Adult Congenital Heart Disease

Canadian Journal of Cardiology 30 (2014) S410eS419 Review Adult Congenital Heart Disease: A Growing Epidemic Pablo Ávila, MD, Lise-Andree Mercier, MD, Annie Dore, MD, François Marcotte, MD, François-Pierre Mongeon, MD, MS, Reda Ibrahim, MD, Anita Asgar, MD, Joaquim Miro, MD, Gregor Andelﬁnger, MD, Blandine Mondesert, MD, Pierre de Guise, MD, Nancy Poirier, MD, and Paul Khairy, MD, PhD Montreal Heart Institute Adult Congenital Center, Universite de Montreal, Montreal, Quebec, Canada

ABSTRACT RESUM E Medical and surgical breakthroughs in the care of children born with Les avancees medicales et chirurgicales dans la prise en charge des heart defects have generated a growing population of adult survivors enfants nes avec des malformations cardiaques ont gen er e un nombre and spawned a new subspecialty of cardiology: adult congenital heart croissant de survivants adultes et ont donne naissance à une nouvelle disease. The prevalence of adult congenital heart disease is escalating sous-specialit e de la cardiologie : la cardiopathie congenitale chez at a rampant rate, outpacing the relatively static prevalence of pedi- l’adulte. La prevalence des adultes atteints de cardiopathie con- atric congenital heart disease, because adults now surpass children in genitale s’ampliﬁe à un rythme effren e, depassant la prevalence rel- numbers by a ratio of 2:1. As such, congenital heart disease can no ativement stable des cardiopathies congenitales pediatriques, puisque longer be considered primarily a pediatric specialty. Most congenital les adultes depassent maintenant en nombre les enfants avec un ratio heart defects are not curable and require lifelong specialized care. de 2 : 1. En tant que telle, la cardiopathie congenitale ne peut plus être Health care systems worldwide are challenged to meet the unique consider ee comme etant essentiellement une specialit ep ediatrique. needs of this increasingly complex patient population, including the La plupart des anomalies cardiaques congenitales ne sont pas cur- development of supraregional centres of excellence to provide ables et necessitent des soins specialis es à vie. Les systèmes de sante comprehensive and multidisciplinary specialized care. In this review, du monde entier sont amenes à repondre aux besoins particuliers de we explore the incidence and prevalence of congenital heart disease cette population de patients de plus en plus complexe, y compris par le and their changing patterns, address organization and delivery of care, developpement de centres d’excellence supraregionaux aﬁn de fournir highlight the importance of appropriate training and dedicated des soins specialis es d’ensemble et multidisciplinaires. Dans cette research, summarize the high burden of health care resource utiliza- revue, nous examinons l’incidence et la prevalence des cardiopathies tion, and provide an overview of common issues encountered in adults congenitales et leurs particularites changeantes, nous abordons l’or- with congenital heart disease. ganisation et la prestation des soins, mettons en evidence l’importance d’une formation appropriee et d’une recherche dedi ee, resumons la charge considerable de l’utilisation des ressources des soins de sante, et nous fournissons un aperçu des problèmes habituels rencontres chez les adultes atteints de cardiopathie congenitale.

Congenital heart malformations are the most common birth estimates suggest that the incidence of congenital heart disease defects and the leading cause of infant mortality.1 Although (excluding patent foramen ovale and bicuspid aortic valve) is several studies have attempted to quantify the incidence of in the order of 7 to 10 per 1000 live births, with moderate and congenital heart disease, generating accurate estimates is severe forms afflicting 3 per 1000 newborns.2-4 Considering complicated by nonstandardized definitions of qualifying pa- that there are approximately 138 million live births per year thologies, nonuniform screening methods, and geographic worldwide yields an estimated 1 million newborns with variability. Notwithstanding these limitations, recent congenital heart disease every year. These rates have remained relatively constant since 2011.5 Received for publication June 7, 2014. Accepted July 23, 2014. In developed countries, a shift toward a greater proportion Corresponding author: Dr Paul Khairy, Montreal Heart Institute Adult of simpler defects has been reported, which might reflect Congenital Center, Montreal Heart Institute, 5000 Belanger St E, Montreal, enhanced sensitivity in detecting milder forms of congenital Quebec H1T 1C8, Canada. Tel.: 1-514-376-3330 3800; fax: 1-514- heart disease combined with a reduction in the incidence of 593-2581. þ Â þ E-mail: [email protected] newborns with the most severe defects because of pregnancy 2 See page S417 for disclosure information. termination on prenatal detection.

