Valvular Operations in Patients With Congenital Heart Disease: Increasing Rates From 1988 to 2005

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1 Valvular Operations in Patients With Congenital Heart Disease: Increasing Rates From 1988 to 2005 Raluca Ionescu-Ittu, MS, Andrew S. Mackie, MD, SM, Michal Abrahamowicz, PhD, Louise Pilote, MD, PhD, Christo Tchervenkov, MD, Giuseppe Martucci, MD, and Ariane J. Marelli, MD McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit), Divisions of Clinical Epidemiology, Cardiology, Internal Medicine, and Pediatric Cardiovascular Surgery, McGill University Health Centre, Montreal, Quebec, Stollery Children s Hospital, Edmonton, Alberta, Canada Background. The congenital heart disease population is aging. We hypothesized that changes in rates of congenital, valvular, and noncongenital surgical operations in congenital heart patients varied with age and disease severity over the last two decades. Methods. We performed time trend analysis using a Quebec congenital heart disease database constructed from administrative data. We included congenital heart patients of all ages having cardiac surgical operations. Heart lesions were classified as severe and other. Cardiac surgical operations were grouped as congenital, valvular (including aortic), and noncongenital (arrhythmia surgery, coronary artery bypass grafting, and cardiac transplants). An adapted Aristotle score was developed to classify procedures based on surgical risk. Yearly surgical rates were measured as surgical operations per 1,000 person-years and analyzed over time using Poisson regression models stratified by age, lesion severity, and cardiac surgery category. Results. From 1988 to 2005 we followed 71,979 patients for 1,009,430 person-years. We identified 17,444 cardiac surgical operations. There was a 31% increase in volumes and a 5% increase in surgical rates over time. In children, congenital surgical operations remained constant, accounting for 80% of all surgical operations. In adults, valvular operations were the most common type of surgical operations, increasing from 42% to 63% of all procedures over time. Rates of valvular operations increased significantly in all adult subgroups and in children with severe lesions. Conclusions. The need for valvular interventions has increased in the last two decades in congenital heart disease patients. These findings should be taken into account when allocating resources that will optimize outcomes for this growing population. (Ann Thorac Surg 2010;90:1563 9) 2010 by The Society of Thoracic Surgeons The number of adults and children with congenital heart disease (CHD) in the general population is increasing, with the highest increase being among adults with severe CHD and children with mild and moderate CHD [1]. Adults with CHD have significantly higher overall health services utilization than adults in the general population [2, 3]. These results are consistent with the clinical evolution of CHD patients who are at life-long risk for hemodynamic, myocardial, and arrhythmic complications [4, 5]. Cardiac surgical and percutaneous interventions are costly and rapidly evolving procedures. Few studies have investigated temporal trends in surgical volumes and changes in the nature of surgical interventions in the growing population of CHD patients. While many important studies have used systematically collected data on CHD surgical operations using customized databases or administrative data, the focus has largely been on the surgical outcomes [6 8]. A handful of center-specific studies in Europe have shown conflicting Accepted for publication July 6, Address correspondence to Dr Marelli, McGill University, McGill Adult Unit for Congenital Heart Disease, McGill University Health Centre, 687 Pine Ave W, Room H4-33, Montreal QC, H3A-1A1, Canada; ariane.marelli@mcgill.ca. results in terms of trends in surgical volumes, with an increase in some countries and a decrease in others [9 11]. We hypothesized that temporal changes in rates of congenital, valvular, and noncongenital surgical operations in CHD patients over the last two decades vary with age and type of CHD lesion. Using a population-based CHD database in Quebec, where access to health care is universal, our objectives were the following: (1) to describe the changes in CHD surgical operations volume in children and adults over the past 18 years; and (2) to investigate the relationship between changes in the cardiac surgical operations volumes and changes in the size and age-severity distribution of the CHD population. Material and Methods The study was approved by the McGill University Health Centre Ethics Board and the Quebec government agency responsible for privacy of access to information. The identities of the study participants were not available to the investigators and no informed consent was needed. Data Sources In Quebec, Canada s second-largest province, a unique health care number is assigned to all individuals at birth 2010 by The Society of Thoracic Surgeons /$36.00 Published by Elsevier Inc doi: /j.athoracsur

