Search

The majority of congenital heart abnormalities of the mitral valve that require surgery in adulthood are regurgitant lesions. Congenital mitral stenosis that requires treatment in adulthood is often in the setting of the Shone complex or rheumatic valve disease and occasionally atrioventricular septal defect (AVSD). A brief summary of the regurgitant and stenotic lesions associated with congenital heart disease is given below. While this valve may be more accurately referred to as the left atrioventricular valve in many circumstances, particularly in the setting of AVSD, the term mitral is used to simplify the prose throughout these sections. In addition, while the term zone of apposition is more accurate anatomically, the term cleft is often used throughout this (and the AVSD) section.

The Fontan operation has undergone many revisions since its introduction by Francis Fontan and Guillermo Kreutzer in 1968. Gone are the venous pathway valves, the right atrial connections to small subpulmonic ventricles, and atriopulmonary connections. The standard therapy for the functionally single-ventricle heart is determined by three distinct time periods. The first is the neonatal management of pulmonary artery flow (by systemic to pulmonary artery shunt, pulmonary artery band, or observation). At approximately 6 months of age, the second stage takes place: systemic to pulmonary artery shunt takedown or pulmonary artery band takedown and a bidirectional Glenn shunt that prepares the single ventricle by volume unloading, resulting in favorable ventricular remodeling. By 2–3 years of age, the Fontan operation is usually performed. Since the introduction of the atrial fenestration, some surgeons have used this obligatory right-to-left shunt in all cases; some surgeons have used it selectively, and others have not used it at all. Most surgeons now use the fenestration selectively, especially in cases with borderline ventricular function, small pulmonary arteries, abundant aortopulmonary artery collaterals, and single-lung physiology.

Clearly, the most common obstructive disease resulting in myocardial ischemia in adults is atherosclerotic coronary artery disease, which often requires coronary artery bypass surgery or transcatheter stenting. A number of congenital lesions can also cause ischemic syndromes that can result in myocardial infarction and sudden death. The treatment options for these anatomic coronary variations are rarely coronary artery bypass, so they deserve our attention and review.

Today, it is extremely rare for an adult patient to present with a patent arterial duct (PAD). Moreover, when such a patient is identified, the preferred and initial treatment involves transcatheter device closure. Surgical options are reserved for complications of endocarditis (see Chap. 16), aneurysm formation, and failure of device closure. The surgeon, therefore, may be called on to treat PAD in the adult as the only lesion or in association with other congenital heart defects.

Atrial septal defects (ASDs) can be characterized as a hole somewhere in the atrial septum. The hole allows blood to flow between the left and right atria, resulting in pulmonary overcirculation in the case of low pulmonary vascular resistance (left-to-right shunt) or cyanosis in the case of high pulmonary vascular resistance (right-to-left shunt; Eisenmenger’s syndrome).

Concomitant arrhythmia surgery in association with initial or repeat congenital heart repairs in adults has assumed an important role in the treatment of congenital heart disease. The most common long-term complications after congenital heart repairs are arrhythmias and heart failure and can lead to decreased functional class, multiple readmissions to the hospital, and sudden death. Every adult patient who presents for repair of congenital heart disease with concomitant arrhythmias should be approached with an opportunity to treat both entities, as many reports have documented the efficacy and utility of this approach. This chapter is organized by type of arrhythmia, mechanism, and treatment options.

Cardiac transplantation in the adult with congenital heart disease is challenged by confounding pathophysiological states as well as anatomic variation in venous and great vessel anatomy. Indications for cardiac transplantation in this population center around a few different conditions

Congenital aortic aneurysms in the adult generally occur in patients with genetic abnormalities such as Marfan, Loeys-Dietz, or Ehlers-Danlos syndromes. Another indication for valve-sparing aortic aneurysm repair is a sinus of Valsalva aneurysm. Surgical management of aortic root pathology in these patients is dependent on aortic leaflet competence, which determines whether a valve-sparing or valve-replacement strategy should be implemented. Aneurysms causing dissections that extend into the transverse arch and descending aorta necessitate a different strategy, which repairs the dissection and re-establishes unobstructed flow into the brachiocephalic arteries. This management scheme is referred to as “de-branching.”

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.

