The Care of Children with Congenital Heart Disease in Their Primary Medical Home

Home , Asplenia

POLICY STATEMENT Organizational Principles to Guide and Define the Child Health Care System and/or Improve the Health of all Children

The Care of Children With M. Regina Lantin-Hermoso, MD, FAAP, FACC,a Stuart Berger, MD, FAAP,b Ami B. Bhatt, MD, FACC,c CongenitalJulia E. Richerson, MD, FAAP,d Robert Heart Morrow, MD, FAAP, Diseasee Michael D. Freed, MD, FAAP,in FACC, f g TheirRobert H. Beekman Primary III, MD, FAAP, FACC, SECTION Medical ON CARDIOLOGY, CARDIAC Home SURGERY

Congenital heart disease (CHD) is the most common birth anomaly. With abstract advances in repair and palliation of these complex lesions, more and more patients are surviving and are discharged from the hospital to return to their families. Patients with CHD have complex health care needs that often must be provided for or coordinated for by the primary care provider (PCP) and medical home. This policy statement aims to provide the PCP with aSection of Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children’s Hospital Heart Center, Houston, general guidelines for the care of the child with congenital heart defects and Texas; bHeart Center, Ann & Robert H. Lurie Children’s Hospital of outlines anticipated problems, serving as a repository of current knowledge Chicago, Chicago, Illinois; cAdult Congenital Heart Disease Program, Massachusetts General Hospital, Boston, Massachusetts; dFamily in a practical, readily accessible format. A timeline approach is used, Health Centers, Louisville, Kentucky; ePhysician Organizations and Academic Relations, Children’s Health Children’s Medical emphasizing the role of the PCP and medical home in the management of Center, Dallas, Texas; fDepartment of Cardiology, Boston Children’s patients with CHD in their various life stages. Hospital, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts; and gDivision of Pediatric Cardiology, Department of Pediatrics, University of Michigan School of Medicine, Ann Arbor, Michigan

Drs Berger and Richerson contributed to the refinement of the design, the writing of specific portions of the manuscript (neonatal and cardiopulmonary resuscitation recommendations and infection, childhood, immunizations, growth, and development, respectively), Primary care providers (PCPs; physicians, physician assistants, and and the critical review of the manuscript; Dr Bhatt contributed to nurse practitioners) working in medical homes (MHs) are tasked the refinement of the concept and design, the writing of specific portions of the manuscript (adolescence, transition, and exercise), with providing and coordinating the multiple health care and the critical review of the manuscript; Dr Morrow contributed needs of patients with congenital heart disease (CHD). With the to the refinement of the concept, design, and outline of the paper and to the critical review of manuscript; Drs Freed and Beekman goals of improving patient outcomes and influencing the care of contributed to the refinement of the concept, design, and outline of these children, the American Academy of Pediatrics (AAP) and the the paper, to the editing of specific portions (exercise and exercise and final recommendations, respectively), and to the critical review American College of Cardiology (ACC) assembled a team of experts, of manuscript; Dr Lantin-Hermoso contributed to the original idea, including representation from the AAP Committee on Practice concept, and design of the paper, to the outline of the topics, to the ’ presentation of the proposal to the American Academy of Pediatrics and Ambulatory Medicine, to review current literature and to (AAP), to ensuring all deadlines and requirements were met, to develop this policy statement. A representative from the AAP s writing, to the compilation of other authors’ contributions, to editing, to the critical review of the manuscript, and to the response to the Family Partnership Network was requested to review this document. AAP reviewers; and all authors approved the final manuscript as The role of the PCP and family engagement across the life span is submitted. emphasized.

Background To cite: Lantin-Hermoso MR, Berger S, Bhatt AB, et al. The Care of Children With Congenital Heart Disease in Their Primary Medical Home. Pediatrics. 2017;140(5):e20172607 Advances in treatment and palliation of CHD result in more patients surviving and being discharged from the hospital with their families. Downloaded from www.aappublications.org/news by guest on October 2, 2021 PEDIATRICS Volume 140, number 5, November 2017:e20172607 From the American Academy of Pediatrics FIGURE 1 Targets of PCP engagement across the life span.

