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This review will outline the optimal, initial palliation for children who are born with a functionally univentricular heart. Optimizing the initial palliation is of critical importance in this patient population to prevent potential problems such as systemic outflow and pulmonary vein obstruction that may complicate further surgical intervention. The palliative techniques that are discussed include pulmonary artery banding, modified Blalock-Taussig shunt, Damus-Kaye-Stansel procedure, modified Norwood, hybrid, and early bidirectional Glenn. Our recommendations for optimal palliation for children with a univentricular heart are based on our experience with nearly 200 patients who had either a lateral tunnel or extracardiac Fontan procedure and 130 patients who had Fontan conversion with arrhythmia surgery.

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., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Surgical techniques for repair of supravalvular aortic stenosis (SVAS) include McGoon's one-patch, Doty's two-patch, and Brom's three-patch method. In this review we evaluated mid-term clinical outcomes of these techniques at our institution., Methods: Our cardiac surgery database identified patients with SVAS repair from 1990 to 2008. Follow-up records, reintervention and reoperation data, and most recent echocardiograms were obtained., Results: From 1990 to 2008, 20 patients (70% male) underwent surgery for SVAS. Mean age was 3.6 +/- 5.6 years. In chronological sequence, 8 patients had single-patch aortoplasty, 4 had the Doty procedure, and 8 received Brom's symmetric three-patch aortoplasty. Of the Brom patients, 6 had Williams syndrome. Aortic cross-clamp times were 40.1 +/- 13.6 minutes (one-patch), 60.3 +/- 38.8 minutes (Doty), and 104 +/- 20.5 minutes (Brom). Perioperative mortality was 5.0% (1 patient in one-patch group). Mean postoperative length of stay was 10 +/- 10.6 days. Follow-up data were available for all survivors (mean follow-up, 6.3 +/- 6.0 years; range, 6 months to 16 years). There were no late deaths. Follow-up echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 33 +/- 18.0 mm Hg (one-patch), 10 +/- 1 mm Hg (Doty), and 18 +/- 12 mm Hg (Brom). All patients in the Doty and Brom groups had less than moderate aortic insufficiency. Reoperations were required in 5 of 8 one-patch patients (62%) for residual aortic stenosis (n = 3), aortic insufficiency (n = 1), and subvalvar stenosis (n = 1). No Doty or Brom patient has required aortic reoperations, which was nearly statistically associated with freedom from reoperation (p = 0.06). Subvalvar stenosis was the only risk factor associated with reoperation (p = 0.0028)., Conclusions: Despite a longer cross-clamp time, SVAS repair by Doty or Brom aortoplasty restores normal hemodynamics and reduces the need for reoperation when compared with the classic one-patch technique. Our current preference for SVAS repair is the Brom three-patch symmetric aortoplasty., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

A detailed hierarchal nomenclature of arrhythmias is offered with definition of its applications to diagnosis and complications. The conceptual and organizational approach to discussion of arrhythmias employs the following sequence: location--mechanism--aetiology--duration. The classification of arrhythmias is heuristically divided into an anatomical hierarchy: atrial, junctional, ventricular, or atrioventricular. Mechanisms are most simplistically classified as either reentrant, such as macro-reentrant atrial tachycardia, previously described as atrial flutter, or focal, such as automatic or micro-reentrant tachycardia, for example, junctional ectopic tachycardia. The aetiology of arrhythmias can be either iatrogenic, such as postsurgical, or non-iatrogenic, such as genetic or congenital, and in many cases is multi-factorial. Assigning an aetiology to an arrhythmia is distinct from understanding the mechanism of the arrhythmia, yet assignment of a possible aetiology of an arrhythmia may have important therapeutic implications in certain clinical settings. For example, postoperative atrial arrhythmias in patients after cardiac transplantation may be harbingers of rejection or consequent to remediable imbalances of electrolytes. The duration, frequency of, and time to occurrence of arrhythmia are temporal measures that further refine arrhythmia definition, and may offer insight into ascription of aetiology. Finally, arrhythmias do not occur in a void, but interact with other organ systems. Arrhythmias not only can result from perturbations of other organ systems, such as renal failure, but can produce dysfunction in other organ systems due to haemodynamic compromise or embolic phenomena.

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up. During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET]. This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both of these two organizations to analyze outcomes of over 150,000 operations involving patients undergoing surgical treatment for congenital cardiac disease. Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between congenital and paediatric cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology. In finalizing our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.

Conversion of the atriopulmonary Fontan to a total cavopulmonary extracardiac connection with concomitant arrhythmia surgery and pacemaker placement is a safe and efficacious procedure for this patient population. From 1994 to 2007 a total of 118 patients have undergone this procedure with one (0.8%) early and nine (7.6%) late deaths. During the course of our experience with Fontan conversion our surgical strategy has evolved to include various ablative techniques to treat macro re-entrant atrial tachycardia, focal (automatic) atrial tachycardia, atrioventricular nodal reentry tachycardia, atrial tachycardia due to accessory connections, atrial fibrillation, and ventricular tachycardia. The various mechanisms that we use to treat the underlying atrial arrhythmias are described in this review. We have also encountered patients with variations of the Fontan and other complex anatomic and pathophysiologic aberrations who were not amenable to standard takedown and ablative procedures. We describe those circumstances and the solutions we found to treat those patients.