Coronary artery disease ranges from congenital in origin such as anomalous aortic origin of a coronary artery (AAOCA) to acquired diseases such as Kawasaki disease, and previously repaired conditions such as transposition of the great arteries. It is not uncommon, in the long run, for proximal coronary stenosis to develop following arterial switch, AAOCA repair, Ross procedure and Kawasaki disease leading to coronary artery bypass grafing (CABG). The objective of this report is to discuss the indications, challenges, and outcomes of CABG in infants, children, and young adults with acquired and congenital lesions.

Survival of children with functionally univentricular hearts has improved due to staged correction, innovative venous-pulmonary artery connections, and structured perioperative care. A rapidly growing number of these patients are reaching adulthood and are being admitted to intensive care units in greater numbers. Although many adults with functionally univentricular hearts will have undergone a Fontan operation, some will present unoperated or with incomplete single ventricle surgical palliation. Acute on chronic heart failure and the end-organ manifestations of this physiologic state are the most common indications for intensive care unit admission. Given the unique cardiovascular anatomy, physiology, and non-cardiac comorbidities present in this patient population, an understanding of the nuances in cardiac critical care evaluation and management is necessary to achieve optimal outcomes. Access to medical and surgical subspecialists with expertise in adult congenital heart disease is often needed. Thus, when feasible, intensive care for this patient population should be provided in a regional adult congenital heart disease center. In this chapter, we summarize the most common indications for intensive care admission in adults with functionally univentricular hearts, as well as the general principles regarding evaluation in the context of critical illness. We also review critical care management strategies that seem most appropriate based upon the unique physiologic characteristics and cardiopulmonary interactions inherent to this patient population.

Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure.

The history of the first 41 years of the Congenital Heart Surgeons Society (CHSS) was recorded in 2015 which chronicled the metamorphosis of a small informal meeting into a mature organization with bylaws, officers, committees, funded research, the Kirklin-Ashburn Fellowship, and a vision to become the premier organization of congenital heart surgery in North America. Chief among these transformations was the implementation and development of the CHSS Data Center. Member participation, fellowship education, and significant outcomes research have been the hallmark of the CHSS. This incremental historical review highlights continued CHSS sentinel advances. Fifty-three CHSS Data Center manuscripts have been published. Citation scores (number of literature citations that each manuscript has accrued) have been collated and analyzed by cohort study. The average citation score for all manuscripts was 75.4 ± 76.3 (range: 1-333). The Kirklin/Ashburn Fellowship continues to thrive with academic achievements and generous contributions to the endowment. The World Journal of Pediatric and Congenital Heart Surgery has been adopted as the official organ of the CHSS. A Past President’s Dinner has been inaugurated serving as a senior advisory committee to the Executive Council. Toronto Work Weekends continue. Congenital Heart Surgeons Society growth has accrued to 159 active members and 82 institutional members. Future considerations include the size, content, and duration of the annual meeting; the potential for increased membership; and political penetrance into national cardiothoracic governing organizations regarding committee appointments, executive council representation, and education initiatives. Congenital Heart Surgeons Society has achieved numerous advances during this incremental period.

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.

The treatment of rare and expensive medical conditions is one of the defining qualities of paediatric cardiology and congenital heart surgery. Increasing concerns over healthcare resource allocation are challenging the merits of treating more expensive forms of congenital heart disease, and this trend will almost certainly continue. In this manuscript, the problems of resource allocation for rare and expensive medical conditions are described from philosophical and economic perspectives. The argument is made that current economic models are limited in the ability to assess the value of treating expensive and rare forms of congenital heart disease. Further, multi-disciplinary approaches are necessary to best determine the merits of treating a patient population such as those with significant congenital heart disease that sometimes requires enormous healthcare resources.

Anomalous aortic origins of the coronary arteries comprise approximately one-third of all coronary artery anomalies and are characterised by coronary arteries with anomalies of aortic origin involving abnormal courses, stenoses, and compression that can lead to myocardial ischaemia and sudden death. Operative techniques to treat these anomalies have not been standardised yet. Moreover, the management of potential complications has not been addressed. Common and rare forms of anomalous aortic origins of the coronary arteries are reviewed and understood standard techniques for an uncomplicated unroofing procedure are illustrated. Also noted are techniques that can be applied to unexpected anatomical findings and unwanted complications that could prove to be life-threatening. Several technical recommendations are offered.