TABLE 1 Selected Advocacy/Parental Support Groups and Resources Adult Congenital Heart Association: http://www.achaheart.org/ Previously unrecognized familial Anthony Bates Foundation: http://www.anthonybates.org psychosocial stressors, racial and Got Transition/Center for Health Care Transition: http://www.gottransition.org/ socioeconomic outcome disparities, It’s My Heart: http://www.childrensheartfoundation.org/publications/its-my-heart other organ system involvement, Little Hearts: https://www.littlehearts.org complications of therapy, feeding Linked By Heart: http://linked-by-heart.org/ Mended Little Hearts: http://www.mendedlittlehearts.org/ challenges, neurodevelopmental Nick of Time: http://nickoftimefoundation.org concerns, and other special needs Parent Heart Watch: www.parentheartwatch.org/ have been garnering more attention. Pediatric Congenital Heart Association: http://conqueringchd.org/ Recommendations regarding Project ADAM: http://www.chw.org/childrens-and-the-community/resources-for-schools/cardiac-arrest- newborn pulse oximetry or critical project-adam/ Simon s Fund: http://www.simonsfund.org CHD (CCHD) screening and infection ’ Sisters-By-Heart: http://www.sisters-by-heart.org/ prevention strategies in children Tiny Smiles: http://www.tinysmiles.net with CHD have been introduced or updated. Patients with CHD have complex health care needs that are to be provided for or coordinated for by be exacerbated by parental guilt, (AED) training for qualified their PCP. This policy statement aims financial and marital stress, challenges caregivers and family members is to be a repository of available new in parental work-life balance, and important. Lay CPR-AED education information for the care of children adversity because of poverty, neglect, may be beneficial and may increase with CHD, highlighting anticipated environmental violence, or caregiver survival in out-of-hospital cardiac problems. A chronologic timeline 4 substance abuse or mental illness. arrest. Many institutions, therefore, approach (Fig 1) is used, emphasizing advocate parental CPR instruction the role of the PCP and/or MH in Disparate outcomes have been before an infant is discharged from the management of patients with observed across racial 2,and3 the hospital. AED availability and CHD and their families in various socioeconomic classes. ‍ A PCP/ CPR training before high school life stages. Frequent communication MH with long-standing relationships graduation are mandated in certain among all care providers is with the family may be the first states.*Prenatal Period Grecommended.eneral Issues Affecting Families of to recognize danger signs and be Children With CHD in a position to provide support and referrals to mental health Psychosocial Issues professionals and groups that CHD is often detected by fetal can provide respite care and echocardiography, usually performed ’ psychologicalNeed for Cardiopulmonary help (Table 1). In response to the stress of their Resuscitation and Automated External child s serious cardiac illness, some Defibrillator Training for Qualified parents report an increased incidence Caregivers *For more information regarding state-specific of emotional distress and psychosocial CHD-related policies, please contact the AAP 1 Division of State Government Affairs at stgov@ risk affecting optimal parenting aap.org. that may also affect the care of other Cardiopulmonary resuscitation (CPR) children in the home. This may and automated external defibrillator Downloaded from www.aappublications.org/news by guest on October 2, 2021 2 FROM THE AMERICAN ACADEMY OF PEDIATRICS ∼ ’ TABLE 2 Genetic Abnormalities Associated With CHD Common, Presently Known Chromosome Select Presently Known Single Gene Defects at 18 to 22 weeks gestation. Abnormalities However, both false-positive and Cri-du-chat syndrome Ehlers-Danlos syndrome false-negative diagnoses can occur, DiGeorge syndrome (22q11) Ellis-van Creveld syndrome and certain lesions may be not be Down syndrome (trisomy 21) Holt-Oram syndrome detected in basic screening because Trisomy 18 and trisomy 13 Marfan syndrome Turner syndrome Mucopolysaccharidoses some may be progressive and others5 may develop later in gestation. Wolf-Hirschhorn syndrome Noonan syndrome