Adult Congenital Heart Surgery: Adult or Pediatric Facility? Adult or Pediatric Surgeon?

Adult Congenital Heart Surgery: Adult or Pediatric Facility? Adult or Pediatric Surgeon? Brian E. Kogon, MD, Courtney Plattner, BA, Traci Leong, PhD, Paul M. Kirshbom, MD, Kirk R. Kanter, MD, Mike McConnell, MD, and Wendy Book, MD Divisions of Cardiothoracic Surgery and Cardiology, Emory University School of Medicine, Rollins School of Public Health, and Sibley Cardiology, Children s Healthcare of Atlanta, Atlanta, Georgia Background. One of the current controversies in the field of adult congenital heart disease is whether patients should be cared for at an adult or pediatric facility and by an adult or pediatric heart surgeon. After transitioning our program from the children s hospital to the adult hospital, we analyzed our experience with each system. Methods. Between 2000 and 2007, 303 operations were performed on adults (age > 18 years) with congenital heart disease. One hundred eighty-five operations were performed in an adult hospital and 118 in a pediatric hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon. Results. Mean age, coexisting medical problems, and preoperative risk factors were higher in both the adult hospital group and adult surgeon group compared with the respective pediatric groups. Mortality was similar at the adult and pediatric hospitals (4.3% versus 5.1%), but was markedly higher in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p 0.0008). By multivariate analysis, risk factors for mortality included older age at the time of surgery (p 0.028), surgery performed at a children s hospital (p 0.013), and surgery performed by an adult heart surgeon (p 0.0004). Conclusions. Congenital heart surgery can be performed in adults with reasonable morbidity and mortality. Caring for an anticipated aging adult congenital population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon. (Ann Thorac Surg 2009;87:833 40) 2009 by The Society of Thoracic Surgeons The increasing number of adult patients with congenital heart disease and the better survival of patients with complex disease into adulthood have increased the need for specific programs that are able to provide comprehensive care to these patients [1]. One of the current controversies in the field of adult congenital heart disease is whether these patients should be cared for at an adult or pediatric hospital and by an adult or pediatric heart surgeon. Clearly, there are advantages and disadvantages of each system. The obvious benefit of the pediatric system is the familiarity with the pathophysiology of the cardiac disease by all of the cardiac team members. The benefits of the adult system include patient comfort issues, along with ancillary and consult service issues. Caring for adults in a children s hospital often creates awkward or uncomfortable social situations. More importantly, however, as the adult congenital cardiac population ages, they likely will develop more complex medical histories requiring the proximity of multiple adult consult and ancillary services. In this scenario, adult programs are not always equipped to deal with the range and complexity Accepted for publication Dec 1, 2008. Address correspondence to Dr Kogon, Emory University, Children s Healthcare of Atlanta, Egleston, 1405 Clifton Rd, Atlanta, GA 30322; e-mail: Brian_kogon@emoryhealthcare.org. of adults with congenital heart disease, whereas pediatric programs cannot be expected to manage the many acquired adult diseases in a pediatric medical environment [1]. It is important to overcome the obstacles and provide these patients the best care as they transition into early adulthood and beyond. During the last 7 years, we have slowly transitioned our program from the children s hospital to the adult hospital. In an effort to continue to provide optimal care for this patient population in the future, we analyzed our experience with each system. Patients and Methods After obtaining institutional review board approval, we retrospectively reviewed the records of adult patients with congenital heart disease who underwent cardiothoracic surgery. Between 2000 and 2007, 303 operations were performed. Patient outcomes were analyzed based on hospital location and surgeon. A risk factor analysis was also performed for mortality. Patient Factors Overall, the mean age at the time of surgery was 31 13 years. Regarding sex distribution, there were 134 men and 169 women. Regarding race, there were 210 Caucasians and 66 African Americans. Sixteen patients were of 2009 by The Society of Thoracic Surgeons 0003-4975/09/$36.00 Published by Elsevier Inc doi:10.1016/j.athoracsur.2008.12.027