2 1564 IONESCU-ITTU ET AL Ann Thorac Surg CONGENITAL HEART DISEASE SURGICAL OPERATIONS 2010;90: and linked to all health services for the duration of a patient s life. Administrative databases include the physician s claims database of the Régie de l Assurance Maladie du Québec (RAMQ) from 1983 to 2005 and the hospital discharge database of Quebec (Med-Echo) from 1987 to The RAMQ database includes a separate file for the physician services delivered to infants during the period after birth when their application for a unique Medicare number is processed (temporary infant file). A province-wide, population-based CHD database was created at our institution by merging the province s two administration databases [1]. During this period, diagnostic codes adhered to the International Classification of Diseases, Ninth Revision (ICD-9). We used diagnostic and procedural codes for CHD in the RAMQ and Med- Echo databases to create the population-based Quebec CHD database that includes 71,513 CHD patients of all ages [1]. Data were linked at an individual level, based on the scrambled permanent Medicare number of the patient. The temporary infant file was used to estimate the number of cardiac surgical operations performed and the number of deaths (n 466) prior to subjects being assigned a permanent Medicare number. An algorithm was developed by our group to classify CHD lesions based on the 24 ICD-9 diagnostic codes for CHD [1]. Patients were identified with CHD if they had at least one diagnostic code for CHD and (or) a CHDspecific surgical procedure billed by selected specialists [1]. Severe CHD was defined based on anatomic diagnosis to include tetralogy of Fallot (TOF) and truncus arteriosus, endocardial cushion defects, transposition complex, univentricular heart, and hypoplastic left heart syndrome [1]. All other lesions were classified as other CHD lesions : atrial septal defect, ventricular septal defect, patent ductus arteriosus, aortic coarctation, Ebstein anomaly, anomalies of the pulmonary artery, congenital tricuspid, pulmonary, mitral, or aortic valve disease, anomalies of the great veins, and unspecified congenital anomalies of the heart and great vessels [1]. All information was cross-referenced between outpatient and inpatient data sources [1]. The resulting CHD database contains comprehensive demographic, diagnostic, and therapeutic records of all patient encounters with the health care system from 1983 to 2005 for all Quebec residents identified with CHD. Documentation of death is theoretically complete in this database, whether it occurred in or out of hospital. By law, the attestation of death is sent to the registrar of civil status who forwards this information to the Quebec Health Insurance body that administers the RAMQ database. Further validation of mortality data was performed at this level by cross-referencing vital status information provided by Quebec institutions that manage pensions and car insurance, such that a date of death was confirmed only if the two institutions agree on identity and vital status. Cardiac surgical operations were identified in the database based on Quebecspecific procedural codes for cardiac surgical operations billed by the cardiothoracic surgeons. Study Population We included CHD patients of all ages who had a cardiac surgical procedure recorded in the RAMQ database. All surgical operations and deaths identified in the temporary infant database were classified as severe CHD, assuming that surgery and (or) death in infancy is a marker of CHD severity. Babies with a patent ductus arteriosus diagnosis in the temporary infant database were excluded from analyses, given that an isolated patent ductus arteriosus diagnosis in infants is expected to reflect prematurity rather than the burden of CHD. Classification of Cardiac Surgical Operations We classified and ranked cardiac surgical operations in CHD patients into three surgical subtypes: (I) congenital operations; (II) valvular and aortic operations; and (III) noncongenital cardiac operations (Table 1). Congenital surgical operations included those operations that specifically