Smith-Lemli-Opitz syndrome Prenatal counseling is dependent Williams syndrome on the gestational age, disease natural history, presence of extra cardiac anomalies,6 and need for fetal intervention. extremity pulses warrant taking a PCP. Home medications and feeding Barring obstetric complications, relevant history and conducting a regimens, in a schedule compatible delivery at or close to term is physical examination. Depending with family home routines, preferred because of the critical on findings, a pediatric cardiology including formula concentration or development of most organ systems, ’ consultation or other diagnostic expressed breast milk preparation particularly the fetal brain and lungs,7 tests, such as electrocardiography and route of administration, are during the last 6 weeks gestation. or echocardiography, may be important to note. The need for Improved outcomes are ’ performed.Genetic Screening anticoagulation and thrombosis demonstrated in newborn infants prophylaxis for patients dependent with CCHD born at 39 to 40 weeks ’ on systemic to pulmonary artery gestation compared with those born 8 When aneuploidy is suspected, shunts, or for those at high13 risk at 37 to 38 weeks gestation. The children with cardiac defects will of thrombosis and stroke, are best time for the family to establish benefit from genetic evaluation important to communicate. The relationships with primary and to discuss prognosis and target international normalized subspecialty care providers, and to recurrence risks. Genetic testing ratio and enoxaparin levels contact support groups if desired is recommended in particular (for patients on warfarin and (NTableeonatal 1), Period is before delivery. for conotruncal abnormalities, enoxaparin, respectively), the Pulse Oximetry or CCHD Screening such as d-transposition, tetralogy reason for anticoagulation, and the of Fallot, truncus arteriosus, or duration of therapy and frequency interrupted aortic arch type B, to of monitoring should ideally be CCHD screening, added to the US rule out 22q chromosome deletion outlined in discharge summaries. Neonatal Recommended Uniform (velocardiofacial or DiGeorge Screening Panel in 2011, may syndrome), because the latter improve the timeliness of a diagnosis may be associated with calcium Patients with heart failure, baseline cyanosis, shunt dependence for of CCHD that9 has eluded prenatal metabolism, neurodevelopmental detection. A sample protocol has and psychiatric abnormalities, or pulmonary blood flow, or single- 10 ventricle physiology have a limited been published,† but this may vary other organ involvement. Other by state or altitude. This screening genetic abnormalities associated with cardiopulmonary reserve and targets lesions with hypoxemia, but cardiac defects are listed in Table 2. are sensitive to intravascular “ ” Infancy/Childhood the test is imperfect, and a neonate volume changes. These patients Care of the Patient With CHD After who passed might still have may decompensate rapidly Hospital Discharge during childhood respiratory or acyanotic,11,12 left-heart obstructive lesions. ‍ Thus, symptoms such gastrointestinal infections, when as tachypnea, an abnormally active respiratory function and enteral For a coordinated transition of precordium, a concerning heart ’ intake are impaired or fluid losses murmur, or a diminution in lower care from the hospital to the MH, are magnified. Such patients need information regarding the patient s close follow-up with their PCP and/or cardiac diagnosis, completed or MH and may require hospitalization planned interventions, residual and intravenous fluids early in †For more information regarding state-specific defects, organ system involvement, their course of treatment. It would CHD-related policies, please contact the AAP and discharge physical examination be ideal if they were identified Division of State Government Affairs at stgov@ aap.org. findings, including weight, oxygen and if recognition strategies and requirements, and baseline interventions were discussed in saturations, must be available to the advance of occurrence. Downloaded from www.aappublications.org/news by guest on October 2, 2021 PEDIATRICS Volume 140, number 5, November 2017 3 Nutrition and Feeding Challenges

