current study was to determine if captopril regulates vascular remodeling by suppressing PAF synthesis and whether administration of a PAF antagonist ameliorates graft CAD. Captopril was found to decrease levels of PAF and PAF-like compounds as well as reduce intimal lesions, decrease cellular rejection grade, and diminish allograft heart weights. Treatment with a PAF antagonist significantly decreased proliferation of the intimal component of the vasculopathy and caused regression of the cardiac hypertrophy, but had no significant effect on cellular rejection. In contrast, untreated animals had elevated plasma PAF levels, elevated heart weights, and severe myointimal proliferation with luminal stenosis 21 days post-transplantation. These observations suggest that graft CAD is mediated, in part, by PAF and PAF-like compounds, and suppression of endogenous PAF may prevent cardiac allograft vasculopathy.

Background . We reviewed our operative experience and long-term results with repair of pectus excavatum and carinatum deformities through a vertical midline approach, including those cases with simultaneous intracardiac repair. Methods . From 1972 through 1998, 120 children underwent pectus deformity repair. Operative technique used a vertical midline incision with subperichondrial resection of deformed cartilages and an anterior sternal osteotomy. Thirty-five patients had a temporary metal bar for retrosternal support for 6 months; 85 underwent repair without a bar. Patients and parents were asked to assess the outcome after pectus repair as poor, fair, good, or excellent. Results . There were 94 male and 26 female patients (mean age, 8.4 years; range, 3 to 21 years). There were 111 cases of pectus excavatum and 9 of pectus carinatum. Fourteen children (11.5%) had an associated congenital heart defect; 9 patients had simultaneous pectus and intracardiac repair. One patient was referred for emergent open heart repair and pectus repair after attempted "Nuss" repair resulted in a perforated right atrium, perforated right ventricle, and partially disrupted tricuspid valve apparatus. There were no deaths and only one significant complication, which required a return to the operating room for bleeding. Morbidity was not higher in patients with simultaneous intracardiac repair. Long-term follow-up was established in 83% of patients. Results were classified as excellent in 64 patients (64%), good in 25 (25%), fair in 8 (8%), and poor in 3 (3%). Thirty (86%) of 35 patients with a sternal bar had excellent results versus 34 (52%) of 65 without a bar ( p = 0.004); 97% of patients who underwent repair with a sternal bar classified the result as excellent or good. Conclusions . Long-term results of pectus excavatum and carinatum repair through a vertical midline approach are excellent. Outcome with a temporary sternal bar is superior to outcome without a bar. Concomitant repair of congenital heart defects and pectus deformity may be performed successfully without additional morbidity.

Background . Mitral valve remodeling techniques were applied to 26 infants and children (mean age, 6.0 years, range, 0.4 to 15.9 years) with various forms of congenital mitral valve disease over a 7-year period. Patients with atrioventricular canal, l-transposition and single ventricle were excluded. Intraoperative transesophageal echocardiography (TEE) was utilized to assess the repair and guide the need for immediate intervention. Methods . Twenty-one patients had mitral regurgitation: 10 with cleft anterior mitral leaflet, 7 with annular dilatation, 1 with normal leaflets with an obstructing cord, 2 with prolapsed leaflets and elongated cords, and 1 with restricted leaflet motion, normal papillary muscles, and shortened cords. Of the 5 mitral stenosis patients, 3 had supravalvular mitral ring, 1 had midvalvular mitral ring, and 1 had a parachute valve. Three of the mitral stenosis patients had additional stenotic lesions. Concurrent repair of associated lesions was performed in 21 patients (78%). Results . Operative mortality was 3.8% (n = 1). There were no late deaths. Immediate rerepair in 4 patients resulted in improved function. All mitral stenosis patients improved. A total of 20 mitral regurgitation patients (95%) improved; 1 required mitral valve replacement. Mean follow-up is 31 months (range, 2 to 81 months). All patients are in New York Heart Association functional class I or II. Conclusions . Mitral valve repair can be successfully performed in infants and children with excellent short- and midterm results. Assessment using transesophageal echocardiography can guide the necessity for immediate rerepair to achieve improved function.