spontaneously close within the first years of life and will not be present in adulthood, whereas other forms of congenital heart disease, such as atrial septal defects, Ebstein anomaly, and congenitally corrected transposition of the great arteries might go unrecognized until adulthood. Furthermore, prevalence estimates might not consider other factors that affect global disease epidemiology, such as migration and population mobility. A Quebec population-based study estimated that, in the year 2010, the prevalence of congenital heart disease in adults (18 years of age and older) was 6.1 per 1000.9 Similar rates were projected in a recent systematic review.14 Extrapolating this statistic to the general population, it might be estimated that there are > 100,000 adults with congenital heart disease in Canada, 1 million in the United States,15 and 1.8 million in Europe.16 It is also noteworthy that the prevalence of Figure 1. Distribution of age at death in patients with congenital heart complex congenital heart disease in adults has been steadily disease in 1987-1988 and 2004-2005. Histogram bars depict the increasing (eg, by 85% from 1985 to 2000), unlike the proportion of all deaths (x-axis) according to age at death (y-axis) in our relatively stable prevalence of complex congenital heart disease 13 cohort of patients with congenital heart disease in the first (1987- in children (Fig. 2). By 2010, adults accounted for 60% of 9 1988; left graph) and final (2004-2005; right graph) years of obser- patients with complex congenital heart disease. vation. Bold black curves with diamonds represent the corresponding age at death distribution in the general Quebec population during the 7 same periods of observation. Reproduced from Khairy et al. Organization and Delivery of Care Although the provision of care for children with congenital Prevalence of Congenital Heart Disease heart disease is well established in developed countries, clinical Most patients born with moderate or severe forms of services for adults are comparatively scarce.17-19 To attend to congenital heart disease in the 1950s and earlier died before the progressive increase in the number of adults with reaching adulthood, with the highest mortality during the first congenital heart disease and the increasing complexity of their week of life.6 Remarkable advances in diagnostic methods, pathologies, specialized adult congenital heart centres have medical management, interventional techniques, congenital emerged in several countries, with increasing volume of ac- heart disease surgery, and perioperative care have led to his- tivities (Fig. 3). torical shifts in population demographic characteristics, and Canada assumed a leadership role in establishing recom- adults now outnumber children with congenital heart defects mendations for optimal organization and delivery of care. In by a ratio of 2:1.7-9 Improved survival has been driven by a 1991, the Canadian Adult Congenital Heart (CACH) reduction in mortality in the youngest age strata, particularly network was established as the first national society dedicated in those with severe forms of congenital heart disease (Fig. 1).7 to the care of adults with congenital heart disease (www. From 1987 to 2005, a > 30% reduction in mortality was cachnet.ca). The Canadian Cardiovascular Society, in collab- reported among patients with congenital heart disease at large, oration with the CACH network, formed a panel of experts in with a 67% mortality reduction in children with complex 1996 to discuss the needs of adults with congenital heart disease.7 More than 85% of children born with heart defects disease and provide recommendations for referral to special- are now expected to survive to adulthood and continue to ized centres. The first management guidelines were presented thrive.10-12 Consequently, the population with congenital at the Canadian Cardiovascular Congress in Montreal in heart disease is aging. For example, the median age of patients 1996, published in the Canadian Journal of Cardiology in with severe defects was 11 years in 1985 compared with 17 1998,20 and subsequently updated in 200121 and 2009.22 years in 2000.13 In 2000, the median age of adults with Similar proposals emerged from the 32nd Bethesda Confer- congenital heart disease was 40 years, with a median age of 29 ence in 2000, which focused on the need for health care in those with severe defects.13 From 1987 to 2005, the me- professionals, patients, and governing agencies to develop dian age at death increased by 15 years, from 60 to 75 years, strategic plans to improve access to and delivery of care for the and the median age at death in those with severe defects adult with congenital heart disease.23 This call to action was increased from 2 to 23 years.7 followed by more specific recommendations from the Amer- In a manner similar to incidence, estimating the prevalence of ican College of Cardiology/American Heart Association and congenital heart disease is fraught with assumptions and subject the European Society of Cardiology (ESC).24,25 to limitations. Prevalence is defined as the number of living Current management guidelines suggest that approxi- subjects with a particular disease during a specific time frame. mately half of the adult population with congenital heart Birth prevalence underestimates the true burden of congenital disease stand to benefit from specialized care within adult heart disease by not capturing those in whom the diagnosis is congenital heart centres. Such care is generally recommended established afterward. Prevalence estimated at a later time ex- for the initial assessment of adults with known or suspected cludes those who died before the assessment, thereby differen- congenital heart disease, follow-up of patients with moderate tially underrepresenting higher-fatality lesions, and potentially and severe lesions, cardiac surgical and nonsurgical in- omitting defects that resolve spontaneously. For example, up to terventions, and risk assessment and support for pregnancy two-thirds of ventricular septal defects diagnosed at birth and noncardiac surgery.17,20,22,25-27 S412 Canadian Journal of Cardiology Volume 30 2014