mar the clinical picture or the ability of caring for children at high risk, to mount an immune response after application of fluoride varnish and Adequate nutrition, necessary for cardiopulmonary bypass. Because sealants, and oral fluoride intake are growth and brain development, 24 children with CHD may have a important. may be challenging in certain Neurodevelopmental and Behavioral lowered capacity to resist and fight children with CHD. This is most Concerns infections, comprehensive routine apparent in infants with pulmonary 17 immunizations, including the overcirculation and in those who recommended schedule for 13-valent underwent stage 1 Norwood conjugate pneumococcal vaccine, are Some children with CHD palliation for a single ventricle, who ‡ important. Patients with functional demonstrate a higher rate of adverse may need 120 to 150 kcal/kg per 25 asplenia should receive subsequent neurodevelopmental outcomes day for adequate growth. Published doses of 23-valent polysaccharide and psychological maladjustment nutritional algorithms may serve 18 26 14 vaccine. Some children, particularly related to low self-esteem. This as reference. Some infants have those with heterotaxy and asplenia may be related to the heart disease limited ability for oral intake because or nonfunctional polysplenia, will be itself, injury during earlier episodes of heart failure, suck and swallow at risk for encapsulated bacteremia, of shock or hypoxemia, genetic incoordination, postoperative which may be prevented with daily issues, prematurity, prolonged vocal cord injury, and airway and antibiotic prophylaxis, at least until 5 hospitalization, cardiopulmonary structural or functional neurologic 19,20 years of age. ‍ Recommendations bypass, intervention sequelae, or abnormalities. Infants who cannot for seasonal protection against other factors. The PCP/MH is tasked be safely orally fed may benefit respiratory syncytial virus to provide careful developmental from nasogastric or gastrostomy ’ (RSV) are available and updated and behavioral surveillance and tubes. Published growth charts are 15 regularly. Ensuring herd immunity screening throughout the child s life available but should be consulted by vaccinating close contacts, as well as ongoing assessment of while keeping in mind that some especially against pertussis and academic progress. Early referrals to infants with heart disease may seasonal influenza, is recommended. mental health providers and prompt follow their own curve, and grow 27 Patients with DiGeorge syndrome treatment are important. at a minimal rate of 5 g to 10 g per would benefit from immunologic day. If growth velocity is suboptimal, Exercise and Sports Participation assessment of their T lymphocyte fortifying the caloric density of function. If a patient is found to be infant formula or expressed breast significantly immunocompromised, milk up to 30 kcal/oz may be consultation with an infectious With normal biventricular function necessary. Potential complications disease specialist may be considered and the absence of hemodynamically in these patients may include to identify an appropriate vaccination significant residual lesions, most gastroesophageal reflux, aspiration strategy, because some patients patients with repaired CHD will risk, osmotic diarrhea, constipation, should not receive live-virus benefit from physical activity consequences of improperly 21 vaccines. Endocarditis prevention and conditioning (exercise mixed formula, and in rare cases, includes family education about prescription rather than restriction). necrotizing enterocolitis. Special Immunization Needs and risks and adherence to current Cardiovascular health will be Infection Prevention guidelines for dental procedures enhanced in virtually all children involving gingival manipulation and with CHD by avoiding a sedentary other potentially bacteremic, high- lifestyle, obesity, and hypertension. Studies of rehabilitation and Preventing, identifying, and risk interventions and surgeries. conditioning programs for managing infections in children Conditions and procedures in patients with CHD have generally with CHD are primary roles of the which endocarditis prophylaxis are 28 revealed benefits. European PCP. Current, recent, or upcoming recommended22 are summarized in recommendations support active anesthesia and surgery generally Table‍ 3. Commitment23 to excellent lifestyles that include recreational are not contraindications for oral hygiene, a dental home capable 16 and competitive sports for most immunization. Efforts should 29 patients with CHD. be made to ensure vaccine administration during hospitalization Some patients with CHD are at ‡ or at discharge, when indicated and Please refer to Advisory Committee on risk for cardiac decompensation 16 Immunization Practices guidelines at www.cdc. age-appropriate. However, practice gov/vaccines/acip. and sudden death, depending may vary because of concerns for a on specific30 lesions and their potential febrile response that may severity. Examples of conditions Downloaded from www.aappublications.org/news by guest on October 2, 2021 4 FROM THE AMERICAN ACADEMY OF PEDIATRICS TABLE 3 Endocarditis Prophylaxis Cardiac Conditions for Which Endocarditis Procedures for Which Antibiotic Prophylaxis Is Procedures for Which Antibiotic Prophylaxis Is Not Prophylaxis Is Recommended Recommended Recommended Personal history of previous infective endocarditis Dental procedures with gingival manipulation or Procedures on the genitourinary or gastrointestinal oral mucosa perforation tract Prosthetic cardiac valves Invasive respiratory tract procedures involving Routine injections through noninfected tissue mucosal incision (ie, tonsillectomy and adenoidectomy) Unrepaired cyanotic CHD, including those palliated Procedures on infected skin or musculoskeletal Prosthodontic or orthodontic appliance placement with shunts and conduits tissue without oral mucosa perforation First 6 mo after complete repair of CHD, with Lip bleeding or oral mucosa trauma prosthetic material or device Repaired CHD with residual defects (persistent Loss of deciduous teeth leaks or abnormal flows at or adjacent to prosthetic patch or device) Heart transplant patients with abnormal cardiac Bronchoscopy without mucosal incision valve function Piercing or tattooing through noninfected skin Vaginal delivery or hysterectomy Adapted from Wilson W, Taubert KA, Gewitz M, et al; American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee; American Heart Association Council on Cardiovascular Disease in the Young; American Heart Association Council on Clinical Cardiology; American Heart Association Council on Cardiovascular Surgery and Anesthesia; Quality of Care and Outcomes Research Interdisciplinary Working Group. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116(15):1736–1754 and Baltimore RS, Gewitz M, Baddour LM, et al; American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young and the Council on Cardiovascular and Stroke Nursing. Infective endocarditis in childhood: 2015 update: a scientific statement from the American Heart Association. Circulation. 2015;132(15):1487–1515.