Background . Our surgical strategy for infant coarctation changed from subclavian flap aortoplasty to resection with extended end-to-end anastomosis in 1991. The purpose of this review was to evaluate the results of that strategy. Methods . From 1991 through 1997, 55 infants underwent repair of coarctation of the aorta using resection with extended end-to-end anastomosis. Isolated coarctation of the aorta was present in 26 patients, 20 patients had a ventricular septal defect, and 9 patients had other associated intracardiac lesions. Mean age at surgery was 0.20 ± 0.24 years (median, 21 days). In 34 patients (62%), arch reconstruction was performed through a left thoracotomy. Twenty patients (36%) had median sternotomy with simultaneous repair of coarctation of the aorta and intracardiac repair of associated lesions. One patient had recoarctation repair through a median sternotomy. All coarctation and ductal tissue was resected and the anastomosis was constructed starting opposite the left carotid artery with running polypropylene suture. Results . There was one early death 26 days after coarctation of the aorta and ventricular septal defect repair in a child on extracorporeal membrane oxygenation for meconium aspiration and 2 late deaths owing to pneumonia and pulmonary hypertension (1) and interventricular hemorrhage (1). There were no instances of paraplegia. Follow-up in survivors ranges from 10 to 76 months (mean, 39.8 ± 17.2 months). Recoarctation has developed in 2 patients, who have had successful balloon dilation 6 and 14 months after the operation. This yields a low recoarctation rate of 3.6%. Conclusions . Resection with extended end-to-end anastomosis yields a low mortality and particularly a low recoarctation rate and is our procedure of choice for infants with coarctation of the aorta.

Objectives: We review our surgical experience patients with atriopulmonary Fontan operations who had obstructive or arrhythmia indications for conversion to total cavopulmonary artery connections, arrhythmia circuit cryoablation, and placement of atrial antitachycardia pacemaker. Methods: Fourteen patients (mean age 14 ± 4 years) had conversion to total cavopulmonary artery connection 8 ± 3 years after the original atriopulmonary Fontan operation primarily for atrial arrhythmias in 11, obstructive lesions in 2, and bradycardia with cyanosis in 1. Arrhythmia circuit cryoablation was performed on 11 patients and 10 had atrial antitachycardia pacemakers. Preoperative functional New York Heart Association class was IV in 9, III in 4, and II in 1. Results: One patient had brain death (7%) presumably caused by resternotomy complications despite an excellent hemodynamic result. Another required reoperation for a maldeployed clamshell device after attempted fenestration closure. Average length of stay was 10 ± 3 days; chest tubes were removed on day 7 ± 3. There were no long-term deaths (mean follow-up 1.7 years, range 5 months to 5 years). Postoperative arrhythmias occurred in five patients, three of whom had successful termination by antitachycardia pacemaker and two who had pharmacologic control of their respective junctional ectopic and slow atrial tachycardia. All patients have improved to New York Heart Association class I or II. Conclusions: Total cavopulmonary artery conversion in association with arrhythmia circuit cryoablation and atrial antitachycardia pacemaker placement can be accomplished with low morbidity and mortality, oftentimes resulting in dramatic increases in functional class and control of life-threatening arrhythmias.(J Thorac Cardiovasc Surg 1998;115:547-56)

BACKGROUND Ventricular-to-pulmonary artery conduits in growing patients with congenital heart disease will require replacement from time to time due to somatic growth, neointimal hyperplasia, and pulmonary artery stenosis. The purpose of this article is to review our experience with ascending aortic extension for significant long-segment pulmonary artery stenosis in patients undergoing reoperation for right ventricular-to-pulmonary artery conduit replacement. METHODS From 1989 to 1997, 8 patients had aortic transection, right pulmonary artery augmentation arterioplasty, and aortic interposition graft (Hemashield in 7 and Gore-tex in 1) in association with right ventricular-to-pulmonary artery conduit replacement in 7 patients and completion Fontan operation in 1 patient. Aortic cross-clamp time was 90 +/- 34 minutes, and the cardiopulmonary bypass time was 205 +/- 37 minutes. RESULTS All patients survived. In those 7 patients who had conduit replacement, the RV/LV ratio declined from 0.78 +/- 0.15 to 0.45 +/- 0.05 postoperatively (P < 0.05). Average length of stay was 8.9 +/- 7.2 days. Follow-up range is 18 months to 8 years (mean 4 years). Two complications included cardiac transplantation for pre-existing poor left ventricular function and accelerated conduit stenosis leading to conduit re-replacement. CONCLUSION Ascending aortic extension facilitates long-segment pulmonary artery augmentation arterioplasty and enlarges the retroaortic space, preventing future compression restenosis.