Figure 2. Number and proportion of adults and children with (A) all forms of congenital heart disease (CHD), and (B) severe CHD in 1985, 1990, 1995, and 2000. Reproduced from Marelli et al.13

Recommended personnel and services for regional adult 2-10 million inhabitants,17,21 which translates to up to 17 congenital centres are summarized in Table 1.25 In short, specialized centres in Canada, 150 in the United States, and 365 centralized care within regional centres should be provided by a in Europe.29 This ﬁgure balances factors such as ease of access multidisciplinary team comprised of cardiologists with expertise and centralization of resources, considering that improved out- in adult congenital heart disease, including imaging, arrhythmia comes are proportional to volume of activity and experience.30 management,28 interventional cardiology, and high-risk ob- Although the recommended number of specialized centres has stetrics, congenital heart surgeons, perioperative care, and psy- been achieved in Canada (ie, 17 centres are registered within the chosocial support.17,25,26 Estimates regarding the ideal number CACH network), most countries in the developed world fall of regional adult congenital centres have varied between 1 per short of this target. For example, in the United States, 108

Figure 3. Number of patients followed at the Montreal Heart Institute Adult Congenital Centre over time. A > 7-fold increase in the number of adults with congenital heart disease was observed over a 15-year period. Ávila et al. S413 ACHD: A Growing Epidemic