target congenital lesions and can be linked to the underlying CHD diagnosis. Valvular and aortic operations were those performed in a patient with CHD having a native and (or) acquired valve lesion, at a time that did not coincide with a congenital surgical operation. For example, if a patient had a pulmonary valve intervention on the same day as a TOF repair, the patient was classified only as a TOF repair without counting the pulmonary valve intervention as a separate procedure. Valvular operations were categorized as left-sided, rightsided, or mixed. When multiple surgical procedures were performed on the same patient in the same day, we counted only one surgical operation for that patient (the one with the highest rank in Table 1), with the exception of arrhythmia operations (pacemaker-icd procedures and implantation, atrial or ventricular surgical ablation, and arrhythmia mapping), which were counted each time they were performed. Specific surgical operations within each subtype were classified into four complexity categories (Table 1, columns 2 to 5) based on an adaptation of the Aristotle complexity score [12]. When a single claims code in our database covered more than one procedure in the Aristotle classification, we assigned the mean score for all procedures in that category. For example, there is a single TOF repair code in the RAMQ database, while there are four TOF procedures in the Aristotle classification (TOF without ventriculotomy, TOF with ventriculotomy, and with or without transannular patch, and TOF with repair of absent pulmonary valve), with scores ranging from 7.5 to 11 (mean 8.5). We grouped all forms of TOF repairs into a single TOF repair category, having a mean Aristotle score of 8.5. Study Design We conducted a time-series analysis of CHD surgical volumes and rates in the CHD database of Quebec. For every calendar year from 1988 to 2005 we measured the size of the underlying CHD population and the total number of CHD surgical operations, overall and in subgroups defined by CHD severity ( severe and other ) and age (children 18

3 Ann Thorac Surg IONESCU-ITTU ET AL 2010;90: CONGENITAL HEART DISEASE SURGICAL OPERATIONS 1565 Table 1. Classification of Cardiac Surgical Operations Types in Congenital Heart Disease Patients Specific Cardiac Surgeries (Mean Aristotle Basic Score) Hierarchy of Cardiac Surgeries I. Congenital surgery Complexity Level 1 (Score 1.5 to 5.9) Shunt closure (4.5) Shunt take down (3.5) ASD closure (3) Patent ductus ligation (3) Complexity Level 2 (Score 6 to 7.9) VSD closure (7.8) Ruptured sinus of Valsalva repair (7.5) Coronary artery fistula repair (7.5) Cavopulmonary anastomosis (7) Systemic arterial to PA shunt (6.8) ASD/VSD creation/ enlargement (6.5) Blalock procedure (6.3) Pulmonary veins repair (6.2) Atrioventricular canal repair (6.1) PA banding and debanding (6) Coarctation repair (6) Vascular ring repair (6) Complexity Level 3 (Score 8 to 9.9) Fontan procedure (9.3) Aortic arch repair (8.9) Fallot repair (8.5) Left ventricle to aorta repair (8.3) Atrial switch (8.1) Transannular patch repair (8) Complexity Level 4 (Score 10 to 15) Norwood procedure (14.5) Konno/Ross-Konno procedure (11.5) Unifocalization (PA anastomosis) (11 ) Rastelli procedure (10.5) Arterial switch (10) Anomalous coronary correction (10) II. Valvular and aortic surgery (on valve with congenital or acquired defect) Right heart (6.6) Pulmonary artery repair Pulmonary valve surgery Tricuspid valve surgery Left heart (8.4) Aortic valve surgery Ross/ Konno procedure Bentall procedure Aortic root replacement Aortic aneurysm repair Aortic dissection repair Mitral valve surgery III. Noncongenital surgery Arrhythmia surgery (4.3) Pacemaker procedures ICD procedures Other Coronary artery bypass (7.5) Transplants/mechanical hearts (11.7) Heart (and lung) transplant Mechanical heart ASD atrial septal defect; ICD Implantable cardioverter defibrillator; PA pulmonary artery; VSD ventricular septal defect. and adults 18 years, including 18 to 35, 36 to 64, and 65 year olds). The size of the CHD population was measured as the number of CHD patients in a certain age-chd severity group alive in each calendar year. Because CHD is present from birth a patient was included in all calendar years he was alive, regardless of when the CHD diagnosis was made during his life. Our analyses of the complexity of cardiac operations were restricted to the period 1998 to 2005, corresponding to the time when the Aristotle score [12] was developed. Statistical Analysis Descriptive statistics on surgical volumes are reported yearly and for three