Historical Prospective: Therapeutic Catheterization Procedures in Congenital Heart Disease

The development of pediatric and congenital therapeutic ("interventional") catheterization procedures is traced from the earliest attempts to the current procedures. Therapeutic cardiac catheterization procedures for pediatric and congenital heart disease have grown from a dream to a reality during only the last three decades. Although some of the technology has come from the adult interventions and has paralleled the development of adult catheter interventions, most of the therapeutic procedures for congenital lesions were developed by a dedicated and persistent group of pediatric cardiologists and originated in the pediatric cardiac catheterization laboratories. Many of the common congenital lesions, which three decades ago required cardiac surgery for treatment, now, as standard accepted therapy, are managed in the catheterization laboratory. The most successful advances have been in the opening of stenotic valves, opening and fixing stenotic vessels with intravascular stents, and, at the opposite extreme, the closure of PDAs, secundum ASDs, some VSDs, and numerous miscellaneous abnormal communications. Although many of these therapies are now the standard of care, all of the catheter therapies in pediatric and congenital lesions are in the infancy of their development and will undergo many improvements and refinements in the future.     

Catheter intervention for adult patients with congenital heart disease

Adult congenital heart disease is one of the most important clinical issues not only for pediatric cardiologists but also adult cardiologists. After the introduction of catheter intervention for atrial septal defect in the pediatric population, therapeutic advantages of this less invasive procedure now focused on even geriatric patients. The most valuable clinical benefit of this procedure is the significant improvement in symptoms and daily activities, which result from the closure of left to right shunt without thoracotomy or cardiopulmonary bypass surgery. Although currently available therapeutic options for device closure for congenital heart disease in Japan are limited to atrial septal defect, patent ductus arteriosus, or some vascular abnormalities such as coronary arteriovenous fistula, various new techniques or devices such as ventricular septal defect device, pulmonary valve implantation, are going to be introduced in the near future. To perform safely and achieve good procedure success, real time imaging plays an important role in interventional procedures. Real time three-dimensional transesophageal echocardiography can provide high quality imaging for anatomical evaluation including defect size, surrounding rim morphology, and the relationship between device and septal rim. In adult patients, optimal management of comorbidities is an important issue, including cardiac function, arrhythmias, pulmonary function, and renal function. In particular, atrial arrhythmias are key issues for long-term outcome. Because the interventional procedures are not complication-free techniques, the establishment of a surgical back-up system is essential for achieving a safe procedure. Finally, the establishment of a team approach including pediatric and adult cardiologists, cardiac surgeons, and anesthesiologists is the most important factor for a good therapeutic outcome. Their roles include pre-interventional hemodynamic evaluation, good imaging technique for anatomical evaluation, management of comorbidities, and surgical back up.     

Outcomes of hospitalization in adults in the United States with atrial septal defect, ventricular septal defect, and atrioventricular septal defect

Atrial septal defect, ventricular septal defect (VSD), and atrioventricular septal defect (AVSD) are among the most common congenital heart lesions, with most children surviving to adulthood. However, the clinical course of these patients is largely unknown, particularly pertaining to inpatient care. The purpose of this study was to assess hospitalizations for septal defects in adults with congenital heart disease (CHD) and risk factors associated with significant morbidity and mortality. The 2007 Nationwide Inpatient Sample was used to assess national prevalence of hospitalizations in adults with CHD with septal defects. Co-morbidities and risk factors for mortality were also determined. There were 84,308 adult CHD admissions in the United States in 2007. Fifty-four percent of adult CHD admissions had diagnoses of septal defects, with 48% having atrial septal defect, 7% having VSD, and 0.4% having AVSD. Overall in-hospital mortality was 2.1%. Common co-morbidities included arrhythmias (31%), heart failure (20%), and diabetes mellitus (18%). On multivariable analysis, independent risk factors for mortality included presence of VSD (odds ratio 3.1, 95% confidence interval [CI] 1.5 to 6.5), trisomy 21 (odds ratio 2.9, 95% CI 1.1 to 7.5), and pulmonary hypertension (odds ratio 1.5, 95% CI 1.0 to 2.4). In conclusion, this study of hospitalizations in adults with septal defects found that admissions are common and associated with significant co-morbidities. Overall mortality is low but is increased in patients with VSD. Cardiac and noncardiac co-morbidities are commonly encountered. Many noncardiac conditions, including trisomy 21 and the youngest and oldest groups, are associated with an increased risk of death.     