Table 1. Personnel and services recommended for regional ACHD concluded that referral to such a centre is associated with a centres significant reduction in all-cause mortality (Fig. 4).36 Type of Service Personnel/Resources Cardiologist specializing in ACHD One or several 24/7 Training and Research Congenital cardiac surgeon Two or several 24/7 An important element in optimizing outcomes is the devel- Nurse/physician assistant/nurse practitioner One or several opment of training programs to meet workforce requirements Cardiac anaesthesiologist Several 24/7 and provide qualified, consistent, and comprehensive care.37 Echocardiography* Two or several 24/7 Includes TEE, intraoperative TEE Exposure of adult cardiology trainees to adult congenital heart Diagnostic catheterization* Yes, 24/7 disease didactic and clinical experience varies widely, reflecting Noncoronary interventional catheterization* Yes, 24/7 the fact that few programs offer advanced training in this sector of Electrophysiology/pacing/ICD implantation* One or several cardiology.38 Surveys suggest that only approximately 25% of Exercise testing Echocardiography cardiologists who care for adults with congenital heart disease Radionuclide 31,37 Cardiopulmonary have received formal training in this discipline. Metabolic The United States is the first country to officially recognize Cardiac imaging/radiology* Cardiac MRI the legitimacy of adult congenital heart disease as a separate CT scanning subspecialty of cardiology.39 In September 2012, the Amer- Nuclear medicine Multidisciplinary teams High-risk obstetrics ican Board of Medical Subspecialties approved a specialized Pulmonary hypertension certification examination via the American Board of Internal Heart failure/transplant Medicine. The first examination is planned for 2015. Pedi- Genetics atric or adult cardiologists require a minimum 2-year fellow- Neurology Nephrology ship within recognized adult congenital training centres to Cardiac pathology qualify. The ESC, with its working group on grown-up 40 Rehabilitation services congenital heart disease, is poised to follow suit. Canada is Social services currently contemplating recognizing adult congenital heart Vocational services disease as an area of focused competence via a Diploma of the Financial counsellors Information technology Database collection Royal College of Physicians and Surgeons of Canada Database support (DRCPSC). While details remain to be determined, a Quality assessment Diploma of the Royal College of Physicians and Surgeons of review/protocols Canada typically requires 1-2 years of additional training by 24/7, availability 24 hours per day, 7 days per week; ACHD, adult Royal College-accredited programs and is competency-based, congenital heart disease; CT, computed tomography; ICD, implantable car- with assessment through a summative portfolio. dioverter defibrillator; MRI, magnetic resonance imaging; TEE, trans- Another critically important element required to advance esophageal echocardiography. the care of adults with congenital heart disease is well-designed * These modalities must be supervised/performed and interpreted by research. Over the past decade, significant strides have been physicians with expertise and training in congenital heart disease. 25 achieved in coordinating research efforts and creating dedi- Reproduced from Warnes et al. cated infrastructures.41 A critical mass of patients and in- vestigators has enabled a new era of multicentre research with self-declared adult congenital heart centres with variable volumes more exacting standards. Several networks and organizations of activity and availability of resources are listed on the American have emerged, some focused on multicentre research as their Congenital Heart Association Web site (www.achaheart.org). main objective, with others providing a supportive environ- An adequate ratio of specialized adult congenital centres is ment to facilitate research. These include, but are not limited no guarantee for optimal care. A larger issue that plagues the to, the International Society for Adult Congenital Heart Dis- field of congenital heart disease is the relatively small pro- ease (www.isachd.org), CACH network, ESC (www.escardio. portion of qualifying patients who actually receive specialized org), Japanese Circulation Society (www.j-circ.or.jp/english), adult-oriented care. For example, a Canadian study found that Society of Thoracic Surgeons (www.sts.org), Congenital Heart < 25% of adults with congenital heart disease are followed by Surgeons’ Society (www.chss.org), and the Adult Congenital specialized centres and that waiting times to access services Heart Association. In the Netherlands, the Interuniversity exceed published recommendations.31 These sobering statis- Cardiology Institute and Heart Foundation developed a highly tics have prompted numerous educational initiatives to raise productive national registry and DNA bank of patients with awareness about the need for lifelong care and have incited congenital heart disease, named CONgenital CORvitia 42 research studies into factors associated with gaps in care and (CONCOR; www.concor.net/en). In North America, an impediments to long-term follow-up.32,33 Common barriers Alliance for Adult Research in Congenital Cardiology was to transfer from pediatric-to adult-oriented health care include founded in 2006 to foster collaborative research, advance inadequate knowledge regarding the need for such care, a knowledge, and improve outcomes (www.aarcc.net). dearth of accessible competent adult care providers, and emotional attachment of patients and families to their pedi- Health Care Resource Utilization atric caregivers and vice-versa.34,35 Lending further credence Despite surgical repair, most forms of congenital heart to the notion that specialized adult congenital centres with disease are associated with sequelae and long-term complica- multidisciplinary expertise offer the best prospects for tions, which engender a high level of health care resource improving outcomes, a recent population-based study utilization.19,43 Rates of emergency room visits, hospital S414 Canadian Journal of Cardiology Volume 30 2014