Respect for patient autonomy is an important and indispensable principle in the ethical practice of clinical medicine. Legal tenets recognise the centrality of this principle and the inherent right of patients of sound mind – properly informed – to make their own personal medical decisions. In the course of everyday medical practice, however, challenging cases may result in ethical dilemmas for the patient, the physician, and society. Resolution of these dilemmas requires a thorough understanding of the underlying principles that allow the clinician to make informed decisions and to offer considered therapeutic options to the patient. We argue in this paper that there is also need for a transition of moral competency from understanding principles to attaining virtue in the classic Aristotelian tradition. Achieving moral virtue is based on a lifetime of learning, practising, and watching how others, who have achieved virtue, act and perform their duties. We further claim that learning moral virtue in medical practice is best realised by incorporating the lessons learnt during daily rounds where frank discussions and considered resolutions can occur under the leadership of senior practitioners who have achieved a semblance of moral excellence.

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.

The President's Award for best scientific paper is presented to esteemed members at each Southern Thoracic Surgical Association annual meeting to stimulate friendly scientific competition. Between 1964 and 2015, 58 awards were presented for clinical expertise in general thoracic surgery (18), adult cardiac surgery (25), and congenital heart surgery (15). Manuscripts were published by The Annals of Thoracic Surgery, and the average number of subsequent citations in professional medical journals as a measure of scientific importance was 43.7 ± 60.2. A seemingly prosaic addition of a yearly scientific award has had an enormous impact on the scientific community and membership participation.

The American College of Cardiology (ACC) and the American Heart Association (AHA) support their members’ goal to improve the care of patients with cardiovascular disease through professional education, research, and development of guidelines and standards and by fostering policies that support optimal patient outcomes. The ACC and AHA recognize the importance of the use of clinical data standards for patient management, assessment of outcomes, and conduct of research, and the importance of defining the processes and outcomes of clinical care, whether in randomized trials, observational studies, registries, or quality improvement initiatives. Clinical data standards strive to define and standardize data relevant to clinical concepts, with the primary goal of facilitating uniform data collection by providing a platform of clinical terms with corresponding definitions and data elements. Broad agreement on a …

Background Surgeons in training for congenital cardiac surgery face considerable challenges owing to procedure complexity, closely scrutinized outcomes, and a steep learning curve. Simulation methods have been initiated in other surgical specialties, but have yet to be established for congenital cardiac surgery trainees. The purpose of this study was to assess high-fidelity simulation as a method to train and improve skills of resident trainees learning critical components of index congenital cardiac surgical procedures. Methods Using 5 neonatal piglets over a period of 2.5 days, the following procedures were simulated: Norwood procedure, arterial switch operation, neonatal Ross procedure, tetralogy of Fallot repair, systemic to pulmonary artery shunt procedures, transmediastinal coarctation repair, atrial septal defect repair, ventricular septal defect repair, and right ventricular to pulmonary artery conduit. Anastomoses were tested with saline, all procedures were timed and video recorded, and resident trainee techniques and skills were critiqued by the instructor. Results All aspects of the procedures were simulated with minimal modifications. Anastomoses were tested, and the procedure successfully replicated without the pressures of operative time. Operative techniques involving suture placement in neonatal tissue, depth perception, and patch size estimation were corrected in real time, resulting in observed improvement of surgical skills. Video review allowed for further pedagogic value through examination and documentation of competency. Conclusions This neonatal porcine simulation model allows surgical trainees in congenital heart surgery to make and correct mistakes in a safe and controlled learning environment without compromising patient safety, thereby fostering surgeon competence and confidence.

Despite tremendous advances in surgical treatment of tetralogy of Fallot, augmenting the small right ventricular outflow tract remains a challenge. Transannular patch augmentation revolutionised surgical management, but did so at the expense of rendering patients with pulmonary insufficiency and the resulting problems associated therewith. Recent surgical efforts have focused on pulmonary valve preservation at initial correction and pulmonary valve restoration after transannular patching, with favourable results. In this manuscript, we review methods of pulmonary valve preservation and restoration.

