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Report of the National Heart, Lung, and Blood Institute’s Working Group on Obesity and Other Cardiovascular Risk Factors in Congenital Heart Disease

2010; Lippincott Williams & Wilkins; Volume: 121; Issue: 9 Linguagem: Inglês

10.1161/circulationaha.109.921544

1524-4539

Victoria L. Pemberton, Brian W. McCrindle, Shari L. Barkin, Stephen R. Daniels, Sarah E. Barlow, Helen J. Binns, Meryl S. Cohen, Christina D. Economos, Myles S. Faith, Samuel S. Gidding, Caren S. Goldberg, Rae-Ellen W. Kavey, Patricia E. Longmuir, Albert P. Rocchini, Linda Van Horn, Jonathan R. Kaltman,

Cardiovascular Function and Risk Factors

HomeCirculationVol. 121, No. 9Report of the National Heart, Lung, and Blood Institute's Working Group on Obesity and Other Cardiovascular Risk Factors in Congenital Heart Disease Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBReport of the National Heart, Lung, and Blood Institute's Working Group on Obesity and Other Cardiovascular Risk Factors in Congenital Heart Disease Victoria L. Pemberton, RNC, MS, Brian W. McCrindle, MD, MPH, Shari Barkin, MD, MSHS, Stephen R. Daniels, MD, PhD, Sarah E. Barlow, MD, MPH, Helen J. Binns, MD, MPH, Meryl S. Cohen, MD, Christina Economos, PhD, Myles S. Faith, PhD, Samuel S. Gidding, MD, Caren S. Goldberg, MD, MS, Rae-Ellen Kavey, MD, MPH, Patricia Longmuir, MSc, Albert P. Rocchini, MD, Linda Van Horn, PhD, RD and Jonathan R. Kaltman, MD Victoria L. PembertonVictoria L. Pemberton From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Brian W. McCrindleBrian W. McCrindle From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Shari BarkinShari Barkin From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Stephen R. DanielsStephen R. Daniels From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Sarah E. BarlowSarah E. Barlow From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Helen J. BinnsHelen J. Binns From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Meryl S. CohenMeryl S. Cohen From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Christina EconomosChristina Economos From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Myles S. FaithMyles S. Faith From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Samuel S. GiddingSamuel S. Gidding From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Caren S. GoldbergCaren S. Goldberg From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Rae-Ellen KaveyRae-Ellen Kavey From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Patricia LongmuirPatricia Longmuir From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Albert P. RocchiniAlbert P. Rocchini From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). , Linda Van HornLinda Van Horn From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). and Jonathan R. KaltmanJonathan R. Kaltman From the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (V.L.P., R.K., J.R.K.); Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada (B.W.M., P.L.); Vanderbilt University, Nashville, Tenn (S.B.); University of Colorado, Boulder (S.R.D.); Baylor College of Medicine, Houston, Tex (S.E.B.); Children's Memorial Hospital and Northwestern University Feinberg School of Medicine, Chicago, Ill (H.J.B.); Children's Hospital of Philadelphia, Philadelphia, Pa (M.S.C.); Tufts University, Boston, Mass (C.E.); University of Pennsylvania, Philadelphia (M.S.F.); A.I. DuPont Hospital for Children, Wilmington, Del (S.S.G.); University of Michigan, Ann Arbor (C.S.G., A.P.R.); and Northwestern University Feinberg School of Medicine, Chicago, Ill (L.V.H.). Originally published9 Mar 2010https://doi.org/10.1161/CIRCULATIONAHA.109.921544Circulation. 2010;121:1153–1159Obesity among North American youth has risen to epidemic levels and is expected to result in costly and burdensome health problems, most notably type 2 diabetes mellitus and premature coronary artery disease. In the 2001–2002 National Health and Nutrition Examination Survey survey, 31% of children aged 6 to 19 years were overweight, defined as body mass index (BMI) at the 85th to 95th percentile, or obese (BMI ≥95th percentile).1 Recent research indicates that a significant portion of children with congenital heart disease (CHD) are also obese or overweight.2 Although much has been written on obesity prevention and management of children with normal hearts,3 little is known about how children with CHD will be affected.The National Heart, Lung, and Blood Institute convened a Working Group (WG) on obesity and other cardiovascular risk factors in congenital heart disease in May 2009 to address knowledge gaps, challenges, and opportunities related to research, policies, and the clinical care of children born with CHD who also have or are at risk for obesity and other cardiovascular risk factors.4 The WG was composed of individuals with expertise in pediatric obesity and pediatric cardiology with the goals of summarizing the existing evidence on obesity and cardiovascular risk prevention and treatment in the CHD population, raising awareness of missing data, and presenting data from "healthy" children with potential applicability to children with CHD.In this report, we discuss the prevalence of obesity, potential etiological factors, possible sequelae, and obesity and cardiovascular risk management and treatment in patients with CHD. The recommendations of the WG are presented.Prevalence of Obesity in CHDThere are no longitudinal data on weight trends in children with CHD, and only recently have concerns been raised about obesity. In 2005, Stefan et al5 demonstrated that children with CHD whose activities are restricted are at particular risk for obesity. The first large study to investigate obesity in children with CHD, published just 2 years ago, found that 26% of 1523 patients, seen in 2 large outpatient pediatric cardiology clinics, were overweight or obese.2 Patients who had undergone the Fontan palliative surgical procedure for single-ventricle physiology fared slightly better, with 16% being overweight or obese. Systolic blood pressure percentile was significantly higher in the overweight/obese group than in the normal-weight