834 KOGON ET AL Ann Thorac Surg ADULT CONGENITAL HEART SURGERY 2009;87:833 40 Table 1. Initial Diagnoses Diagnosis Number of Patients Complex 147 Tetralogy of Fallot 96 Single ventricle 18 d-transposition of the great vessels 17 1-Transposition of the great vessels 6 Double-outlet right ventricle 6 Truncus arteriosus 4 Congenital valve disease 65 Pulmonary 27 Aortic 25 Tricuspid 10 Mitral 3 Septal defects 64 Atrial septal defect 35 Ventricular septal defect 16 Atrioventricular septal defect 13 Thoracic vascular 8 Coarctation of the aorta 4 Patent ductus arteriosus 1 Vascular ring 3 Arrhythmia/dysrhythmia 10 Other 9 Coronary anomaly 2 Cardiomyopathy 4 Tumor 2 Other 1 Total 303 other racial backgrounds and 11 were of unknown descent. Initial congenital diagnoses were categorized as follows: complex (n 147), congenital valve disease (n 65), septal defects (n 64), thoracic vascular (n 8), arrhythmias or dysrhythmias (n 10), and other (n 9; Table 1). Coexisting medical problems were present in 136 of 303 patients (45%), and are completely listed in Table 2. The Society of Thoracic Surgeons database defined preoperative risk factors for mortality were present in 68 of 303 patients (22%) and are listed in Table 3. Operative Factors One hundred eighty-five operations were performed at the adult hospital, and 118 were performed at the children s hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon. The surgeon was designated by the majority of his or her surgical practice. Operations were categorized by primary procedure as follows: valve repair or replacement (n 179), complex open procedure (n 17), septal defect (n 39), thoracic vascular (n 8), pacemaker or automatic implantable cardioverter-defibrillator (n 44), heart failure (n 10), and other (n 6; Table 4). Of the open heart operations, 190 of 249 (76%) were redo sternotomies. To facilitate comparison, each operation was assigned a score from 1 to 6 based on the risk-adjusted congenital heart surgery (RACH) system. This method was designed to adjust for baseline risk differences and allow meaningful comparisons of in-hospital mortality for patients undergoing surgery for congenital heart disease [2]. Pacemaker procedures were assigned a RACH score of 1. The RACH score distribution among hospital location and surgeon is shown in Table 5. Statistics In making comparisons between groups, statistical analyses were performed using the Mann-Whitney U test for continuous variables and 2 test for categorical variables. In evaluating potential risk factors for mortality, a multiple regression analysis was performed. Results Throughout the transition, both the number of cases performed annually increased (14 to 63) and the percentage of cases performed annually at the adult hospital gradually increased (7% to 78%). Comparisons Comparisons were made between those patients who underwent surgery at the adult hospital and those who underwent surgery at the pediatric hospital (Table 6). Mean age (37 versus 22 years; p 0.0001), presence of coexisting medical problems (62% versus 18%; p 0.0001), and presence of Society of Thoracic Surgeons preoperative risk factors (34% versus 4%; p 0.0001) were all higher in the adult hospital group compared with the pediatric hospital group. However, there was no difference in the RACH scores, and the mortality was similar between hospitals. Comparisons were also made between those patients who underwent surgery by an adult cardiac surgeon and a pediatric cardiac surgeon (Table 6). Mean age (42 versus 28 years; p 0.0001), presence of coexisting medical problems (72% versus 40%; p 0.0001), presence of Society of Thoracic Surgeons preoperative risk factors (39% versus 19%; p 0.006), and RACH score (2.38 versus 2.06; p 0.024) were all higher in the adult surgeon group compared with the pediatric surgeon group. There was also a marked increase in mortality in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p 0.0008). Mortality Overall mortality was 14 of 303 (4.6%). Details of the mortalities are provided in Table 7, and the relationship between RACH score and mortality is provided in Table 8. A multivariate risk factor analysis was performed for mortality ( 30 days; Table 9). Risk factors for mortality included older age at the time of surgery (p 0.028), surgery performed at a children s hospital (p 0.013), and surgery performed by an adult heart surgeon (p 0.0004). Mortality was unaffected by prior operations, the