periods: period 1 (1988 to 1993), period 2 (1994 to 1999), and period 3 (2000 to 2005). Yearly surgical volumes and relative frequencies of subtypes of surgical operations were calculated as the number of cardiac operations performed in the CHD population in that year and the proportion of that subtype relative to the total. The average operative volume and the size of the CHD population during each period were calculated as the mean of the yearly volumes and population, respectively in the period. Yearly operative rates were calculated per 1,000 person-years (PY) by indexing the surgical volumes to the CHD population alive in that year. Period surgical rates were calculated by indexing the total operative volume in the period to the total PY accumulated by the CHD population in the period. Poisson intercept-only models were used to estimate the 95% confidence intervals (CI) of the surgical rates. Poisson bivariate models were used to estimate the change in surgical rates from period 1 to period 3 stratified by age, CHD severity, and surgical subtype. Each bivariate Poisson model used the group-specific surgical rates as the outcome and the time period as the exposure, with period 1 being the reference category. From this analysis we report rate ratios and 95% CI. Analyses were performed using SAS statistical software (version 8.02; SAS, Cary, NC). Results From 1988 to 2005 a total of 71,979 CHD patients were followed for 1,009,430 PY and had 17,444 cardiac surgical operations (17.5 operations per 1,000 PY, 95% CI 17.2 to

4 1566 IONESCU-ITTU ET AL Ann Thorac Surg CONGENITAL HEART DISEASE SURGICAL OPERATIONS 2010;90: ). Overall, the cardiac surgical volumes increased from an average of 836 per year in period 1 to 1,095 per year in period 3 (31% increase). The size of the CHD population has also increased, from an average 49,873 subjects in period 1 to 62,084 subjects in period 3 (24% increase). When surgical volumes were indexed to the size of the CHD population, this corresponded to a 5% increase in surgical rates, from 16.8 (16.3 to 17.2) in period 1 to 17.6 (17.2 to 18.1) per 1,000 PY in period 3. Temporal Trends in Surgical Volumes in Children and Adults Figure 1 compares the surgical volumes in the three periods in children and adults, by type of surgery. The cardiac operative volumes decreased slightly after period 2 in children (from 2,293 in period 1 to 2,111 in period 3), and increased significantly in adults (from 2,726 operations in period 1 to 4,461 in period 3). The increase in operative volumes in adults was mostly accounted for by an increase in the volumes of valvular and aortic operations, which more than doubled from period 1 to period 3(Fig 1). Indeed, the relative frequency of valvular and aortic operations in adults has increased from 42.1% of all cardiac surgical operations in 1988 to 63.2% in 2005 (Fig 2A). In contrast, in all periods, the majority of operations in children ( 80%) were congenital (Figs 1 and 2B). Complexity of CHD Surgical Operations in Children and Adults Children accounted for most congenital surgical operations performed from 1998 to 2005, with the largest number of congenital operations corresponding to level 2 complexity (Fig 3A). Indeed, level 2 congenital operations included repairs for CHD defects that are very frequent in children, such as VSDs and aortic coarctation (Table 1). The other three complexity level groups were relatively evenly distributed in children. In contrast, very few congenital operations in adults were of high level 4 complexity (Fig 3A). The most frequent type of valvular, aortic, and noncongenital surgical operations (Fig 3B) were of complexity level 3, in adults. This group includes the left-heart valvular and aortic operations. Fig 2. Temporal trends in the relative frequency of cardiac surgery subtypes in adults (A) and children (B). Temporal Trends in Rates of Cardiac Surgical Operations in Adults When surgical volumes were indexed to the size of the adult CHD population, the resulting surgical rates were 4500 Congenital surgeries Valvular and aortic surgeries Other cardiac surgeries N = 4,461 Cardiac surgery volumes N = 3,652 1,149 1,055 N = 2,726 N = 2,293 N = 2, N = 2, ,759 1, ,234 1,971 2,001 1, Children Adults Children Adults Children Adults Period 1 ( ) Period 2 ( ) Period 3 ( ) Fig 1. Surgical operations volumes in children and adults in three periods: 1988 to 1993 (period 1), 1994 to 1999 (period 2), and 2000 to 2005 (period 3). Fig 3. Complexity of congenital (A), valvular and aortic, and noncongenital (B) surgical operations in children and adults from 1998 to 2005.