Purpose: A link has been created between the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the Congenital Heart Surgeons’ Society Database (CHSS-D). Five matrices have been created that facilitate the automated identification of patients who are potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices are now used to (1) estimate the denominator of patients eligible for CHSS studies and (2) compare “eligible and enrolled patients” to “potentially eligible and not enrolled patients” to assess the generalizability of CHSS studies. Methods: The matrices were applied to 40 consenting institutions that participate in both the STS-CHSD and the CHSS to (1) estimate the denominator of patients that are potentially eligible for CHSS studies, (2) estimate the completeness of enrollment of patients eligible for CHSS studies among all CHSS sites, (3) estimate the completeness of enrollment of patients eligible for CHSS studies among those CHSS institutions participating in each CHSS cohort study, and (4) compare “eligible and enrolled patients” to “potentially eligible and not enrolled patients” to assess the generalizability of CHSS studies. The matrices were applied to all participants in the STS-CHSD to identify patients who underwent frequently performed operations and compare “eligible and enrolled patients” to “potentially eligible and not enrolled patients” in following five domains: (1) age at surgery, (2) gender, (3) race, (4) discharge mortality, and (5) postoperative length of stay. Completeness of enrollment was defined as the number of actually enrolled patients divided by the number of patients identified as being potentially eligible for enrollment. Results: For the CHSS Critical Left Ventricular Outflow Tract Study (LVOTO) study, for the Norwood procedure, completeness of enrollment at centers actively participating in the LVOTO study was 34%. For the Norwood operation, discharge mortality was 15% among 227 enrolled patients and 16% among 1768 nonenrolled potentially eligible patients from the 40 consenting institutions. Median postoperative length of stay was 31 days and 26 days for these enrolled and nonenrolled patients. For the CHSS anomalous aortic origin of a coronary artery (AAOCA) study, for AAOCA repair, completeness of enrollment at centers actively participating in the AAOCA study was 40%. Conclusion: Determination of the denominator of patients eligible for CHSS studies and comparison of “eligible and enrolled patients” to “potentially eligible and not enrolled patients” provides an estimate of the extent to which patients in CHSS studies are representative of the overall population of eligible patients; however, opportunities exist to improve enrollment.

Purpose: The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) is the largest Registry in the world of patients who have undergone congenital and pediatric cardiac surgical operations. The Congenital Heart Surgeons’ Society Database (CHSS-D) is an Academic Database designed for specialized detailed analyses of specific congenital cardiac malformations and related treatment strategies. The goal of this project was to create a link between the STS-CHSD and the CHSS-D in order to facilitate studies not possible using either individual database alone and to help identify patients who are potentially eligible for enrollment in CHSS studies. Methods: Centers were classified on the basis of participation in the STS-CHSD, the CHSS-D, or both. Five matrices, based on CHSS inclusionary criteria and STS-CHSD codes, were created to facilitate the automated identification of patients in the STS-CHSD who meet eligibility criteria for the five active CHSS studies. The matrices were evaluated with a manual adjudication process and were iteratively refined. The sensitivity and specificity of the original matrices and the refined matrices were assessed. Results: In January 2012, a total of 100 centers participated in the STS-CHSD and 74 centers participated in the CHSS. A total of 70 centers participate in both and 40 of these 70 agreed to participate in this linkage project. The manual adjudication process and the refinement of the matrices resulted in an increase in the sensitivity of the matrices from 93% to 100% and an increase in the specificity of the matrices from 94% to 98%. Conclusion: Matrices were created to facilitate the automated identification of patients potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices have a sensitivity of 100% and a specificity of 98%. In addition to facilitating identification of patients potentially eligible for enrollment in CHSS studies, these matrices will allow (1) estimation of the denominator of patients potentially eligible for CHSS studies and (2) comparison of eligible and enrolled patients to potentially eligible and not enrolled patients to assess the generalizability of CHSS studies.

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.