Update on the Use and Outcomes of Implantable Cardioverter Defibrillators in Pediatric Patients

OPINION STATEMENT: The vast majority of implantable cardioverter defibrillators (ICDs) continue to be implanted in the adult population. Accordingly, manufacturers develop devices and leads primarily for the adult population. Whilst the number of ICDs implanted in children is small in comparison, the potential benefits are large to this group. It is a common frustration among pediatric cardiologists whom implant devices that impressive technological developments continue to be developed for the adult population; as the population of children with ICDs is small, robust clinical studies often lag behind. By necessity, pediatric cardiologists and cardiothoracic surgeons have developed innovative techniques utilizing adult components in unusual configurations for children with complex congenital heart disease. As in the adult population, inappropriate shocks are one of the most limiting and concerning complications in the use of ICDs. Unfortunately, as will be discussed below, children are at increased risk of inappropriate shocks when compared with adults. The true impact of inappropriate shocks is increasingly being realized, and much of the focus in management of children with ICDs surrounds the prevention of inappropriate shocks.     

Transition of care to adult congenital heart centres: what do patients know about their heart condition?

BACKGROUND: The population of adults with congenital heart disease is growing rapidly, and the transition of care from the pediatric to the adult setting is often not optimal. OBJECTIVES: To assess the level of knowledge that adults with congenital heart disease have of their condition. METHODS: All new patients referred to an adult congenital heart centre were asked to answer questions about their clinical diagnosis, their need and reason for anti-bioprophylaxis, and their risks of pregnancy when applicable. The patient's age at first visit, source of referral and time from last visit in cardiology (defined as the last visit with a cardiologist) were also noted. RESULTS: From November 1999 to July 2000, 104 patients (42 men) were referred to the adult centre by pediatric cardiologists (56), medical cardiologists (26), general practitioners (11), dentists (two) or obstetricians (two), or they were self-referred (seven). The mean age at the time of referral was 28 11 years (range 16 to 72 years, median 24 years). The time from the last visit in cardiology varied widely from one month to 25 years (median three years), with 29 patients (28%) having had no follow-up for more than five years. Among these, 14 patients had no follow-up for more than 10 years, with six patients having been referred for complications related to their cardiac anatomy, such as heart failure (Eisenmenger, Ebstein), syncope (operated and unoperated tetralogy of Fallot) and arrhythmias (atrial septal defect, unoperated Fallot). The clinical diagnosis was completely unknown by 36 patients (34.6%), including patients with repaired Fallot (three patients), Mustard procedure (two), severe aortic stenosis (two), severe pulmonary stenosis (one), Eisenmenger (one), unoperated Fallot (one), ventricular septal defect (six) and bicuspid aortic valve (seven). Seventy-three patients (79%) knew about antibiotic prophylaxis, but 50% did not comprehend why they needed it. Many women (66%) had never discussed the risks of pregnancy with their physician. CONCLUSIONS: A large percentage of adults with congenital heart abnormalities show a poor level of knowledge about their heart condition. These observations suggest the importance of structured transitional programs, the impact of which will need to be validated in prospective studies.     

The adult with congenital heart disease: cardiac catheterization as a therapeutic intervention

The adult with congenital heart disease who undergoes cardiac catheterization at the present time is most likely to have complex heart disease and is left with clinically important sequelae or residual defects, ventricular dysfunction or arrhythmias. Residual defects such as paravalvular leaks, coronary fistulas and pulmonary artery narrowings may be corrected with transcatheter techniques. Patients with simple forms of congenital heart disease (for example, atrial septal defect, patent ductus arteriosus, aortic valve stenosis, pulmonary valve stenosis) will go to the catheterization laboratory for treatment, not diagnosis. Certain lesions previously considered benign (for example, patent foramen ovale) may require definitive interventional therapy to reduce the risk of stroke from paradoxic embolism.     