5 Ann Thorac Surg IONESCU-ITTU ET AL 2010;90: CONGENITAL HEART DISEASE SURGICAL OPERATIONS 1567 relatively constant for congenital and noncongenital operations but increased sharply for valvular and aortic operations; from 4.9 per 1,000 PY (4.2 to 5.8) in 1988 to 10.7 per 1,000 PY (9.7 to 11.8) in 2005 (Fig 4). The rates of congenital and noncongenital surgical operations were lower than those of valvular and aortic surgical operations in all years. When broken down by age groups, the rates of valvular and aortic surgical operations increased from period 1 to period 3 in all age groups: from 1.5 to 1.9 per 1,000 PY in 18 to 35 year olds; from 7.2 to 13.7 per 1,000 in 36 to 64 year olds; and from 13.2 to 27.2 per 1,000 in ages 65 and over. Figure 5 presents the changes in the operative rates from period 1 to period 3, in subgroups defined by age, CHD severity and surgery subtype. The largest change in surgical rates observed in the last 18 years was an increase in the rates of valvular and aortic surgical operations in almost all age-chd severity groups (rate ratio 1.82, 95% CI 1.32 to 2.50 in children with severe CHD; RR 2.72, 1.50 to 4.94 in adults with severe CHD; and RR 1.93, 1.80 to 2.06 in adults with other CHD), concomitant with a decrease in the congenital surgical operations rates in all groups except children with severe CHD (RR 0.58, 0.52 to 0.65 in children with other CHD; RR 0.61, 0.44 to 0.85 in adults with severe CHD; and RR 0.72, 0.64 to 0.82 in adults with other CHD). There was also an increase in the rates of noncongenital operations in children with severe CHD (RR 1.73, 1.37 to 2.18) and adults with other CHD (RR 1.12, 1.02 to 1.23), but these were less impressive than the changes in the other two surgery subgroups. Comment To our knowledge this is the only study analyzing temporal trends in subtypes of cardiac surgery in a population-based sample of children and adults with CHD. From 1988 to 2005 a total of 71,979 CHD patients were followed for 1,009,430 PY and had 17,444 cardiac surgical operations. Congenital operations remained, in the last 18 years, the most common form of cardiac surgery in children and, in all years, accounted for about 80% of all cardiac operations in children. In contrast, valvular and aortic surgery was the most common form of cardiac Fig 4. Temporal trends in the rates of cardiac (congenital, valvular and aortic, and noncongenital) surgical operations in adults. Fig 5. Stratified analysis of change in surgical rates from 1988 to 1993 (period 1) and 2000 to 2005 (period 3) by age group, severity of congenital heart disease (CHD), and surgery subtype. RR (represent rate) ratios comparing the late period 2000 to 2005 versus the early period 1988 to 1993 in subgroups defined above when RR is less than 1, the rate of surgical operations decreased from the early to the late period; when RR is greater than 1 the rate of surgical operations increased from the early to the late period. A confidence interval (CI) that crosses 1 is not statistically significant at p less than surgery in adults. Overall, there was a 31% increase in cardiac surgical volumes and a 5% overall increase in cardiac surgical rates in the CHD population over the observation period. The increase was largely accounted for by an increase in valvular and aortic surgical operations in adults with both severe and other CHD and in children with severe CHD. The increase occurred despite a slight decrease in the operative rates for congenital surgery in adults and children with other CHD. Interestingly, the congenital operative rates remained constant over time in children with severe CHD. Studies on cardiac surgical trends in the CHD population are scant. An increase in the volume of congenital heart operations has been reported from 1947 to 1997 in a population-based study of 538 CHD patients of all ages [9], but the surgical volumes were not indexed to a CHD population denominator. One study that reports surgical rates, in addition to surgical volumes, is a populationbased study performed in one center that services the Copenhagen area [13]. The study linked 225 congenital and valvular operations to an approximate 4,000 ACHD (adult congenital heart disease) patients followed in the center from 1998 to 2008, yielding an estimated rate of 8 operations per 1,000 patients, a rate that is consistent with our rates of 4.6 per1,000 and 13.8 per1,000 for congenital, valvular, and aortic surgery, respectively. The overall increase in the rate of cardiac operations observed in our study was mainly accounted for by an increase in the valvular and aortic surgical operations in the ACHD patients. To our knowledge, the only other study that documented temporal trends in valvular surgery in CHD