A 9-year-old boy who was born with bicuspid aortic stenosis underwent two unsuccessful aortic valvuloplasty interventions, and by 2 years of age he developed restrictive cardiomyopathy caused by left ventricular endocardial fibroelastosis and diastolic dysfunction. The attending cardiologist referred the patient to a high-volume, high-profile congenital cardiac surgical programme 1000 miles away that has a team with considerable experience with left ventricular endocardial fibroelastosis resection and a reputation of achieving good results. Owing to problems with insurance coverage, the parents sought other options for the care of their child in their home state. Dr George Miller is a well-respected local congenital and paediatric cardiac surgeon with considerable experience with the Ross operation as well as with right ventricular endocardial fibroelastosis resection. When talking with Dr Miller, he implied that there is little difference between right ventricular endocardial fibroelastosis and left ventricular endocardial fibroelastosis resection, and stated that he would perform the operation with low mortality based on his overall experience. Dr Miller stated that the local institution could provide an equivalent surgical procedure with comparable outcomes, without the patient and family having to travel out of state. A fundamental dilemma that often arises in clinical surgical practice concerns the conduct of assessing and performing new procedures, especially in rare cases, for which the collective global experience is scant. Although Dr Miller has performed right ventricular endocardial fibroelastosis resection, this procedure differs from left ventricular endocardial fibroelastosis resection, and he cannot be sure that he will indeed be able to perform the procedure better than the high-volume surgeon. This ethical situation is best understood in terms of the principles of respect for patient autonomy, beneficence, non-maleficence, and justice. The tension between the imperatives of beneficence and the obligation to respect the autonomy of the patient by acting only with the patient’s best interest in mind is discussed.

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.

Arrhythmia surgery in patients with congenital disease is challenged by the range of anatomic variants, arrhythmia types, and intramyocardial scar location. Experimental and clinical studies have elucidated the mechanisms of arrhythmias for accessory connections, atrial fibrillation, atrial reentry tachycardia, nodal reentry tachycardia, focal or automatic atrial tachycardia, and ventricular tachycardia. The surgical and transcatheter possibilities are numerous, and the congenital heart surgeon should have a comprehensive understanding of all arrhythmia types and potential methods of ablation. The purpose of this article is to introduce resternotomy techniques for safe mediastinal reentry and to review operative techniques of arrhythmia surgery in association with congenital heart disease.

Certain congenital heart anomalies make patients more susceptible to arrhythmia development throughout their lives. This poses the question whether prophylactic arrhythmia surgery should be incorporated into reparative open heart procedures for congenital heart disease. There is currently no consensus on what constitutes a standard prophylactic procedure, owing to the questions that remain regarding lesions to be performed; energy sources to use; proximity of energy source or incisions to coronary arteries, sinoatrial node, atrioventricular node; circumstances for right atrial, left atrial, or biatrial appendectomy; and whether to perform a right, left, or biatrial maze procedure. These considerations are important because prophylactic arrhythmia procedures are performed without knowing if the patient will actually develop an arrhythmia in his or her lifetime. By reviewing and summarizing the literature, congenital heart disease patients who are at risk for developing atrial arrhythmias can be identified and lesion sets can be suggested in an effort to standardize experimental protocols for prophylactic arrhythmia surgery.

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.

Pentalogy of Cantrell is a rare diagnosis consisting of several midline defects of the sternum, abdominal wall, diaphragm, pericardium, and heart. One of the known features is ventricular diverticulum that can represent a technical challenge surgically. This is a follow-up case report of the successful simultaneous resection of a biventricular diverticulum and omphalocele repair after previous report of repair of complex intracardiac disease in the same patient.

Plastic bronchitis is a rare, life-threatening condition characterized by the formation of mucofibrinous casts within the pulmonary bronchi. In patients with congenital heart disease, it is most frequently observed in single ventricular anatomies after Fontan palliation. The pathophysiology of plastic bronchitis remains unknown, and a consistently effective treatment strategy has yet to be identified. We report two cases of plastic bronchitis in patients with Fontan physiology. The first was treated with Fontan conversion and, despite encouraging short-term results, experienced recurrence of cast formation seven months postoperatively. The second underwent cardiac transplantation and has been free of bronchial casts for over one year. In addition, we explore the similarities between plastic bronchitis and protein-losing enteropathy, considering theories of their pathophysiologic mechanisms and reports of mutually effective treatment strategies. We propose that bronchial cast formation may result from the confluence of genetic makeup, inflammation, and the Fontan physiology and conclude that further investigation into therapies directed at these factors is merited.

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.

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.