Ann Thorac Surg KOGON ET AL 2009;87:833 40 ADULT CONGENITAL HEART SURGERY 835 Table 2. Coexisting Medical Problems Genetic (16) Neurologic (30) Down syndrome 11 Cerebrovascular accident 15 Noonan syndrome 2 Seizures 8 Nonspecified 2 Migraines 4 Turner syndrome 1 Mental retardation 3 Pulmonary (23) Psychiatric (32) Asthma 10 Depression, anxiety, bipolar 28 Sleep apnea 8 Attention deficit/hyperactivity disorder 2 Restrictive lung disease 3 Tourette syndrome 1 Pneumothorax 1 Substance abuse 1 Pulmonary embolus 1 Renal (5) Infection (4) Insufficiency 4 Pneumonia 2 Failure/dialysis 1 Sepsis 1 Hematologic (9) Endocarditis 1 Anemia 8 Endocrine (53) Coagulopathy 1 Dyslipidemia 18 Rheumatology/immunology (11) Hypothyroid 18 Autoimmune disease 11 Diabetes 11 Immunologic (1) Obesity 5 Hypogammaglobulinemia 1 Hyperthyroid 1 Gynecologic (4) Cardiovascular (51) Ovarian/breast cyst 2 Hypertension 33 Endometriosis 1 Atrial arrhythmias 8 Uterine fibroids 1 Peripheral vascular disease 4 Genitourinary (5) Tobacco 2 Benign prostatic hypertrophy 2 Postpartum cardiomyopathy 2 Recurrent urinary tract infections 1 Deep venous thrombosis 1 Cystitis 1 Pulmonary hypertension 1 Kidney stones 1 Gastrointestinal/liver (37) Orthopedic (4) Gastroesophageal reflux 20 Osteoporosis 2 Hepatitis 7 Kyphoscoliosis 2 Diverticulitis 3 Neoplastic (2) Peptic ulcer disease 2 Lymphoma 2 Liver insufficiency 2 Inflammatory bowel syndrome 2 Hirschsprung disease 1 presence of Society of Thoracic Surgeons database defined risk factors for mortality, and RACH score. Comment With improvements in technology, operative technique, and critical care medicine, more and more children with congenital heart disease are surviving into adulthood. It is now estimated that the number of adults with congenital heart disease in the United States is more than 800,000, and that, for the first time, this number equals the number of children with congenital heart disease [3, 4]. With the emergence of this ever-growing adult congenital population, controversy has arisen regarding its care. Should these patients be cared for at an adult or pediatric facility? Should these patients be cared for by an adult or pediatric cardiac surgeon? Bethesda Conference In 2001, the American College of Cardiology convened the 32nd Bethesda Conference to study the needs of these patients and to invite expert participants to recommend changes that will improve these patients care and access to the health-care system. The conference made recommendations regarding organization of care, workforce description and educational requirements, access to care, and special needs of adult patients with congenital heart disease (noncardiac surgery, reproductive issues, exercise and rehabilitation, and psychosocial issues) [4]. Although these recommendations were made for establishing a program, consensus was not established regarding the optimal setting. International and National Programs Canada, with its national health-care system, has established regional adult centers for congenital heart disease

836 KOGON ET AL Ann Thorac Surg ADULT CONGENITAL HEART SURGERY 2009;87:833 40 Table 3. Preoperative Society of Thoracic Surgery Database Defined Risk Factors for Mortality Hypertension 33 Chronic lung disease 21 Dyslipidemia 17 Cerebrovascular disease 14 Diabetes 12 Obesity 5 Renal insufficiency 3 Peripheral vascular disease 3 Current or recent tobacco use 2 Renal failure dialysis 1 Infectious endocarditis 1 Total 112 in 68 patients care. Although their system has grown to include 15 programs throughout the country, the mean reported surgical volumes (6 programs) are 33.8 cases per year, with only one program performing more than 75 cases per year [5 7]. The United Kingdom has also established adult centers for the grown-up congenital heart patient. Only 3 of 18 cardiac surgical centers performed more than 30 cases per year, and only 2 specialized units were fully equipped and staffed [8]. On the other hand, a Hungarian program has established an adult congenital heart program within a tertiary care facility of pediatric cardiology [9]. A recent multicenter analysis from Europe also suggests that the great majority of adult congenital heart disease cases are still being performed in pediatric centers [10]. In the United States, the Adult Congenital Heart Association clinic directory shows a total number of 57 programs. Of these programs, the mean number of operations performed per year is 44, with only 8 programs performing more than 75 cases per year [7]. These programs vary as to whether they function within a pediatric hospital or adult hospital. Information from a consortium of 37 freestanding children s hospitals showed 707 admissions for cardiac surgery in adults between July 2005 and June 2007. Median age at surgery was 26 years (range, 21 to 86 years). Adult cardiac surgery performed as a proportion of overall cardiac surgery at these pediatric hospitals varied from 0% to 11.1%. The majority of procedures were related to pacemaker or defibrillator implantation and semilunar valve surgery, rather than complex intracardiac repair or palliation. Comorbid conditions