Methods to repair tetralogy of Fallot have evolved from large ventriculotomy, large transannular patch placement techniques to smaller, transatrial approaches with valve-sparing strategies in select patients. Despite these advances, there continue to be patients in whom transannular patch is inevitable, and the management of the resulting pulmonary insufficiency that develops from this has been the source of considerable discussion. Techniques aimed at restoring pulmonary valve competence utilizing the remaining valve leaflets after transannular patch placement have recently been proposed for very select patient populations. We review the technical aspects of the operation including removal of the transannular patch and bicuspidization of a formerly tricuspid pulmonary valve, which results in a competent, nonstenotic valve. This report confirms the feasibility of these operative details and highlights the importance of planning before initial repair of tetralogy of Fallot as a way to prepare for a future valve restoration procedure and therefore avoid prosthetic valve placement.

We report a surgical approach using hepatic vein–to–azygos vein connection without cardiopulmonary bypass or deep hypothermic circulatory arrest in a patient with heterotaxy syndrome and interrupted inferior vena cava with persistence of pulmonary arteriovenous malformations (PAVMs) after previous Fontan completion. We advocate early performance of hepatic vein–to–azygos vein connection following the Kawashima operation for heterotaxy with functionally univentricular heart and interrupted inferior vena cava. We review the physiology of heterotaxy syndrome with congenital heart disease and justify our approach in the context of a review of previous surgical strategies used in this patient population.

While surgical repair of tetralogy of Fallot (TOF) is generally associated with good early outcomes, late complications affect long-term survival and may require reoperation. Pulmonary regurgitation (PR) and tricuspid regurgitation (TR) may increase the risk of arrhythmias, reduced cardiac function, and sudden death. Tricuspid valve function can be compromised secondarily in the setting of PR or directly as a result of injury or alteration of the valve related to the original TOF repair. This article reviews the etiologic mechanisms, pathophysiological implications, and surgical interventions for TR. Effective management following TOF repair requires consideration of TR to optimize late outcomes.

Thomas Baffes developed one of the first operations for transposition of the great arteries directing inferior vena cava flow to the left atrium using an interposed homograft in the era before open heart surgery. He performed 117 Baffes operations from 1953 to 1960, with 30% overall mortality, and an additional 85 Baffes procedures before 1968, allowing many to survive until the atrial baffle operations. During the early days before hospitals had cardiopulmonary bypass machines, Tom Baffes and colleagues purchased a heart-lung machine and transported it to various Chicago hospitals to treat patients and stimulate interest in this emerging technology.

Transposition of the great arteries (TGA) is a congenital heart defect in which there is malalignment of the aorta and pulmonary artery where the right ventricle (RV) gives rise to the aorta and the left ventricle (LV) gives rise to the pulmonary artery. Therefore there is atrioventricular concordance and ventricular–great vessel discordance, and the result is systemic cyanosis. There are three main categories of TGA

Vascular rings are congenital vascular anomalies of the aortic arch system which compress the esophagus and trachea. The two major categories of vascular rings are double aortic arch and right aortic arch with left ligamentum. A diagnosis of a vascular ring requires a high index of suspicion by the clinician. Clues to this diagnosis are noisy breathing, a barky cough, recurrent upper respiratory tract infections, and dysphagia. Vascular rings are repaired through a thoracotomy with division of the ligamentum, division of the smaller of the two aortic arches for double aortic arch, and resection of a Kommerell diverticulum if present. Almost all patients have resolution of their symptoms over a period of 6 months to 1 year. Patients with a pulmonary artery sling frequently have associated tracheal stenosis from complete cartilaginous tracheal rings. Repair is performed through a median sternotomy with the use of cardiopulmonary bypass. Simultaneous repair of associated intracardiac lesions and slide tracheoplasty for congenital tracheal stenosis are performed. Care of these patients requires multidisciplinary cooperation between cardiothoracic surgery, otolaryngology, anesthesia, and critical care.