TABLE 4 Exercise Recommendations in CHD Cardiac Concern Low-Intensity Sports Exercise Restriction at highest risk during strenuous activity include those with severe Aortic dilation, aneurysm X X (depending on severity) ventricular outflow obstruction, Moderate to severely depressed ventricular X function hypertrophic cardiomyopathy, Severe pulmonary HTN X congestive heart failure, coronary Severe systemic HTN (untreated) X insufficiency, pulmonary Severe aortic stenosis X hypertension, severe untreated Coarctation of the aorta X X (depending on severity) systemic hypertension, Marfan Untreated cyanotic heart disease X Untreated anomalous coronary artery X syndrome and aortic dilation, origin with an interarterial or intramural exercise-induced arrhythmias, and course long QT syndrome. Some other Exercise-induced arrhythmia X lesions of concern are listed in Adapted from Van Hare GF, Ackerman MJ, Evangelista JA, et al. Eligibility and disqualification recommendations for ‍Table 4. Recommendations vary competitive athletes with cardiovascular abnormalities: task force 4: congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol. 2015;66(21):2372–2384. by individual patient, because risk Recommendations may vary by individual patient. HTN, hypertension. may be altered with intervention and severity of residual lesions. Late Childhood/Adolescence Close collaboration between the Obesity cardiologist and the PCP is essential, surgery were found to have and care must be taken to avoid a two-fold higher mortality giving the family conflicting advice. risk. In contrast, perioperative Patients with CHD are not immune Noncardiac Surgery complications were fewer to the growing trend of obesity33,34 and in ambulatory32 noncardiac inactivity in North America. ‍ In surgeries. Patients with CHD a study of more than 700 children Up to 40% of children with CHD requiring noncardiac surgery with CHD, 28% were overweight and require noncardiac surgery by 5 benefit from careful evaluation and 31 17% had at least 135 BMI calculation years of age. Severity of heart multidisciplinary planning, including indicating obesity. The etiology of disease, cyanosis, pulmonary a thorough understanding of their obesity is multifactorial and includes hypertension, or congestive anatomy and physiology, with poor nutritional choices and physical heart failure increase the risk of inactivity from perceived handicap by input from their PCP, cardiologist, “ ” perioperative morbidity. Those anesthesiologist, and surgeon to the child or parent, possibly an offshoot requiring inpatient noncardiac minimize the risk of adverse events. of the vulnerable child syndrome. Downloaded from www.aappublications.org/news by guest on October 2, 2021 PEDIATRICS Volume 140, number 5, November 2017 5 TABLE 5 CHD Considered High-risk for Maternal Morbidity and Mortality Overweight or obese patients have a 1. Patients with mechanical heart valves infective endocarditis prophylaxis and lower percent-predicted maximum 2. Single ventricle patients with Fontan anticoagulation, exercise restrictions if oxygen consumption and a higher palliation applicable, and a care plan describing blood pressure response to exertion. 3. Unrepaired or palliated cyanotic lesions the circumstances that might constitute Those who exercise experience fewer 4. Patients with Marfan syndrome and aortic an emergency, with specific guidance dilation complications. Adolescents with 5. Severe systemic AV valve regurgitation for action. This cardiology coordination greater risk knowledge35 may adopt a 6. Severe left ventricular outflow tract note would ideally be shared among all more favorable diet. Physical activity obstruction or aortic stenosis care providers and with the MH and the and nutrition counseling are important 7. Significant pulmonary hypertension family. because obesity may have unique and 8. Moderate to severe systemic ventricle Advocacy and Legislation dysfunction harmful implications in children and Adapted from Greutmann M, Pieper PG. Pregnancy in adolescentsRisk Reduction with and CHD. Transition to Adult women with congenital heart disease. Eur Heart J. Care 2015;36(37):2491–2499. AV, atrioventricular. The AAP, state AAP chapters, the ACC, and other advocacy groups aim to minimize the impact of CHD. Through their efforts, some states Fostering patient engagement by have passed legislation that ensures providing the knowledge and skills that newborn infants are screened for care participation, self-advocacy, and accessible medical information for CCHD, that students are trained career planning, and job suitability is an important in the care of patients with in lifesaving CPR before high school important role for the PCP. Substance CHD. Although cost and technology graduation, that opportunities abuse, teen-aged pregnancy, and deficiencies are barriers to universal for physical activity to reduce information regarding appropriate acceptance, many centers are espousing obesity are available, that tobacco contraception and safe sex practices are the use of EHRs in compliance with use be prevented, that insurance topics for discussion. Certain CHDs, listed the US federal mandate that includes not be denied for preexisting in Table 5, carry a high risk of maternal both incentives for implementation 36 conditions, and that public funding morbidity and mortality. When of EHR systems40,41 and penalties for for CHD research be increased. developmentally appropriate, giving nonadapters. ‍ EHRs may prove Initiatives establishing pediatric adolescents increasing responsibility useful to flag patients with CHD who patient-centered MHs are likewise for their health and encouraging will benefit from the recommendations underway. The ACC Adult Congenital provider visits without a parent for older described in this statement. For and Pediatric Cardiology Section teenagers are important goals. example, single-ventricle interstage patients may be flagged as being at a has advocated for congressional Successful transition to adult care is a high risk for mortality and morbidity support of legislation important to multidisciplinary process that begins and as susceptible to dehydration and the CHD community, such as the Advancing Care for Exceptional in the teen-aged years and is facilitated intercurrent respiratory illnesses. ’ by the AAP MH initiative aiming to This is particularly important when Kids Act of 2015, ongoing funding ensure the timely and appropriate patients present for care at new of the Children s Health Insurance conclusion of pediatric relationships institutions. Caregivers may also be Program, and funding from the prompted to provide RSV or subacute Centers for Disease Control and and a shift from childhood37 dependence “ ” to adult responsibility. It behooves bacterial endocarditis prophylaxis Prevention to support surveillance the PCP to remind the patient, family, during well-child visits if an advisory and research in CHD. The AAP and other providers that a successful is highlighted. EHR use facilitates Division of State Government transition may improve outcomes. growth and weight trending. Medical Affairs provides state-specific Older teenagers and young adults lost information portability, interoperability, information on CHD-related to follow-up have a higher incidence of and multiinstitutional sharing policies. Individual states may enact are helpful for continuity and test legislation or adopt regulations or unplanned hospital admissions via the “ § duplication avoidance. A regularly standard-of-practice guidance. emergency department, and gaps38, in39 ” Ocardiacther Endeavors care increase Important morbidity to. ‍ updated and accurate cardiology Children With CHD coordination note is ideal for multidisciplinary care. This note may Electronic Health Records contain contact information for care §For more information regarding state-specific team members, the patient diagnosis, CHD-related policies, please contact the AAP Division of State Government Affairs at stgov@ medications, baseline saturations, aap.org. Electronic health records (EHRs) may interventions performed, information provide accurate, up-to-date, legible, on implants or pacemakers, the need for Downloaded from www.aappublications.org/news by guest on October 2, 2021 6 FROM THE AMERICAN ACADEMY OF PEDIATRICS Summary when indicated Acknowledgments