Figure 4. Time-series analysis: referral to specialized adult congenital heart disease (ACHD) centres and ACHD patient mortality. Observed specialized ACHD centre referral (black line) and ACHD mortality (grey line) per 1000 ACHD population per year, between 1990 and 2005. The dashed lines indicate expected trends after the change points identified by Poisson regression, and the black or grey lines represent the observed trends. Reproduced with permission from Mylotte et al.36 admissions, and cardiac interventions are several-fold greater volume and/or pressure overload, inability of a systemic than in the general population.44-47 Figure 5 depicts common morphologic right ventricle or single ventricle circulation causes of hospitalizations.46 Models of health-related costs and to adequately meet metabolic needs, unequal flow distri- outcomes in adults with congenital heart disease provide butions to lung fields, variable pulmonary vascular integ- economic justification for expensive interventions on the basis rity, and abnormalities of myocardial tissue architecture, of striking benefits on quality-adjusted life years.48 electrical conduction, and blood supply.61 As such, management guidelines for acquired heart disease cannot be Overview of Common Issues extrapolated to the complex forms of heart failure Common complications that contribute to the growing encountered in adults with congenital heart disease. epidemic of adults with congenital heart disease include ar- Despite the heterogeneity and imprecisions, on the whole, rhythmias, heart failure, pulmonary hypertension, endo- heart failure appears to be increasing in prevalence as the carditis, pregnancy-related issues, and cardiac interventions. population ages. It is among the most common causes of hospitalization (see Fig. 5),45,46,52 with some studies sug- Arrhythmias gesting that it has surpassed sudden cardiac death as the 8,51,62 Arrhythmias are the most common long-term complication in leading cause of mortality. The incidence of systolic adults with congenital heart disease, the leading source of systemic ventricular dysfunction is highest in those with cyanotic lesions, systemic right ventricles, and uni- morbidity and hospital admissions, and a major cause of 63 mortality.46,49-52 All forms of tachy- and bradyarrhythmias might ventricular hearts. In some forms of congenital heart 53 disease, diastolic systemic ventricular dysfunction has been be encountered. Figure 6 schematically summarizes factors 64 54 correlated with arrhythmias and mortality. Even in the predisposing to arrhythmias. The prevalence of tachyarrhyth- fi mias generally correlates with increasing age and complexity of absence of overt heart failure as classically de ned, some 28,55,56 degree of exercise intolerance is documented in up to one- the congenital heart lesion (Fig. 7). Sinus node dysfunc- 65 fi tion is highly prevalent in patients with surgery at or near the third. When contrasted against quanti ed exercise ca- pacity, functional symptoms are notoriously underreported superior vena cava to the right atrial junction; atrioventricular 66 block is common in those with displaced conduction systems and as a result of lifelong adaptation to a chronic disorder. 57-60 Exercise intolerance consistently portends poorer midterm in the acute postoperative setting. In 2014, the Pediatric and 66 Congenital Electrophysiology Society together with the Heart outcomes, such as hospitalizations and mortality. Rhythm Society proposed recommendations on the recognition Moreover, in an increasing number of adults with and management of arrhythmias in adults with congenital heart congenital heart disease and end-stage circulatory failure in disease, which were endorsed by the American College of Car- whom cardiac transplantation is not a viable option, end- of-life issues and palliative care assume a prominent diology, American Heart Association, European Heart Rhythm 67,68 Association, Canadian Heart Rhythm Society, and the Interna- role. tional Society for Adult Congenital Heart Disease.28 Pulmonary hypertension Heart failure Any nonrestrictive communication causing left-to-right Mechanisms for heart failure in adults with congenital shunting with increased pulmonary blood flow might pro- heart disease might involve circulatory overload, ventricular duce pathological changes to the pulmonary vascular bed, Ávila et al. S415 ACHD: A Growing Epidemic

Figure 5. Most frequent cardiovascular admission diagnoses/symptoms in the CONgenital CORvitia (CONCOR) Dutch national registry population from 2001 to 2005 (n 5798). All cardiovascular admissions with n 100 are displayed. Nos, not otherwise speciﬁed. Reproduced from Verheugt et al.46 ¼ ¼ including vasoconstriction, inﬂammation, cell proliferation, intracardiac shunts has resulted in a reduction in the preva- and vascular remodelling, resulting in increased pulmonary lence of Eisenmenger syndrome.78 Nevertheless, pulmonary vascular resistance.69,70 Pulmonary arterial hypertension is hypertension persists in 3%-13% of patients after septal defect prevalent in 4%-10% of adults with congenital heart disease closure71,79 and implies a worse prognosis.80 and is associated with exercise intolerance and heart fail- Endocarditis ure.69,71 At the extreme end of the spectrum, Eisenmenger syndrome is a multisystemic disorder associated with a 10- to The incidence of endocarditis (approximately 1.1 per 1000 12-fold increase in mortality in those with complex congenital patient-years) in adults with congenital heart disease is 20-fold heart disease.69,72-77 If managed appropriately, many such greater than in the general population,81,82 with a 12% patients survive past the third or fourth decade of life.72 In recurrence rate.81,83 Factors associated with endocarditis developed countries, earlier recognition and closure of include the type of congenital heart defect, presence of

Figure 6. Factors leading to arrhythmias in (A) before, and (B) after surgery for congenital heart disease. AV, atrioventricular; VT, ventricular tachycardia. Adapted from Escudero et al.54 S416 Canadian Journal of Cardiology Volume 30 2014