Anomalies of the coronary arteries, either congenital or acquired, can be characterized as abnormalities of the standard anatomy of two patent and separately arising right and left arterial blood vessels. The coronaries are the first branches of the ascending aorta, arise from their respective sinuses of Valsalva, and gradually branch distally to the myocardium. Anomalies can be characterized as a lack of origin, abnormal origin, anomalous course, lack of patency, abnormal connections, and/or abnormal drainage of the coronary vessels. Interruptions or lack of flow can cause significant morbidity and mortality due to ischemia, infarction, and fistulous connections, which can lead to cardiac failure. Operative intervention for congenital coronary artery anomalies is highly dependent on the specific lesion. The technique required to resolve the problem is generally grounded in coronary under-perfusion.

Significant advances have been made in extracorporeal life support, which has resulted in the increased use of post-cardiotomy extracorporeal membrane oxygenation. Retrospective studies have contributed to the ongoing evolution of selection criteria for post-cardiotomy extracorporeal membrane oxygenation. Current indications include failure to wean from cardiopulmonary bypass, haemodynamic collapse, pulmonary hypertension, post-repair of hypoplastic left heart syndrome, or need for bridge to transplantation. Short- and mid-term results are improving. Ethical concerns still attend the process, however. Moral risks related to post-cardiotomy extracorporeal membrane oxygenation may be encountered before, during, and after the open heart procedure. At each stage of the decision-making process, moral risks are encountered by many factors that may result in decisions that may be contrary to the best interests of the patient, parents, or use of shared societal resources. These moral risks centre around the selection process, informed consent, decision making in the operating room, and post-operative maintenance of extracorporeal membrane oxygenation. Consideration of such risks is affected by questions of haemodynamic stability, haematologic compromise, neurologic status, and family concerns. We conclude that thorough understanding of the relevant scientific literature, heightened awareness of moral risks, and incorporation of ethical tenets in clinical deliberation will guide the clinician to do the right thing.

Significant challenges face developing countries as a result of the maldistribution of access to healthcare throughout the world, specifically access to paediatric cardiac care. Sustainable paediatric cardiac programmes must be established in developing countries to provide care to all children with congenital heart disease. Education and research are essential components to sustainable paediatric cardiac programmes in developing countries to define local problems and the incidence of disease, and to generate solutions thereto related. Research can contribute to developing local expertise, improving technology, providing opportunities for local talent, generating financial resources, enhancing the dignity of people, and the facilitating resolution of health problems throughout the world. Clinical trials conducted in developing countries should meet the same ethical standards as trials based in developed countries.

