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Children with Congenital Heart Disease Are Not Getting the Follow-Up They Need

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Article:  Children with Congenital Heart Disease Are Not Getting the Follow-Up They Need: We Must Do Better!


  1. Mackie AS, Ionescu-Ittu R, Therrien J, Pilote L, Abrahamowicz M, Marelli AJ.,  Children and adults with congenital heart disease lost to follow-up: who and when?.,  Circulation,  120 (4) 302-9. View in PubMed

Clinical Question

Are patients with congenital heart disease receiving the follow-up care they need?


In the current issue of Circulation, Mackie et al. report on the follow-up of all children born in Quebec in 1983 and alive at 22 years of age who were diagnosed with congenital heart disease (CHD) before 6 years of age.[1] Furthermore, only 65% of the children born in Quebec in 1983 with CHD were diagnosed before 6 years of age. Why are these data important? Congenital heart defects are the most common group of birth defects, occurring in approximately eight in 1,000 newborns.[2] The success of pediatric cardiologists and cardiac surgeons over the past 25 years is that approximately 90% of children with CHD now survive well into adulthood.[3]

Over the past 15 years, we have become acutely aware of the lack of adequate transition of children with CHD to adulthood and the need to train subspecialist physicians to take care of adults with CHD. Many patients, once leaving the care of their pediatric cardiologist at approximately 18 years of age, often do not see another cardiologist, let alone a cardiologist with expertise in adult congenital heart disease (ACHD) for 10 years or longer. This, in itself, is a major health problem, as adults with CHD need a cardiologist who, in addition to understanding ACHD, also is well trained in the acquired cardiac disorders that occur in adult subjects without CHD. The potential complications of CHD may also change as the patient ages, with the development of cardiovascular problems due to the "aging repaired heart" that were unforeseen to the pediatric cardiologist during childhood. A large number of patients with ACHD have no regular cardiology care, or are not aware that they should continue to have regular cardiology follow-up as adults, and could benefit from the ongoing care.

In the U.S. Natural History Study, 40% of patients with aortic stenosis, pulmonary stenosis, and/or ventricular septal defect had not had a cardiac examination in more than 10 years.[4] Additionally, in a general population of patients who have not been evaluated and followed as rigorously as in the Natural History study, we would expect this rate to be significantly higher. However, most of us believed that, as long as the patients remained under 18 years of age, they were at least getting appropriate follow-up care by a pediatric cardiologist.

Mackie et al. demonstrate that we are not even successful in giving adequate follow-up care for children with CHD during childhood. They evaluated three cohorts: 6 to 12 years old, 13 to 17 years old, and 18 to 22 years old. Failure to receive cardiac follow-up after the 6th, 13th, and 18th birthday occurred in 28%, 47%, and 61% of the patients, respectively. Among the patients classified as having "severe" lesions, only 79% were seen after their 18th birthdays, and although the majority of patients did see their primary care physicians, lack of cardiac follow-up, especially for patients with severe CHD, likely results in less than ideal medical care for these patients. Furthermore, lacking this appropriate cardiology follow-up in childhood will only exacerbate the lack of follow-up with patients as they enter adulthood.

Many ACHD patients may not realize they should be receiving regular care and, even for those who seek care, access may be limited by lack of an adequate number of pediatric and adult cardiologists appropriately cross trained. Thus, as our success has improved the outcomes for many children with CHD, it is our responsibility to educate all patients, family members, and our medical colleagues whether they are the patients’ pediatricians, internists, or subspecialists for comorbid conditions. Children born with CHD require lifelong, regular, cardiology follow-up by a physician trained in ACHD. The exception would be the rare patient with mild disease who the cardiologist dismisses from further cardiology follow-up. One instance would be a patient with mild pulmonary stenosis in whom the pulmonary stenosis has not progressed during childhood.

Additionally, children with more complicated CHD are now living longer and require more comprehensive, long-term follow-up than was required several decades ago when it was rare for a child with complex CHD to survive into adulthood. Furthermore, many female patients with ACHD want to have families. This requires a careful and thorough risk-benefit assessment by their cardiologists and obstetricians to determine if carrying a child to term is safe and in the best interests of the mother and the child.

Current estimates suggest that more adults than children are now living with CHD, and this population is estimated to be growing at approximately 5% per year.[5] This growth rate indicates that the current total ACHD population is close to 2 million.[6] It is possible that this figure is low because of the many ACHD patients who are not routinely followed. Furthermore, although in the past it was thought that congenital heart defects seen in adults represented the mild and simple portion of the spectrum, it now appears that the number of adults with conditions of moderate or severe complexity outweighs that of children. Continuing medical breakthroughs will result in new populations of adult survivors with ever more complex CHD.

Clinical Implication/Application

In 2004, a Working Group on Research in Adult Congenital Heart Disease was convened under the sponsorship of the National Heart, Lung, and Blood Institute and the Office of Rare Diseases, the National Institutes of Health/Department of Health and Human Services to make recommendations on research needs in ACHD. The purpose of the working group was to advise the National Heart, Lung, and Blood Institute on the current state of the art in ACHD and identify potential barriers to ensuring optimal clinical care, and to make specific recommendations for overcoming those barriers. Two of the areas reviewed were the epidemiology of ACHD and long-term outcomes of patients with complex CHD. The working group's recommendations included outreach and educational programs for ACHD, creation of a network of ACHD regional centers, and development of a training program for physicians to provide care for ACHD patients.[7]

Outreach programs should identify the "lost" population of adults with CHD. With appropriate support, we could develop a database to track outcomes and develop practice guidelines for ACHD. The network could foster collaborative multicenter clinical trials to address the ever-growing list of complications these patients face. Cross-fertilization is essential between pediatric cardiologists and adult cardiologists, as these patients are not merely large children with CHD but adults with aging hearts with CHD superimposed upon the risk factors for acquired heart disease that we all face. Neither follow-up care by a pediatric cardiologist for the adult with CHD nor care by an adult cardiologist will give these patients the attention they require and deserve.

We must do better. We cannot merely get these children to adulthood and then abandon them; or worse, not even follow the patients as they should be followed during childhood. All of this puts a very different face on the requirements for research and education needed to maintain optimal health and prevent secondary disability in this ever-increasing population. The current economic crisis only exacerbates these problems.


  1. Mackie AS, Ionescu-Ittu R, Therrien J, et al. Children and adults with congenital heart disease lost to follow-up: who and when? Circulation 2009;120(4):302-309.
  2. Hoffman JI. Incidence of congenital heart disease: I. Postnatal incidence. Pediatr Cardiol 1995;16:103-113.
  3. Garson A Jr, Allen HD, Gersony WM, et al. The cost of congenital heart disease in children and adults. A model for multicenter assessment of price and practice variation. Arch Pediatr Adolesc Med 1994;148(10):1039-45.
  4. O'Fallon WM, Crowson CS, Rings LJ, et al. Second natural history study of congenital heart defects. Materials and methods. Circulation 1993;87(2 Suppl):14-15.
  5. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-900.
  6. Hoffman JI, Kaplan S, Liberthson RR. Prevalence of congenital heart disease. Am Heart J 2004;147:425-39.
  7. Williams RG, Pearson GD, Barst RJ, et al. Report of the National Heart, Lung, and Blood Institute Working Group on research in adult congenital heart disease. J Am Coll Cardiol 2006:47(4):701-707.  
-- The opinions expressed in this commentary are not necessarily those of the editors or of the American Heart Association