members . Be Patients with CHD and their families cognizant of the risks of live- We acknowledge the help of have multiple needs. Care and virus vaccines in patients with Ms Veronica Logan for her ’ support provided by the PCP/MH, DiGeorge syndrome. Encourage administrative assistance and as outlined in the recommendations dental hygiene and adhere Ms Amy Basken, MS, of the AAP s ’ below, are invaluable for improved to endocarditis prophylaxis Family Partnership Network for her regimens when applicable; outcomes throughout the patient s Linvaluableead Author input.s life span. Recommendations 8. Anticipate other organ M. Regina Lantin-Hermoso, MD, FAAP, FACC involvement, thrombosis Stuart Berger, MD, FAAP risk, neurodevelopmental Ami B. Bhatt, MD, FACC 1. Promote care coordination and learning difficulties, Julia E. Richerson, MD, FAAP Robert Morrow, MD, FAAP and communication among the complications of therapy, and Michael D. Freed, MD, FAAP, FACC family, PCP, and subspecialists susceptibility to childhood Robert H. Beekman III, MD, FAAP, FACC at all times, but especially during illnesses; the transition from the hospital AAP Section on Cardiology and to the home or from pediatric to 9. Assist in promoting a lifestyle Cardiac Surgery Executive Committee, adult care; of good nutrition and physical 2015–2016 activity in children and L. LuAnn Minich, MD, FAAP, Chairperson 2. Advocate for infrastructure adolescents. In most cases, Michael John Ackerman, MD, PhD, FAAP support for caregivers, and exercise prescription is Stuart Berger, MD, FAAP recognize stressors that can appropriate; Robert Douglas Benjamin Jaquiss, MD, FAAP affect care across the life span; Kathy Jennifer Jenkins, MD, FAAP 10. Counsel patients against illicit William T. Mahle, MD, FAAP 3. Facilitate patient access to drug, alcohol, and tobacco use, Bradley S. Marino, MD, FAAP pediatric subspecialty care and Julie A. Vincent, MD, FAAP unprotected sex, and teen-aged medications; William Robert Morrow MD, FAAP, Immediate Past pregnancy; Chairperson 4. Be up to date on pediatric basic and advanced life support. 11. Foster self-reliance and facilitate Staff smooth transitioning to adult Encourage caregivers to undergo Vivian Thorne CPR training for patients at an health care; and increased risk of sudden death; 12. Support accuracy in EHRs and Abbreviations 5. Augment a thorough newborn use them to flag patients who history and physical examination may benefit from the above (including palpation of lower recommendations and to help AAP: American Academy of extremity pulses) with neonatal facilitate provider access to Pediatrics pulse oximetry to improve the medical information, despite ACC: American College of likelihood of recognizing CCHD, patient geographic mobility. Cardiology acknowledging differing legal Implementation AED: automated external obligations to do so in various defibrillator jurisdictions; CCHD: critical congenital heart 6. Facilitate nutritional support disease This document aims to reinforce to encourage growth and CHD: congenital heart disease best-practice recommendations as development in infants; CPR: cardiopulmonary they pertain to children with CHD, resuscitation 7. Prescribe antibiotics for asplenia, for which diagnosis codes already EHR: electronic health record seasonal RSV prophylaxis exist. Time-based coding to value MH: medical home for high-risk patients, and incremental work may be applied PCP: primary care provider influenza vaccination for to the processes outlined in this RSV: respiratory syncytial virus eligible patients and family statement.