A low input impedance, intrathoracic artificial lung is being developed for use in acute respiratory failure or as a bridge to transplantation. The device uses microporous, hollow fibers in a 0.74 void fraction, 1.83 m2 surface area bundle. The bundle is placed within a thermoformed polyethylene terephthalate glucose modified housing with a gross volume of 800 cm3. The blood inlet and outlet are 18 mm inner diameter vascular grafts. Between the inlet graft and the device is a 1 inch inner diameter, thin-walled, latex tubing compliance chamber. These devices were implanted in Yorkshire pigs via median sternotomy with an end to side anastomosis to the pulmonary artery and left atrium. The distal pulmonary artery was occluded to divert the right ventricular output to the device. Pigs 1 and 2 were supported fully for 24 hrs and then killed. Pig 3 was supported partially for 20 hrs and died from bleeding complications. The first implant, in a 55 kg male pig, transferred an average of 176 ml/min +/- 42.4 of O2 and 190 ml/min +/- 39.7 of CO2 with an average blood flow rate of 2.71/min +/- 0.46. The normalized average right ventricular output power, Pn, was 0.062 W/(L/min) +/- 0.0082, and the average device resistance, R, was 3.5 mmHg/(L/min) +/- 0.62. The second implant, in a 60 kg male pig, transferred an average of 204 ml/min +/- 22.5 of O2 and 242 ml/min +/- 17.2 of CO2 with an average blood flow rate of 3.7 L/min +/- 0.45, Pn of 0.064 W/(L/min) +/- 0.0067, and R of 4.3 mmHg/(L/min) +/- 0.89. The third implant, in an 89 kg male pig, transferred an average of 156 ml/min +/- 39.6 of O2 and 187 ml/min +/- 21.4 of CO2 with an average blood flow rate of 2.5 L/min +/- 0.49, Pn of 0.052 W/(l/min) +/- 0.0067, and R of 3.4 mmHg/(L/min) +/- 0.74. These experiments suggest that such an artificial lung can temporarily support the gas transfer requirements of adult humans without over-loading the right ventricle.

A new prototype of a pumping artificial lung (PAL) has been designed and tested. The device performs the functions of both the pump and oxygenator components of an extracorporeal perfusion circuit. Previous prototypes that the authors developed (Type A) had gas exchanging microporous fibers formed into propeller-like vanes that, upon rotation, pump the blood. The design of the new PAL prototypes (Type B) uses the rotation of an annular bank of fibers to drive flow. The fiber bank, including sealed gas manifolds, lies within the housing of a modified Bio-Medicus BMP-50 pump head (Bio-Medicus, Eden Prairie, MN). Rotation of the fiber bank is driven through a magnetic coupling. Inlet and outlet gas lines enter the pump head through a sealed bearing. The Type A PAL suffered from insufficient pumping rates and gas exchange, necessitating redesign. The authors have constructed two PAL-B prototypes with a priming volume of only 140 ml and gas exchange surface areas of 0.16 and 0.60 m2. During in vitro saline testing, these prototypes showed significant pump performance, pumping 7.0 L/min against zero head at 3,500 rpm. The larger prototype had exchange rates in saline of up to 71 ml O2/min and 75 ml CO2/min. Gas exchange fluxes (O2 = 119 ml/[min $$ m2] and CO2 = 125 ml/[min $$ m2]) for the PAL-B are significantly higher than that of commercially available oxygenators in saline. Future prototypes will have increased surface area and fibers smaller than the 0.038 cm outside diameter fibers used in the present prototypes. A primary concern in using microporous fibers to push the blood was the durability of the fibers at high pump speeds, High speeds exhibited no negative effects on gas exchange abilities or fiber duarbility

Background. Current corrective surgical approaches for the Taussig-Bing heart include arterial switch with ventricular septal defect (VSD) closure and intraventricular repair as described by Kawashima. Methods. Between 1983 and 1994, 20 children underwent intracardiac repair of Taussig-Bing anomaly. Mean age at operation was 17 months (range, 1 week to 9 years). Prior palliation included pulmonary artery band (15) with coarctation repair (8) and atrial septectomy (1). Arterial switch with VSD closure was performed in 16 patients, 10 with anteroposterior great arteries. Kawashima repair was performed in 4 patients, all with side-by-side great arteries. Results. After arterial switch, there was one operative death (6.2%) due to myocardial ischemia and three late deaths (18.7%) due to pulmonary hypertension, gastrointestinal bleeding, and acute lymphocytic leukemia. In the Kawashima repair group there have been no deaths. After arterial switch, 9 patients underwent 11 reoperations for residual coarctation (3), residual pulmonary artery stenosis (2), aortic valve replacement, aortic valvuloplasty, unrecognized VSD, mitral valvuloplasty, mediastinitis, and pacemaker insertion. After Kawashima repair, 1 patient underwent reoperation for baffle stenosis and 1 for an unrecognized VSD. Conclusions. For children with Taussig-Bing anomaly, the Kawashima intraventricular repair (for side-by-side great arteries) preserves the native aortic valve and avoids coronary dissection. The arterial switch operation with VSD closure can be applied without ventriculotomy to all great artery relationships and allows neonatal repair with or without concomitant coarctation repair. Both techniques yield excellent early and intermediate-term results despite the high rates of prerepair palliation and postrepair reoperation for both groups.