Care of adults with congenital heart disease--a challenge for the new millennium

The care of complex congenital heart defect (CHD) patients should be continuous through life. Medium- and high-risk patients should be seen in special facilities for grown-up congenital heart (GUCH) patients, and followed for life. CHD in the adult is different than CHD in the child. Transitional programs should be available to prepare the adolescent patient to take charge of his/her own health. The patient should be transferred smoothly from pediatric to adult care. GUCH care should be regionally anchored in special facilities (groups or sometimes individuals) willing to make a commitment to their care. Ideally, pediatric and adult cardiologists will collaborate. GUCH care should be multidisciplinary wherever possible--and certainly in supraregional centers. Surgery, diagnostic catheterization, interventional cath procedures, EP management, and even MRI should be done in selected centers with high quality services for all cases other than the most simple lesions. Care should be available at all times. Surgeons who operate on children with similar conditions should perform the most GUCH surgery. GUCH surgery should be performed in centers with adequate institutional and individual surgeon's volumes. In determining which surgeons and units have adequate volumes, both pediatric and adult volumes should be combined.     

Adult patients with congenital heart abnormalities: present and future

The incidence of congenital heart disease is 8 to 10 per 1000 newborn. Due to the advances of heart surgery, medical treatment and interventional cardiology the primarily high mortality rate of relevant congenital heart disease has declined from 80 % to 20 %. Nowadays more than 80 % of children with relevant congenital heart disease reach adulthood. Currently approx. 150 000 adults with significant congenital heart anomalies live in Germany presently. Their number is rising continuously. With the exception of an patent Ductus Botalli and a small atrial septal defect, which could be operated in early childhood curatively, all other congenital heart defects need regular control, since even after successful primary interventional or operative treatment significant residue or sequelae have to be expected. Optimal care of these patients begins in the childhood and must be continued down to the high adulthood. Almost all of them need a careful follow-up in order to recognize residue or sequelae and to treat them in time. The complexity of many congenital heart defects makes a multidisciplinary approach necessary. A specialized, interdisciplinary cooperation between cardiologists, pediatric cardiologists, cardiac surgeons and other specialists is mandatory.     

Congenital heart disease in adults: catheterization laboratory considerations.

Congenital heart defects are the most common birth defects and represent an increasing proportion of adolescent and adult patients followed by cardiologists. While many of these patients have undergone successful palliative or corrective surgery with excellent functional results, most of them still require careful follow-up. Further, even complex lesions may first be diagnosed in adolescence and adulthood. Therefore, cardiologists caring for adults need to become more familiar with these defects. Assessment of the patient with known or suspected congenital heart defects requires a careful history, physical examination, and noninvasive assessment. In addition, the catheterization laboratory remains a critical venue for diagnosis and, increasingly, therapy. Pressure measurements, oximetry, and angiography remain cornerstones of diagnosis in selected patients and a variety of interventional procedures have become viable therapeutic alternatives in both pre- and postoperative patients.     

Echocardiographic diagnosis alone for the complete repair of major congenital heart defects

Objectives. The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair.Background. Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects.Methods. To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery.Results. Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality.Conclusions. This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.     

Transcatheter closure of intracardiac defects in adults

There has been a dramatic increase in the adult congenital heart disease population and appreciation of intracardiac shunt lesions discovered or acquired in adults over the past several years. Fortunately, this increase has been met with advances in imaging modalities, which permit a more accurate noninvasive assessment of cardiac defects. Additionally, the evolutions in both device technology as well as fluoroscopic and echocardiographic image guidance have permitted the safe and effective catheter-based closure of numerous intracardiac defects. With catheter-based closure procedures now considered the treatment of choice in most cases of intracardiac defect repair in adults, it is imperative that clinicians possess a sound understanding of intracardiac shunt lesions and indications for repair or closure so that they may better care for this unique subset of adult patients. This review will focus on the anatomy, pathophysiology, and current transcatheter therapeutic options for adult patients with patent foramen ovale (PFO), atrial septal defects (ASD), and ventricular septal defects (VSD).     

Prevalence of congenital heart disease

Background

Today most patients with congenital heart disease survive childhood to be cared for by adult cardiologists. The number of physicians that should be trained to manage these lesions is unknown because we do not know the number of patients.

Methods

To answer this question, the expected numbers of infants with each major type of congenital heart defect born in each 5-year period since 1940 were estimated from birth rates and incidence. The numbers expected to survive with or without treatment were estimated from data on natural history and the results of treatment. Finally, lesions were categorized as simple, moderate, or complex, based on the amount of expertise in management needed for optimal patient care.