likely to require other subspecialty care was present in greater than 30% of patients [11]. Our Transition With the pediatric hospital being consistently at capacity and an adult congenital population growing in number and complexity, we have chosen to transition our adult congenital program to the adult hospital with surgery being performed by a congenital heart surgeon. Our rationale for the transition to the adult hospital is the following: 1. Presence of an enlarging complex population. Approximately 85% of children with significant congenital heart problems survive to adolescence, and it is predicted that admissions to specialized adult congenital heart disease units will increase in number and complexity [3, 8]. The profile of this patient population will also change with time, not only because of advancing age, but also with improved survival of patients with complex anomalies [4]. 2. Presence of concomitant adult cardiac disease. It has been suggested that a dedicated unit is ideally located, and administered, within the directorate of adult cardiology. Acquired heart disease will occur Table 4. Adult Congenital Heart Disease Major Procedure Procedure Number of Patients Valve surgery 179 Pulmonary valve 119 Aortic valve 26 Tricuspid valve 13 Mitral valve 11 Right ventricle-pulmonary artery conduit 10 Complex open heart 17 Biventricular heart Double outlet right ventricle 2 Double chamber right ventricle 3 Subvalvar left ventricular outflow tract 1 obstruction Pulmonary artery stenosis 1 Anomalous systemic venous drainage 1 Single ventricle heart Glenn 2 Fontan 1 Fontan conversion 6 Septal defects 39 Atrial septal defect 29 Ventricular septal defect 7 Atrioventricular septal defect 3 Thoracic vascular 8 Coarctation of the aorta 4 Patent ductus arteriosus 1 Vascular ring 3 Arrhythmia/dysrhythmia 44 Pacemaker implantation 15 Defibrillator 5 Pacemaker/defibrillator revision 24 Heart failure 10 Orthotopic heart transplantation 8 Ventricular assist device 2 Other 6 Coronary artery bypass 2 Pericardiectomy 2 Tumor excision 1 Other 1 Total 303

Ann Thorac Surg KOGON ET AL 2009;87:833 40 ADULT CONGENITAL HEART SURGERY 837 Table 5. Risk-Adjusted Congenital Heart Surgery Score Distribution Between Hospital and Surgeon Pediatric Hospital Adult Hospital RACH Score Pediatric Surgeon Adult Surgeon Pediatric Surgeon Adult Surgeon 1 48 0 23 6 77 2 17 0 75 15 107 3 44 0 35 20 99 4 3 0 0 1 4 5 0 0 0 0 0 6 0 0 0 0 0 Undesignated 6 0 6 4 16 Totals 118 0 139 46 303 Totals RACH risk-adjusted congenital heart surgery. more frequently as this population ages. Development of coronary arterial disease, or systemic hypertension, can cause new or worsening symptoms requiring treatment of both the congenital and acquired disorders. The combined congenital and acquired problems may require input from adult cardiologists [8]. 3. Presence of comorbidities. A high proportion of patients have important noncardiac medical and surgical problems requiring specialist advice. The presence of other subspecialties on the same site, therefore, is highly desirable. The most common needs appear for noncardiac surgery, endocrine, rheumatologic, hepatologic, neurologic, orthopedic, and renal expertise [8, 12]. Our rationale for having the surgeries performed by a pediatric heart surgeon include the following: 1. Complexity of surgery. The majority of patients will have had one or more palliative or corrective operations in childhood. Few are curative, and most require long-term follow up, and often further surgery. It is suggested that surgeons who operate on children with similar conditions should perform most adult congenital heart surgery. A study of national practice patterns for management of Table 6. Comparison Between the Pediatric and Adult Hospital and Pediatric and Adult Surgeon Pediatric Hospital (n 118) Adult Hospital (n 185) Variable No. of Patients Mean SD No. of Patients Mean SD p Value Patient factors Age at surgery (y) 22 6.6 37 12.9 0.0001 Coexisting medical problems 21 (18%) 115 (62%) 0.0001 STS database defined risk factors 5 (4.2%) 63 (34.1%) 0.0001 Previous operations 81 (69%) 143 (77%) 0.124 Operative factors RACH score 2.01 0.9 2.17 0.7 0.978 Outcomes Mortality 6 (5.1%) 8 (4.3%) 0.153 Pediatric Surgeon (n 257) Adult Surgeon (n 46) Variable No. of Patients Mean SD No. of Patients Mean SD p Value Patient factors Age at surgery (y) 28 11.4 42 14.6 0.0001 Coexisting medical problems 103 (40%) 33 (72%) 0.0001 STS database defined risk factors 50 (19%) 18 (39%) 0.006 Previous operations 189 (74%) 35 (76%) 0.857 Operative factors RACH score 2.06 0.8 2.38 0.7 0.024 Outcomes Mortality 7 (2.7%) 7 (15.2) 0.0008 RACH risk-adjusted congenital heart surgery; SD standard deviation; STS Society of Thoracic Surgeons.