6 1568 IONESCU-ITTU ET AL Ann Thorac Surg CONGENITAL HEART DISEASE SURGICAL OPERATIONS 2010;90: patients is a case-series of 54 patients with aortic valve replacement operated at the Toronto General Hospital in Canada [14]. This study also showed an increase in this type of surgery from 1976 to 1999 [14]. Although there is an obvious lack of data on changes in valvular surgery in CHD patients, the results are not surprising, as a similar increase in valvular and aortic surgical operations has been observed in non-chd populations [15, 16]. In contrast, we observed a decrease in the congenital operations in children with other CHD and in adults. Such changes in the volumes and rates of congenital and valvular surgical operations could be related to changes in the clinical characteristics of the underlying populations and (or) changes in the clinical practice. The increasing trends persisted after indexing the surgical volumes to the size of the CHD population, suggesting that the changing demographics of the CHD population reflect a change in the complexity of patients with CHD. Indeed, as CHD patients survive longer, they potentially face new and more complex comorbidities. One important change in clinical practice that could affect the trends is the advent of percutaneous interventions. In children, percutaneous interventions have partially replaced complexity level 2 operations such as shunt lesions and coarctation [17 19], while in adults they replaced valvular operations. We hypothesize that in children the need for congenital intervention did not change over time; therefore the decrease in congenital operations for children with other CHD is due to the increase in percutaneous interventions for shunt lesions and coarctation. However, for adults we hypothesize that the need for valvular interventions has increased over time, translating both into an increase in valvular surgical operations and an increase in valvular percutaneous interventions. In this study we observe only the trends in valvular surgical operations and, for that reason, the interpretation of the need for valvular interventions in the CHD population should be made with caution. Common valve operations in the CHD population include bicuspid aortic valves [20], thus it is possible that the increase in valvular operations is mainly for aortic valve surgery. Indeed, Figure 3 shows that most valvular, aortic, and noncongenital surgical operations in adults were in the complexity level 3 group that includes left-sided valvular surgery, supporting this hypothesis. The proportion of patients with TOF undergoing pulmonary valve replacement is also rising and likely contributes to the observed increase, although the absolute numbers remain relatively small [21, 22]. In terms of the decrease we observed in the rates of congenital surgery among adults with CHD, another factor that could affect the rates, in addition to percutaneous interventions, is the increase in complete repair of CHD lesions in infancy and childhood, a trend that became well established beginning in the late 1980s [8, 23]. The strength of our study stems from our provincewide population-based sample of CHD patients with long-term follow-up. As a population-based study in a province with universal health coverage, there are no concerns about the representativeness of the study sample. Our study also had several limitations. First, we relied on administrative databases, which sometimes lack specificity in the coding of diagnoses. For instance, the ICD-9 diagnostic classification does not include a specific code for bicuspid aortic valve, so patients with bicuspid aortic valve are coded either with congenital aortic stenosis, congenital aortic regurgitation, or a code for unspecified anomalies of the heart. For this reason, we could not distinguish between native, residual, or acquired aortic valve disease in a CHD patient. Given that the same is true for all valvular pathology, to facilitate the interpretation of the results we opted to create a separate category for the valvular and aortic surgical operations, distinct from the pure congenital surgery category. Second, our database covers only the period of 1983 to 2005 from the lifetime of the patients, and thus we cannot distinguish in all patients which surgical operations are first operations and which are reoperations. Therefore, especially in adults, we cannot specify what proportion of the increase is accounted for by a change in the rate of reoperations. However, we did not seek to identify the causes of the observed temporal changes in the cardiac surgical volumes and rates in the CHD population. Rather, our purpose was to analyze population-based trends in cardiac surgical volumes among CHD patients of all ages, using concurrently measured prevalent CHD cases in the general population as the denominator. Finally, the adaptation of the Aristotle complexity score to our database is less granular than the one described by Jacobs and colleagues [12] to account for differences in surgical risk based on case mix. As a result, we have a limited ability to distinguish within-lesion complexity. Notwithstanding this limitation, we believe that our classification still gives a fair picture of how the surgical volumes are distributed by complexity. Indeed, our findings are comparable in terms of distribution of Aristotle complexity in children with CHD with those reported in larger and more comprehensive databases. Specifically, the most frequent congenital surgical operations in children in our study were of complexity level 2, corresponding to a mean Aristotle score of 6 to 7.9, similar to the mean Aristotle score of 6.7 reported in the databases of the Society of Thoracic Surgeons and the European Association of Cardiothoracic Surgery for over 14,000 procedures in children [24]. The number of valvular and aortic surgical operations has increased in the last two decades, despite the fact that this period coincided with an increase in the interventional cardiac catheterization as a nonsurgical alternative to valvular surgery operations [25]. The observed increase in surgical volumes and rates for valvular and aortic surgery in adults likely reflects the changing demographics and case mix of patients aging with congenital heart disease. Understanding the changes in the demographics and the complexity of this growing population of adults with CHD is imperative to future planning for, and funding of, the care of these individuals. We need to prepare for this population of patients which appears to be at increasing lifelong risk for future surgery that is likely to be valvular. Studies examining the impact

7 Ann Thorac Surg IONESCU-ITTU ET AL 2010;90: CONGENITAL HEART DISEASE SURGICAL OPERATIONS 1569 of resource allocation for life-long diseases such as CHD may become increasingly important. Dr Mackie was supported by the Fonds de la Recherche en Santé du Québec. Drs Abrahamowicz and Pilote are supported by the James McGill Research Chair. Dr Abrahamowicz is supported by the Canadian Institutes of Health Research and the Natural Sciences and Engineering Council of Canada. Dr Pilote is also supported by the Fonds de la Recherche en Santé du Québec. Dr Marelli is supported by the Heart and Stroke Foundation of Canada and Quebec. References 1. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007;115: Mackie AS, Pilote L, Ionescu-Ittu R, Rahme E, Marelli AJ. Health care resource utilization in adults with congenital heart disease. Am J Cardiol 2007;99: Moons P, Siebens K, De Geest S, Abraham I, Budts W, Gewillig M. A pilot study of expenditures on, and utilization of resources in, health care in adults with congenital heart disease. Cardiol Young 2001;11: Boneva RS, Botto LD, Moore CA, Yang Q, Correa A, Erickson JD. Mortality associated with congenital heart defects in the United States: trends and racial disparities, Circulation 2001;103: Bouchardy J, Therrien J, Pilote L, et al. Atrial arrhythmias in adults with congenital heart disease. Circulation 2009;120: Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect. Ann Thorac Surg 2000;69(4 suppl): S Jenkins KJ, Gauvreau K. Center-specific differences in mortality: preliminary analyses using the Risk Adjustment in Congenital Heart Surgery (RACHS-1) method. J Thorac Cardiovasc Surg 2002;124: Tchervenkov CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia ventricular septal defect. Ann Thorac Surg 2000;69(4 suppl):s Grech V, Elliott MJ. Evolution of surgical trends in congenital heart disease: a population based study. Int J Cardiol 1998;66: Monro JL. Surgery for congenital heart disease in Europe Eur J Cardiothorac Surg 1998;13: Unger F. Open heart surgery in Europe Eur J Cardiothorac Surg 1996;10: Jacobs JP, Mavroudis C, Jacobs ML, et al. Lessons learned from the data analysis of the second harvest ( ) of the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. Eur J Cardiothorac Surg 2004;26: Klcovansky J, Sondergaard L, Helvind M, Andersen HO. Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase? Interact Cardiovasc Thorac Surg 2008;7: Rao V, Van Arsdell GS, David TE, Azakie A, Williams WG. Aortic valve repair for adult congenital heart disease: A 22-year experience. Circulation 2000;102(19 suppl 3):III Nowicki ER, Weintraub RW, Birkmeyer NJ, et al. Mitral valve repair and replacement in northern New England. Am Heart J 2003;145: Northrup WF 3rd, Dubois KA, Kshettry VR, Teskey JM, Nicoloff DM. Trends in aortic valve surgery in a large multi-surgeon, multi-hospital practice, J Heart Valve Dis 2002;11: Coats L, Tsang V, Khambadkone S, et al. The potential impact of percutaneous pulmonary valve stent implantation on right ventricular outflow tract re-intervention. Eur J Cardiothorac Surg 2005;27: Fawzy ME, Awad M, Hassan W, Al Kadhi Y, Shoukri M, Fadley F. Long-term outcome (up to 15 years) of balloon angioplasty of discrete native coarctation of the aorta in adolescents and adults. J Am Coll Cardiol 2004;43: Hein R, Büscheck F, Fischer E, et al. Atrial and ventricular septal defects can safely be closed by percutaneous intervention. J Interv Cardiol 2005;18: Warnes CA, Liberthson R, Danielson GK, et al. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001;37: Oechslin EN, Harrison DA, Harris L, et al. Reoperation in adults with repair of tetralogy of Fallot: indications and outcomes. J Thorac Cardiovasc Surg 1999;118: Yetman AT, Lee KJ, Hamilton R, Morrow WR, McCrindle BW. Exercise capacity after repair of Tetralogy of Fallot in infancy. Am J Cardiol 2001;87:1021 3; A Kirklin JW, Blackstone EH, Tchervenkov CI, Castaneda AR. Clinical outcomes after the arterial switch operation for transposition. Patient, support, procedural, and institutional risk factors. Congenital Heart Surgeons Society. Circulation 1992;86: Lacour-Gayet F, Clarke D, Jacobs J, et al. The Aristotle score: a complexity-adjusted method to evaluate surgical results. Eur J Cardiothorac Surg 2004;25: Inglessis I, Landzberg MJ. Interventional catheterization in adult congenital heart disease. Circulation 2007;115:

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