Results

From 1940 to 2002, about 1 million patients with simple lesions, and half that number each with moderate and complex lesions, were born in the United States. If all were treated, there would be 750,000 survivors with simple lesions, 400,000 with moderate lesions, and 180,000 with complex lesions; in addition, there would be 3,000,000 subjects alive with bicuspid aortic valves. Without treatment, the survival in each group would be 400,000, 220,000, and 30,000, respectively. The actual numbers surviving will be between these 2 sets of estimates.

Conclusions

Survival of patients with congenital heart disease, treated or untreated, is expected to produce large numbers of adults with congenital disease, and it is likely that many more adult cardiologists will need to be trained to manage moderate and complex congenital lesions.     

Is it safe to perform cardiac catheterizations on adults with congenital heart disease in a pediatric catheterization laboratory?

OBJECTIVE: To determine the complication rate during the catheterization in adults with congenital heart disease (CHD) in a pediatric catheterization laboratory (PCL). BACKGROUND: An increasing number of patients with CHD are surviving into adulthood, with diagnostic and interventional cardiac catheterization being essential for the management of their disease. The complication rate during the catheterization of adults with CHD has not been reported. METHODS: A retrospective chart review was performed on all adult patients (>18 years) with CHD who underwent diagnostic or interventional catheterization in our PCL within the past 8.5 years. RESULTS: A total of 576 procedures were performed on 436 adult patients (median age 26 years). Complex heart disease was present in 387/576 (67%) procedures. An isolated atrial septal defect or patent foramen ovale was present in 115/576 (20%) procedures, and 51/576 (9%) procedures were performed on patients with structurally normal hearts with arrhythmias. Interventional catheterization was performed in 378/576 (66%) procedures. There were complications during 61/576 (10.6%) procedures; 19 were considered major and 42 minor. Major complications were death (1), ventricular fibrillation (1), hypotension requiring inotropes (7), atrial flutter (3), retroperitoneal hematoma, pneumothorax, hemothorax, aortic dissection, renal failure, myocardial ischemia and stent malposition (1 each). The most common minor complications were vascular entry site hematomas and hypotension not requiring inotropes. Procedures performed on patients > or = 45 years of age had a 19% occurrence of complications overall compared with 9% occurrence rate in patients of age < 45 years (P < 0.01). CONCLUSIONS: The complication rate during the catheterization of adults with CHD in a PCL is similar to the complication rate of children with CHD undergoing cardiac catheterization. The older subset of patients are more likely to encounter complications overall. The encountered complications could be handled effectively in the PCL. With screening in place, it is safe to perform cardiac catheterization on most adults with CHD in a PCL.     

Incidence of congenital heart disease in Qatari children

We surveyed the incidence of congenital heart disease in 49 887 native live born children in the period between 1984 to 1994 in Qatar. Each child with clinically suspected congenital heart disease underwent echocardiographic examination. Magnetic resonance imaging, cardiac catheterization and surgical intervention were done at the discretion of the patient's pediatric cardiologist. Virtually no postmortem examinations were performed. Children with congenital heart disease were entered into a computerized database and were then followed for 1–11 years. Congenital heart disease was diagnosed in 610 of 49 887 children for an incidence of 12.23/1000 live births. The reasons for the high incidence were high proportion of small muscular ventricular septal defects discovered before the time of their spontaneous closure, referral to and follow up by a single group of pediatric cardiologists, location of the pediatric cardiology service in the same setting where nearly all of the deliveries took place, freely available health care service, and echocardiographic examination of every child with a clinical diagnosis of congenital heart disease.     

Complications of cardiac catheterization in adults and children with congenital heart disease in the current era

The number of adults with congenital heart disease (CHD) requiring diagnostic and/or therapeutic cardiac catheterization has been increasing. However, there have been few studies on the complications of performing cardiac catheterization in adults with CHD. The aim of this study was to determine the incidence of complications during congenital cardiac catheterization in both adults and pediatric patients. A total of 2134 consecutive cardiac catheterizations performed between 2003 and 2008 were prospectively analyzed. Complications were graded from 1 to 5 based on severity and these, with ≥grade 3 being defined as major. During the study period, 576 procedures (393 diagnostic, 90 interventional, and 93 electrophysiological) were performed in adult patients (≥18 years). Complex heart disease was present in 435 of 576 procedures (75.6 %). A total of 65 complications (11.3 %) with 13 major complications including 1 death (2.3 %) were encountered. The most common complications were arrhythmias. The majority of complications were successfully treated or temporary, and all but one of the patients were without residua. Of the 1558 pediatric procedures performed during the same period, we found a total of 229 complications (14.7 %), of which 89 (5.7 %) were major complications including 5 deaths. The safety of performing cardiac catheterization for adult CHD appears to be similar to that for pediatric patients. The complication rates in adults with CHD are low, but not negligible.     