group, illuminating the potential for long-term complications in this vulnerable population.Pasquali et al6 recently demonstrated that nearly one third of patients aged 6 to 19 years who had undergone the arterial switch operation or Ross procedure (both require reimplantation of coronary arteries) were overweight or obese. These children had higher left ventricular mass and, as in the general pediatric population, were more likely to have an obese parent (defined as BMI >30 kg/m2). Reported activity level and diet did not differ significantly from those of the normal-weight population. Of note, inactivity was common in the entire cohort.There are more adults (>1 million)7 than children with CHD, and the number is expected to grow at ≈5% per year.8 Obesity is of great concern in adults with CHD, with 54% having a BMI >25 kg/m2.9 The most common complications in adults with CHD are arrhythmia, heart failure, endocarditis, cardiac conduit obstructions, valve regurgitation, and pulmonary hypertension.10 Although little evidence exists to suggest the manner in which obesity may affect adults with CHD, it is reasonable that obesity will adversely influence at least heart failure and pulmonary hypertension in these individuals.Etiology of Obesity and Cardiovascular Risk in CHDThe development of obesity in children derives from a number of etiological factors, including biological and physiological (genetic predisposition and appetite), personal (physical activity and diet), environmental, societal, and healthcare-related factors.11 Although obesity has not been studied systematically in individuals with CHD, compelling contributing factors include nutrition and physical activity. The physiology of certain types of heart disease may affect cardiovascular risk.NutritionFor children with serious CHD, the battle to gain sufficient weight has traditionally begun in infancy. Heart failure, hemodynamic compromise, and metabolic response to stress after cardiac surgery place additional burdens on the neonate with limited metabolic reserves.12 Severity of the congenital defect and cyanosis appear to dictate both the energy requirement and the nutrient intake. From birth through the first 2 years of life, body weight, growth rates, and energy intakes in children with CHD are often below the norm.13Postnatal nutritional interventions aim to increase calories through supplemental feedings, alternative feeding methods, and feeding protocols developed to promote weight gain.12 In non-CHD infants, rapid growth has been associated with enhanced risk of obesity, diabetes mellitus, hypertension, cardiovascular disease, and osteopenia in later life.14 When children who were small for gestational age were compared with children of normal birth weight at age 4 years, they already had increased central adiposity and insulin resistance.15 Key questions remain about the manner in which early feeding behaviors, increased caloric density early in life, and dietary recommendations affect early growth patterns in children with CHD and whether families treat their child with CHD differently than usual-care families.Physical ActivityThe American Heart Association guidelines indicate that most children with CHD can be fully active,16 but children with CHD are less likely to meet physical activity recommendations than their peers.17 Behavioral factors appear to influence inactivity more than the severity of the condition. Self-esteem and physical self-concept are compromised in some children with CHD and may limit their inclination for activity.18Children with CHD often lead sedentary lives even after their heart defect has been treated successfully. In a study of 144 children aged 7 to 18 years after the Fontan procedure, the mean self-reported time spent in physical activity was 7 hours per week compared with 28 hours per week engaged in sedentary activities. However, accelerometry indicated that only 38% of study subjects actually met physical activity recommendations for children and adolescents.19 Research has focused on measurements of exercise capacity (maximal heart rate or oxygen consumption) as indicators of cardiac performance. Children with CHD have normal peak heart rates (189±12 bpm)20 but decreased peak oxygen consumption,21 which is indicative of suboptimal fitness. The benefits and safety of exercise training for increasing fitness have been described for this population,22 with increased fitness levels persisting over the long term.23Children with CHD and their parents often self-limit exercise or sports,24 which may be unnecessary and even counterproductive. Children who are formally restricted from physical activity or competitive sports have a higher BMI over time.5 Parental anxiety about the safety of physical activity may exceed their interest in promoting fitness. Less physical activity in childhood extends into adulthood, where individuals again fail to achieve recommended levels of physical activity.25 Daily physical activity is closely associated with decreased risk for cardiovascular disease, obesity, diabetes mellitus, and hypertension.Pediatric cardiologists are frequently asked to provide recommendations for physical activity, but these may be inappropriately extrapolated from published guidelines for competitive athletics in children with heart disease,26 providing little practical advice for parents about more routine physical activity. Lunt and colleagues27 found that only 19% of young adult subjects with CHD received advice about physical activity. Although guidelines exist for competitive sports participation in athletes with specific cardiovascular abnormalities28 and for recreational activities in children with genetic heart conditions,29 none exist for safe physical activity for individuals with complex forms of CHD or for those whose hemodynamics are suboptimal. Development of guidelines and consensus statements to encourage regular physical activity outside of competitive sports will require research to determine the type, duration, and intensity of activity in which an individual with a particular cardiac lesion can participate safely.30Specific Malformations and Their TreatmentCardiovascular