Table 7. Mortality Summary Patient Age (y) Initial Congenital Diagnosis Procedure Surgeon Hospital RACH Score Medical History Complications 1 22 TOF/AVSD MVR, RV PA conduit P P 3 Down syndrome Respiratory failure (pneumonia), 18 sepsis 2 75 PA aneurysm PVR, 3-vessel CABG A A 2 DM, hyperlipidemia, HTN, Failure to wean from CPB 1 depression 3 51 VSD PVR A A 2 Ankylosing spondylitis, Hypotension, sepsis 3 GERD 4 43 TOF PVR, Bentall A A 3 AAA Difficulty weaning from CPB, 1 open chest, biventricular failure 5 36 TOF PVR P P 3 Sepsis 22 6 46 Ebstein anomaly Epicardial pacemaker A A 1 Ischemic bowel, cardiogenic 8 shock 7 25 PA/IVS Fontan conversion P P 3 Arrhythmia, cardiac arrest 4 8 58 TOF/AVSD MVR, Maze A A 3 Sleep apnea, HTN, Bilateral effusions thoracentesis 10 hyperlipidemia 9 45 CoA, AS Bentall A A 3 GERD, anemia, Biventricular failure 28 pneumonia 10 18 DCM BiVAD P P Liver insufficiency, arrhythmias, 29 ECMO 11 20 PA/IVS OHT P P Sudden death at home 18 12 63 TOF PVR, 3-vessel CABG A A 2 GERD, hypothyroid, HTN, anxiety Liver insufficiency, ARDS 10 13 43 TOF PVR, Glenn takedown P A 2 HTN, CVA, Increased liver enzymes Arrhythmias, cardiac arrest, renal failure 14 19 Single ventricle TVR P A 3 Air embolism, anoxic brain injury A adult; AAA abdominal aortic aneurysm; ARDS adult respiratory distress syndrome; AS aortic stenosis; AVSD atrioventricular septal defect; CABG coronary artery bypass graft; CoA coarctation of the aorta; CPB cardiopulmonary bypass; CVA cerebrovascular accident; DCM dilated cardiomyopathy; DM diabetes mellitus; ECMO extracorporeal membrane oxygenation; GERD gastroesophageal reflux disease; HTN hypertension; IVS intact ventricular septum; MVR mitral valve replacement; OHT orthotopic heart transplant; p pediatric; PA pulmonary artery; PVR pulmonary valve replacement; RACH risk-adjusted congenital heart surgery; RV right ventricle; TOF tetralogy of Fallot; TVR tricuspid valve replacement; VAD ventricular assist device; VSD ventricular septal defect. Days to Death 22 4 838 KOGON ET AL Ann Thorac Surg ADULT CONGENITAL HEART SURGERY 2009;87:833 40

Ann Thorac Surg KOGON ET AL 2009;87:833 40 ADULT CONGENITAL HEART SURGERY 839 adults with congenital heart disease has shown that in-hospital death rates for patients operated on by pediatric heart surgeons were lower than death rates for those operated on by nonpediatric heart surgeons [13]. Unique surgical problems exist in this group of patients, and surgeons who carry out operations on complex congenital heart disease need to be an integral part of these specialized units [8, 14]. 2. Reoperative surgery. In one US center following more than 1,800 patients, 1,243 of whom had cardiac surgery, almost 50% patients had two or more operations and 23% had three or more operations. The need for reoperation again emphasizes the need for special surgical expertise in congenital heart disease [4]. Table 8. Mortality Related to Risk-Adjusted Congenital Heart Surgery Score and Surgeon RACH Score Pediatric Surgeon Adult Surgeon Overall Mortality 1 0/71 1/6 1.20% 2 1/92 3/15 3.70% 3 4/79 3/20 7.10% 4 0/3 0/1 0% 5 0 0 0% 6 0 0 0% Undesignated 2/12 0/4 12.50% Totals 7/257 7/46 RACH risk-adjusted congenital heart surgery. Table 9. Risk Factor Analysis for Mortality