Congenital heart disease in adults. Part I. Non-cyanotic heart diseases]

Over the last decades major advances have been made in the diagnosis and treatment of congenital heart disease. Therefore, many children with simple and complex congenital heart defects can now survive to adulthood and represent an expanding population. Most of them are not "cured" and need specialized follow-up. This two-part review presents the more common acyanotic and cyanotic congenital heart diseases that adult cardiologists are likely to face in their daily practice and discusses recent treatment advances.     

New Directives in Cardiac Imaging: Imaging the Adult With Congenital Heart Disease

Advances in pediatric surgical and interventional techniques and medical care over the past 50 years have revolutionized the care of children with congenital heart disease. Survival to adulthood is now expected and, as such, there is a growing population of adults which is exceeding the pediatric population with congenital heart disease. Noninvasive cardiac imaging with modalities such as echocardiography, computed tomography, and cardiac magnetic resonance imaging are integral to the care of adults with congenital heart disease. These modalities are used for diagnosis, surveillance for complications late after surgery and catheter-based interventions, and in decision-making for medical, interventional, and surgical therapies. In this review we will discuss noninvasive imaging modalities used to assess congenital cardiac lesions, imaging strategies for select congenital lesions, and comment on the future of cardiac imaging in congenital heart disease.     

Echocardiographic Evaluation of Adult Postoperative Congenital Heart Disease

Diagnostic and surgical advances have resulted in a large and growing population of adult patients with congenital heart disease. Proper care of these individuals requires knowledge of the original defects as well as the multitude of variations on surgical intervention for those lesions. Most complex congenital cardiac malformations amenable to "biventricular repair" are cyanotic and include a mixture of septal defects, obstructive and regurgitant valvular, subvalvular, or supravalvular lesions, and malposition of the cardiac chambers and great arteries. Whether they reach adulthood unoperated largely depends upon the balance of pulmonary blood flow. Examples of anatomical substrates that are usually candidates for "biventricular repair" include conotruncal abnormalities such as tetralogy of Fallot, transposition complexes of the great arteries, double-outlet right or left ventricle, and truncus arteriosus. "Biventricular repair" results in two well formed functional ventricles with a subpulmonic ventricle and a subaortic ventricle that are able to provide a circulation in series without admixture of venous and systemic blood. A unifying theme includes patch closure of a ventricular septal defect and reconstitution of ventricular-to-pulmonary arterial blood flow.     

Patterns of congenital heart disease in unoperated adults: a 20-year experience in a developing country

BACKGROUND: The number of patients with congenital cardiac disease reaching adulthood is increasing steadily. Many adults with such disease face both medical and surgical difficulties. HYPOTHESIS: This retrospective study was undertaken to assess the frequency and outcome of congenital heart disease (CHD) in unoperated adults. METHODS: The charts of all patients with unoperated CHD, who were admitted to a tertiary care center in Beirut, Lebanon, between 1980 and 2000 were reviewed. Of these, 206 patients (52% men, age at admittance 18-71 years [32.8 +/- 13.3 years]) with a diagnosis of CHD were evaluated. Atrial septal defect (ASD) was the most common cardiac malformation with a relative frequency of 53%, followed by ventricular septal defects (11%), tetralogy of Fallot (11%), aortic anomalies (7%), pulmonary stenosis (6%), and Ebstein anomaly (4%). Most patients were symptomatic upon presentation, with dyspnea on exertion being the most common presenting symptom. Twenty-seven patients (13%) had cyanotic CHD. Of 179 acyanotic patients, 113 (63%), and 17 of 27 cyanotic patients (63%) underwent surgical intervention. In-hospital surgical complications for the acyanotic group included cerebrovascular accident (2%) and heart block (1%). Total surgical mortality was 4 of 130 (3%). One patient with tetralogy of Fallot presented with endocarditis and died. CONCLUSION: Atrial septal defect is the most common defect reported in our experience; however, it occurs more frequently than that reported in the literature. Although most patients were symptomatic on presentation, their functional status was stable. Accordingly, their hospital course, whether managed medically or surgically, held a relatively low complication rate. This could be attributed to the uncomplicated nature of pathologies in our series. The surgical mortality and in-hospital complications were slighter higher than those reported for similar lesions if repaired during childhood. This study reflects the relative frequency of various cardiac malformations in selected patients with "grown up" congenital heart disease (GUCH) and their natural survival pattern.     