The management and quantification of bone loss is a major challenge in primary and revision total hip replacement. Defining the normal three-dimensional (3D) anatomy of the acetabular vault could aid in assessing pathologic changes and in designing prosthetic joint components. We performed a quantitative assessment of normal 3D acetabular vault structure to define the shape and location of weight-bearing acetabular bone referred to as the vault. Images from 70 normal hip computed tomography images were used to define the 3D acetabular vault anatomy and develop a 3D model. Variation in vault shape was quantified by measuring the distance between every surface point on a subject’s hemipelvis and the reference vault. Variation among different hip alignments was assessed using 19 scans from subjects with varus, valgus and dysplastic hip morphologies. The acetabular vault model had 96.6% (95% CI: 91.7–101.5), 97.8% (95% CI: 94.5–101.1) and 96.4% (95% CI: 98.7–94.1) of the surface points within 3 mm of normal male, normal female and abnormal hip specimens, respectively. Comparison of acetabular vault model fit between gender and hip types revealed that it was only significantly different between normal males and normal females (P = 0.0194) and between normal males and dysplastic females (P = 0.0377). A conserved 3D acetabular vault shape and location exists that can accommodate various hip morphologies. Defining a normal vault may increase the precision with which hip pathology can be identified and may also serve as a preoperative assessment tool for planning total hip arthroplasty.

Total anomalous pulmonary venous connection with obstruction constitutes a surgical emergency. Medical therapy is palliative and unlikely to result in significant or sustained physiologic improvement. Two cases demonstrate the successful use of a novel management scheme in which patients are admitted directly to the operating room for diagnosis confirmation and treatment, obviating the need for time-consuming preoperative assessment in an intensive care unit before surgery.

Background Extracardiac conduit and lateral atrial tunnel total cavopulmonary connection are both widely used in the management of functionally univentricular hearts. The effect of the type of connection on early outcomes after Fontan operation remains unclear. We evaluated the effect of Fontan type on early outcome in a large clinical database. Methods Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing the Fontan operation (2000 to 2009) were included. We used multivariable analysis to evaluate the effect of Fontan type (extracardiac conduit vs lateral tunnel) on in-hospital death, Fontan takedown/revision, Fontan failure (in-hospital death or Fontan takedown/revision), postoperative length of stay, and complications, adjusting for patient, procedural, and center factors. Results The study included 2,747 patients (61% male) from 68 centers. A right-dominant ventricle was present in 45%. Extracardiac conduit Fontan (vs lateral atrial tunnel) was performed in 63%; in all, 65% were fenestrated. In multivariable analysis with adjustment for patient, procedural (including fenestration), and center factors (including Fontan volume), the extracardiac conduit Fontan was associated with significantly higher Fontan takedown/revision (odds ratio, 2.73; 95% confidence interval, 1.09 to 6.87) and Fontan failure (odds ratio, 2.28; 95% confidence interval, 1.13 to 4.59), and longer postoperative hospital stay (adjusted estimated difference in postoperative hospital stay: +1.4 days). Conclusions These multicenter data suggest that of the two prevalent forms of Fontan connection in current use, the lateral atrial tunnel Fontan may be associated with superior early outcomes.

This article presents 21 “Quality Measures for Congenital and Pediatric Cardiac Surgery” that were developed and approved by the Society of Thoracic Surgeons (STS) and endorsed by the Congenital Heart Surgeons’ Society (CHSS). These Quality Measures are organized according to Donabedian’s Triad of Structure, Process, and Outcome. It is hoped that these quality measures can aid in congenital and pediatric cardiac surgical quality assessment and quality improvement initiatives.