Risk Factor Our Current Program The core structure of our adult congenital heart disease program consists of three pediatric heart surgeons (one primarily) and two cardiologists (one pediatric cardiologist and one adult cardiologist). With the exception of the pediatric cardiologist, all physician members of the team have admitting privileges at the adult hospital. For surgical patients, intraoperative transesophageal echocardiography is provided by the anesthesiologists. Postoperatively, the cardiac intensive care is provided jointly by the surgical team and an anesthesiology-based critical care team. The ward care is provided by the cardiac surgical service. For nonsurgical patients requiring admission, ward and intensive care is provided by the adult congenital cardiology service. We have established educational programs for the residents, fellows, nurses, and ancillary services to improve familiarity with the pathophysiology of the adult congenital heart disease patients. After overcoming this issue, we are now realizing all of the benefits of having these patients at the adult facility. Although there are no formal practice patterns that dictate the flow of patients, territorial issues do not seem to be problematic. As our program grows and gains more visibility within the hospital, the adult cardiologists are more consistently referring their adult congenital patients through the system, and the adult cardiac surgeons are more consistently deferring surgery to their congenital surgical partners. Referrals from outside cardiologists and cardiac surgeons are also steadily increasing. Consultant services are becoming increasingly interested and involved in these unique patients as well. Almost exclusively, our current practice is to perform all of the operations, including those that involve complex single-ventricle physiology, at the adult hospital with a congenital cardiac surgeon. Conclusions The interest in adult congenital heart disease is increasing along with the patient population. Although recommendations have been made for establishing an adult congenital heart disease program, each individual institution varies, and programs have developed at both adult and pediatric hospitals with both adult and pediatric surgeons. However, as this population grows, it is anticipated that it will increase in age and complexity. Caring for an anticipated aging adult congenital heart disease population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon. References No Mortality (n 289) Mortality (n 14) p Value Age at surgery (y) 31 12.6 40 17.6 0.028 RACH score 2.09 0.81 2.5 0.67 0.421 Prior operations Yes 212 12 0.147 No 77 2 STS database risk factors Yes 64 4 0.71 No 225 10 Surgery performed at a pediatric hospital Yes 112 6 0.013 No 177 8 Surgery performed by an adult heart surgeon Yes 39 7 0.0004 No 250 7 RACH risk-adjusted congenital heart surgery; Thoracic Surgeons. STS Society of 1. Calabro R, Sarubbi B, D Alto M, Russo MG. Organization of care for adults with congenital heart disease. G Ital Cardiol 2006;7:336 43. 2. Jenkins K, Gauvreau K, Newburger J, Spray T, Moller J, Iezzoni L. Consensus-based method for risk adjustment for surgery for congenital heart disease. J Thorac Cardiovasc Surg 2002;123:110 8.