Caring for adults with congenital cardiac disease: successes and challenges for 2007 and beyond

Patients with congenital cardiac disease require lifelong medical care. Current challenges that face practitioners who care for adults with congenital heart disease include identifying the best location for procedures, which could be a children's hospital, an adult hospital, or a tertiary care facility; providing appropriate antenatal management of pregnant women with congenitally malformed hearts, and continuing this care in the peripartum period; and securing the infrastructure and expertise of the non-cardiac subspecialties, such as nephrology, hepatology, pulmonary medicine, and haematology. The objectives of this review are to outline the common problems that confront this population of patients and the medical community, to identify challenges encountered in establishing a programme for care of adults with congenitally malformed hearts, and to review the spectrum of disease and operations that have been identified in a high volume tertiary care centre for adult patients with congenital cardiac disease. Three chosen examples of the fundamental problems facing the practitioner and patient in the United States of America in 2007 are the neglected patient with congenital cardiac disease, weak infrastructure for adults with congenital cardiac disease, and family planning and management of pregnancy for patients with congenital cardiac disease. Patients with adult congenital cardiac disease often do not receive appropriate surveillance. Three fundamental reasons for this problem are, first, that most adults with congenitally malformed hearts have been lost to follow-up by specialists, and are either receiving community care or no care at all. Second, patients and their families have not been educated about their malformed hearts, what to expect, and how to protect their interests most effectively. Third, adult physicians have not been educated about the complexity of the adult with a congenitally malformed heart. This combination can be fatal for adults with complications related to their congenitally malformed heart, or its prior treatment. Two solutions would improve surveillance and care for the next generation of patients coming out of the care of paediatric cardiologists. The first would be to educate patients and their families during childhood and adolescence. They would learn the names of the diagnoses and treatments, the problems they need to anticipate and avoid, the importance of expert surveillance, career and family planning information, and appropriate self-management. The second solution would be to encourage an orderly transfer of patients from paediatric to adult practice, usually at about 18 years of age, and at the time of graduation from high school. Clinics for adults with congenital cardiac disease depend upon multidisciplinary collaboration with specialties in areas such as congenital cardiac imaging, diagnostic and interventional catheterization, congenital cardiac surgery and anaesthesia, heart failure, transplantation, electrophysiology, reproductive and high risk pregnancy services, genetics, pulmonary hypertension, hepatology, nephrology, haematology, and others. None of these services are easily available "off the rack", although with time, experience, and determination, these services can develop very well. Facilities with experienced personnel to provide competent care for adults with congenital cardiac disease are becoming increasingly available. Parents and patients should learn that these facilities exist, and be directed to one by their paediatric caregivers when the time comes for transition to adult care. With the steady increase in the number of adults with congenital heart disease, an ever increasing number of women with such disease are becoming pregnant. Services are not widely available to assess competently and plan a pregnancy for those with more complex disease. It is essential to have a close interplay between the obstetrician, the adult congenital cardiologist, the fetal medicine perinatologist, and neonatologist. In both a community based programme and a tertiary care centre, the nuances and complexities of congenital cardiac anatomy, coupled with the high probability of previous operation during childhood, makes the trained congenital cardiothoracic surgeon best suited to deal with the surgical needs of this growing population. It is clear that the majority of adults with congenital heart disease are not "cured", but require lifelong comprehensive care from specialists who have expertise in this complex arena. There is a growing cadre of healthcare professionals dedicated to improving the care of these patients. More information has become available about their care, and will be improved upon in the next decade. With the support of the general paediatric and paediatric cardiologic communities, and of the Adult Congenital Heart Association, and with the persistence of the providers of care for adults with congenital cardiac disease currently staffing clinics, the care of these patients should become more secure in the next decade as we mature our capabilities.