risk may vary by type of heart defect. For example, it is possible that specific conditions in which the coronary arteries are directly affected or altered surgically may confer greater risk for premature atherosclerotic coronary artery disease.7 Coronary artery reimplantation at the time of transposition repair has been shown to result in abnormal coronary flow reserve,31 and intracoronary ultrasound reveals that some patients develop intimal proliferation, a precursor to atherosclerosis.32 Left-sided obstructive lesions may also be associated with accentuated cardiovascular risk. Coarctation of the aorta, even after repair, is commonly associated with systemic hypertension; aortic stenosis can be associated with left ventricular hypertrophy and diastolic dysfunction, known risk factors for adult-onset cardiovascular morbidity and mortality.7 Interestingly, in a study of adults with CHD, no cyanotic patient had coronary artery disease, leading to the hypothesis that cyanosis may be protective.33Sequelae of Obesity and Cardiovascular Risk Factors in the Patient With CHDThe relationship between childhood obesity and subsequent cardiovascular risk has been well described in children without CHD. These data suggest that the adverse effects of obesity on the patient with CHD include its potential impact on physiology, functional health status, and quality of life.Pathophysiological SequelaeObesity is associated with the presence or future development of hypertension, insulin resistance, dyslipidemia, increased inflammatory cytokines, sleep apnea, autonomic imbalance, and abnormal cardiac remodeling.34 In the obese individual without CHD, hypertension is often the result of increased fluid retention associated with an increased plasma volume and cardiac output.35 Fluid retention is caused by activation of both the sympathetic nervous system and the renin-angiotensin-aldosterone system.36 The physiological consequences of insulin resistance include altered lipid metabolism (increased triglycerides, decreased high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol particles that are small, dense, and atherogenic),37 altered vascular reactivity in both peripheral and coronary arterial beds,38 nonalcoholic fatty liver,39 and the development of type 2 diabetes mellitus. Obesity is also associated with an increase in the inflammatory cytokines known to be related to atherosclerosis and coronary artery disease.40 The autonomic imbalance associated with obesity includes withdrawal of parasympathetic tone and sympathetic predominance with a resultant reduction in heart rate variability and increased risk of atrial and ventricular dysrhythmias.41 Finally, obesity is also associated with diastolic and systolic ventricular dysfunction, left ventricular hypertrophy, and congestive heart failure.42,43 All of these factors have potential adverse consequences for individuals with CHD, many of whom are already at risk for ventricular dysfunction, heart rate and rhythm disturbances, and overt heart failure.Obesity may adversely affect an individual with CHD and is illustrated by the patient after Fontan repair for a single ventricle. The staged surgical approach, culminating in the Fontan procedure, results in passive systemic venous flow rather than active pumping of blood to the lungs because the single ventricle must pump to the systemic circulation. An increase in fluid volume or sleep apnea associated with obesity would increase already elevated right-sided volume and filling pressures, leading to increased risk of edema and ascites. Increased inflammatory cytokines may enhance the thrombotic risk or play a role in the pathogenesis of protein-losing enteropathy, a serious morbidity that occurs in ≈5% of patients with Fontan physiology.44 The autonomic imbalance may lead to a further increase in the already significant risk for atrial rhythm disorders. Finally, obesity could exacerbate the diastolic dysfunction already seen in >70% of children and young adults with Fontan circulation.45The manner in which the pathophysiology of CHD interacts with obesity and other cardiovascular risk factors is unknown. Patients with CHD have an abnormal myocardial substrate on which these traditional cardiovascular risk factors are superimposed. Not only is the heart abnormally formed but the myocardium is also subject to periods of cyanosis and ischemia/reperfusion during 1 or more surgical operations. A recent study demonstrated that atherosclerotic coronary artery disease in adult patients with CHD with no history of ischemic heart disease was associated with abnormal systolic and diastolic dimensions of the systemic ventricle.33 These findings, although preliminary, certainly suggest the potential implications of traditional cardiovascular risk factors in the setting of CHD.Functional Health Status and Quality of LifeResearch on the impact of obesity on functional health status and quality of life shows that compared with lean children, obese children report poorer quality of life46 and are more likely to have functional limitations. Young adolescents have more difficulties with depression, self-esteem, and school and social functioning.47Children with CHD are at higher risk for behavioral and emotional problems,48 with neurological deficits and behavioral abnormalities among the most concerning long-term morbidities. Neurodevelopmental impairments and exercise limitations are factors that may contribute to lower perceived quality of life for these children. The typical neurodevelopmental impairments observed in children with CHD include abnormalities in memory and higher-order language function, visual-spatial relations, and executive function.49 Attention-deficit/hyperactivity disorder has also been found to occur at higher rates.50Obese children and adolescents are at increased risk for obstructive sleep apnea, which can lead to memory impairment51 and behavioral abnormalities.52 Although the effects of obesity and obstructive sleep apnea have not yet been measured for patients with CHD specifically, the possible combination of obesity superimposed on this at-risk population is likely to lead to worsened school out