840 KOGON ET AL Ann Thorac Surg ADULT CONGENITAL HEART SURGERY 2009;87:833 40 3. Gatzoulis M, Webb G, Daubeny P. Diagnosis and management of adult congenital heart disease. Edinburgh: Churchill Livingstone, 2003. 4. Webb G, Williams R. 32nd Bethesda Conference: care of the adult with congenital heart disease. J Am Coll Cardiol 2001;37:1161 98. 5. Canadian Adult Congenital Heart Network. Available at http://www.cachnet.org/. Last accessed July 1, 2008. 6. Rosenberg HC, Webb G. Referral of young patients with congenital heart disease to adult centres. The Canadian Adult Congenital Heart Disease Network. Can J Cardiol 1996;12:600 2. 7. Adult Congenital Heart Association. Available at http:// www.achaheart.org/for_members/clinicdirectory/index.php. Last accessed July 1, 2008. 8. Report of the British Cardiac Society Working Party. Grown-up congenital heart (guch) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK. Heart 2002;88(Suppl 1):i1 14. 9. Kiraly L, Temesvari A, Szekely A, et al. Adult congenital heart program in a tertiary care facility of pediatric cardiology. Orv Hetil 2005;146:209 14. 10. Vida VL, Berggren H, Brawn WJ, et al. Risk of surgery for congenital heart disease in the adult: a multicentered European study. Ann Thorac Surg 2007;83:161 8. 11. Mahle WT, Kirshbom PM, Kanter KR, Kogon BE. Cardiac surgery in adults performed at children s hospitals: trends and outcomes. J Thorac Cardiovasc Surg 2008;136:307 11. 12. Dearani JA, Connolly HM, Martinez R, Fontanet H, Webb GD. Caring for adults with congenital heart disease: successes and challenges for 2007 and beyond. Cardiol Young 2007;17(Suppl 2):87 96. 13. Karamlou T, Diggs BS, Person T, Ungerleider RM, Welke KF. National practice patters for management of adult congenital heart disease: operations by pediatric heart surgeons decrease in-hospital death. Circulation 2008; 118:2345 52. 14. Webb G. Care of adults with congenital heart disease a challenge for the new millennium. Thorac Cardiovasc Surg 2001;49:30 4. INVITED COMMENTARY Kogon and associates [1] have presented their experience with adult congenital heart patients who underwent operations at a pediatric hospital versus an adult hospital. They concluded that adult patients with congenital heart disease should have their care at an adult institution with operations performed by a congenital heart surgeon. A recent study by Karamlou and associates [2] confirmed similar trends using the Nationwide Inpatient Sample from 1988 to 2003 (152,277 patients). Institutions with separate pediatric and adult facilities have debated the optimal care dilemma for adults with congenital heart disease for many years. There is a curious approach-avoidance situation in regard to these patients. Adult practitioners, directors of residency training programs, nursing personnel, and administrators have disagreed with their pediatric counterparts and not always from the same perspective. Some adult program practitioners want to care for these patients, others are hesitant for many reasons owing to clinical competence, visionary commitment, and resident training; and the same is true for pediatric practitioners. Economic, practical, educational, and programmatic considerations come to the fore. Unfortunately, payer mix can play a major role in patient referral and institutional zeal in caring for these patients. Pediatric resident program directors argue that pediatric residents should not be responsible for adult patients. Pediatric nurses often say, The reason that I went into pediatric nursing was to take care for kids, not adults. On the other side of this conundrum is the fact that the congenital heart surgery team is usually based at a free-standing children s hospital. The operations that are performed on adult congenital heart patients are generally the same as those performed on children. Consequently, the operating team that is best suited to care for these patients is the pediatric heart surgery team. The obvious important issues are by whom, where, and under what circumstances do these patients obtain the best possible care? It seems that free-standing pediatric hospitals will be permanently challenged to perform adult congenital heart surgery for a variety of reasons owing to expert clinical care. The most that anyone could ask of the system is to perform congenital heart surgery on patients less than 25 to 30 years of age. After this age range, the problems of adult acquired diseases come into consideration. Kogon and associates [1] have had the foresight to understand these issues and propose that congenital heart surgeons perform the operations on adult patients with congenital heart disease in an adult facility with all the supportive care that might be required for agerelated disease entities. In the end, participating centers will determine what levels of care are necessary for the adult patient with congenital heart disease. So far, the published determinant of success is decreased mortality. In the future, it will be more important to document and compare mortality and morbidity as determining success factors. Constantine Mavroudis, MD Pediatric and Congenital Heart Surgery Cleveland Clinic Lerner College of Medicine Case Western Reserve University 9500 Euclid Ave Cleveland, OH 44195 e-mail: mavrouc@ccf.org References 1. Kogon BE, Plattner C, Leong T, et al. Adult congenital heart surgery: Adult or pediatric facility? Adult or pediatric surgeon? Ann Thorac Surg 2009;87:833 40. 2. Karamlou T, Diggs BS, Person T, Ungerleider RM, Welke KF. National Practice Patterns for Management of Adult Congenital Heart Disease. Operation by Pediatric Heart Surgeons Decreases In-Hospital Death. Circulation 2008;118:2345 52. 2009 by The Society of Thoracic Surgeons 0003-4975/09/$36.00 Published by Elsevier Inc doi:10.1016/j.athoracsur.2009.01.002