As of September 2007, 120 patients have undergone a Fontan conversion procedure at Children's Memorial Hospital (Chicago, IL). One of the primary indications for surgery in these patients has been arrhythmias, either atrial reentry tachycardia or atrial fibrillation. The surgical treatment of those two lesions has been with the modified right atrial maze and the Cox-maze III. The purpose of this review is to describe our strategy for performing the maze procedure in these single-ventricle patients. The primary tool for performing the maze in this series has been with the cryocatheter with cryoablation at -160 degrees C for 1 minute. These cryoablation lesions have been combined with standard surgical incisions in the right and left atria. The early mortality in this series is 1%, late mortality is 5%. Freedom from atrial reentry tachycardia recurrence at 5 years is 86%. Freedom from atrial fibrillation recurrence is 98% at 5 years.

Surgical management of pediatric patients with the diagnosis of complete atrioventricular canal (CAVC) is accomplished by using one of three procedures: (1) the classic single-patch, (2) two-patch, or (3) modified single-patch technique. Of these, the modified single-patch is currently the best technique available for repair of CAVC. The goal of this review is to describe our experience with the modified single-patch technique and explain how it evolved into our procedure of choice for repair of CAVC in our pediatric population. We analyzed specific outcomes (operative mortality, late mortality, mitral valve reoperation, and the incidence of heart block) from our center and those of other institutions that used the modified single-patch technique and compared these with the most current results of the classic single-patch and two-patch techniques reported from several other centers. Our analysis showed that while the occurrence of operative and late mortality was comparable in all three techniques, there was clearly a lower incidence of late reoperations for mitral valve insufficiency and a lower rate of heart block in patients who were repaired with the modified single-patch technique. For these reasons and because of its simplicity, we have adopted the modified single-patch technique as our procedure of choice for repair of complete atrioventricular canal in infants and children.

The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by converting the original atriopulmonary connection to a total cavopulmonary artery extracardiac connection and treat the underlying atrial arrhythmias. Successful outcomes of this procedure are dependent on a thorough understanding of several factors: the patient's fundamental diagnosis of single-ventricle anatomy, the resultant cardiac configuration from the original atriopulmonary Fontan connection, right atrial dilatation that leads to atrial flutter or fibrillation, and associated congenital cardiac anomalies. The purpose of this article is to present some of the more challenging anatomic and electrophysiologic problems we have encountered with Fontan conversion and arrhythmia surgery and the innovative solutions we have used to treat them. The cases reviewed herein include: takedown of a Bjork-Fontan modification, right ventricular hypertension and tricuspid regurgitation after atriopulmonary Fontan for pulmonary atresia and intact ventricular septum, takedown of atrioventricular valve isolation patch for right-sided maze procedure, resultant hemodynamic considerations leading to intraoperative pulmonary vein stenosis after Fontan conversion, unwanted inferior vena cava retraction during the extracardiac connection, right atrial cannulation in the presence of a right atrial clot, distended left superior vena cava causing left pulmonary vein stenosis, dropped atrial septum, and the modified right-sided maze procedure for various single-ventricle pathology. Since 1994 we have performed Fontan conversion with arrhythmia surgery on 109 patients with a 0.9% mortality rate. We attribute our program's success in no small measure to the strong collaborative efforts of the cardiothoracic surgery and cardiology teams.

Background: The purpose of this study is to analyze atrioventricular valve procedures when performed in association with repeat Fontan operations and to determine the influence of atrioventricular valvar pathology, ventricular function, and arrhythmias on outcome., Methods: Between December 1994 and August 2004, 80 patients had repeat Fontan operations that included arrhythmia surgery (78 of 80), venous pathway revision (78 of 80), atrioventricular valve repair-replacement (15 of 80), and other associated procedures. Mean ages were the following: at operation, 20.3 +/- 8.4 years; at prior Fontan, 7.1 +/- 5.8 years. Atrioventricular valve procedures were performed on 8 functionally mitral and 7 functionally tricuspid valves. The average cross-clamp and cardiopulmonary bypass times were 61.9 +/- 42.8 minutes and 218 +/- 82 minutes, respectively., Results: Ventricular dysfunction (8% vs 54%, p < 0.0001), valvar dysfunction (13% vs 25%, p < 0.05), and atrial arrhythmias (18% vs 86%, p < 0.0001) increased during the preceding 12.0 +/- 4.7 years before the most recent Fontan operation. Multivariate analysis for death, orthotopic cardiac transplantation (OCT), or renal dialysis showed severe ventricular dysfunction, age greater than 25 years, right or ambiguous functional ventricle, and ischemic time greater than 100 minutes to be highly significant. Notably, cardiac index, elevated end diastolic pressure, and atrial fibrillation were not predictors of outcome. Mitral valve repairs were inconsistent due to probable technical misjudgments; most tricuspid valves could not be repaired. Operative and late mortality were 1.2% and 5.0%, respectively. Emergent and late OCT were 1.2% and 3.7%, respectively., Conclusions: Risk factors for poor outcome are severe ventricular dysfunction, right or ambiguous single ventricle, age greater than 25 years, and ischemic time greater than 100 minutes. Mitral valves are potentially more amenable to repair than are tricuspid valves. Prosthetic valve replacement should be considered when valve repair is questionable.