Figure 7. Risk estimates for arrhythmias according to type of congenital heart disease. Approximate risk estimates for atrial tachycardia (AT), atrial ﬁbrillation (AF), other supraventricular arrhythmias, ventricular arrhythmia, sinus node dysfunction (SND), atrioventricular (AV) block, and ventricular dyssynchrony are shown across various congenital heart defects (CHDs) of simple, moderate, and severe complexity. D- and L-transposition denote complete and congenitally corrected transposition of the great arteries, respectively. The colour-coded pattern ranges from minimal (no shading) to mild (light blue), moderate (medium blue), and high (dark blue) risk. Reproduced Khairy et al.28 multiple defects, and male sex.81 Systemic embolization, heart should be managed by a multidisciplinary team with appro- failure, and valvular regurgitation are common complica- priate expertise and delivery should take place in specialized tions.84 Surgery is required in up to one-third of patients.84,85 centres.25-27,91 Improvements in antibiotic therapy and earlier surgical management have resulted in lower rates of complications and Congenital cardiac surgeries and interventions mortality over the past decades, with current in-hospital 81,83,84 Surgical and catheter procedures are on the increase in mortality ranging from 4% to 9%. Educational coun- 92 selling, including hygienic measures and antibiotic prophylaxis adults with congenital heart disease. Percutaneous devices are increasingly used to close septal defects, abnormal vascular when indicated, should be incorporated in the care of adults ﬂ with congenital heart disease. connections, leaks across conduits and baf es, and pseudoa- neurysms; covered stents have enhanced the safety with which aortic coarctation, conduit stenosis, and branch pulmonary Pregnancy stenosis can be treated; and percutaneous valves have broad- When considered collectively, congenital malformations ened the range of therapeutic options in selected patients.93 are the most prevalent form of heart disease in pregnant Cardiovascular surgery is also increasingly performed in women86,87 and the leading cardiac cause of maternal adults with congenital heart disease to rectify residual or morbidity and mortality in developed countries.25 Pregnancy progressive lesions in the setting of previous reparative surgery, is the most common noncardiac cause of hospitalization in address previously unoperated disease, palliate nonreparable women with congenital heart disease.45,46 Congenital heart defects, and transplant failing hearts.94 Complications after disease carries increased maternal and fetal risks, particularly in surgery occur in 15%-25% of patients and include arrhyth- the setting of pulmonary arterial hypertension, subaortic or mias, low cardiac output, stroke, bleeds, and renal failure. subpulmonary ventricular dysfunction, left-sided valvular Perioperative mortality risk depends on numerous factors but obstruction, unrepaired aortic coarctation, or aortic root is generally within the 3%-8% range.95-98 dilatation in association with other anomalies such as a bicuspid aortic valve, Marfan, or Turner syndrome.86-89 Heart failure and arrhythmias are the most common maternal Conclusion complications, whereas frequent neonatal adverse outcomes Adult congenital heart disease is the most rapidly growing include small for gestational age birth weight, respiratory sector of cardiology today, with a patient population that is distress, and intraventricular cerebral hemorrhage.90 Women swiftly increasing in size and complexity. Unique issues, with moderate and complex forms of congenital heart disease challenges, and considerations are involved in managing adults Ávila et al. S417 ACHD: A Growing Epidemic with congenital heart disease, including arrhythmias, heart 14. van der Bom T, Bouma BJ, Meijboom FJ, Zwinderman AH, Mulder BJ. failure, pulmonary hypertension, endocarditis, pregnancy, The prevalence of adult congenital heart disease, results from a systematic interventions, and surgery. An increasing body of evidence review and evidence based calculation. Am Heart J 2012;164:568-75. suggests that outcomes can be improved by referral to 15. Hoffman JI, Kaplan S, Liberthson RR. Prevalence of congenital heart specialized centres with multidisciplinary teams dedicated to disease. Am Heart J 2004;147:425-39. clinical care, training, and research in adult congenital heart disease. 16. Moons P, Meijboom FJ, Baumgartner H, et al. Structure and activities of adult congenital heart disease programmes in Europe. Eur Heart J 2010;31:1305-10.

Funding Sources 17. Landzberg MJ, Murphy DJ Jr, Davidson WR Jr, et al. Task force 4: Dr Ávila received a research grant from the Fundación organization of delivery systems for adults with congenital heart disease. Alfonso Martín Escudero (Spain). Dr Khairy is supported by a J Am Coll Cardiol 2001;37:1187-93. Canada Research Chair in Adult Congenital Heart Disease 18. Skorton DJ, Garson A Jr, Allen HD, et al. Task force 5: adults with and Electrophysiology. congenital heart disease: access to care. J Am Coll Cardiol 2001;37: 1193-8.

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