This document is copyrighted and is property of the American Academy of Pediatrics and its Board of Directors. All authors have filed conflict of interest statements with the American Academy of Pediatrics. Any conflicts have been resolved through a process approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any commercial involvement in the development of the content of this publication.

Downloaded from www.aappublications.org/news by guest on October 2, 2021 PEDIATRICS Volume 140, number 5, November 2017 7 Policy statements from the American Academy of Pediatrics benefit from expertise and resources of liaisons and internal (AAP) and external reviewers. However, policy statements from the American Academy of Pediatrics may not reflect the views of the liaisons or the organizations or government agencies that they represent.

The guidance in this statement does not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into account individual circumstances, may be appropriate.

All policy statements from the American Academy of Pediatrics automatically expire 5 years after publication unless reaffirmed, revised, or retired at or before that time.

DOI: https://doi.org/10.1542/peds.2017-2607 Address correspondence to M. Regina Lantin-Hermoso, MD. E-mail: [email protected]

PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).

FUNDING: No external funding.

POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

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Downloaded from www.aappublications.org/news by guest on October 2, 2021 10 FROM THE AMERICAN ACADEMY OF PEDIATRICS The Care of Children With Congenital Heart Disease in Their Primary Medical Home M. Regina Lantin-Hermoso, Stuart Berger, Ami B. Bhatt, Julia E. Richerson, Robert Morrow, Michael D. Freed, Robert H. Beekman III, SECTION ON CARDIOLOGY and CARDIAC SURGERY Pediatrics originally published online October 30, 2017;

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Downloaded from www.aappublications.org/news by guest on October 2, 2021 The Care of Children With Congenital Heart Disease in Their Primary Medical Home M. Regina Lantin-Hermoso, Stuart Berger, Ami B. Bhatt, Julia E. Richerson, Robert Morrow, Michael D. Freed, Robert H. Beekman III, SECTION ON CARDIOLOGY and CARDIAC SURGERY Pediatrics originally published online October 30, 2017;

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