Landmark Articles in the World of Pediatric Cardiology and Pediatric Cardiac Surgery

Commentary by the Section on Cardiology and Cardiac Surgery

The American Academy of Pediatrics (AAP) initially established the Section of Pediatric Cardiology in November 1955 on the recommendation of the AAP Committee on Rheumatic Fever and Cardiac Disease.

Because of the extremely close relationship between pediatric cardiologists and pediatric cardiac surgeons, it was later renamed as the Section on Cardiology and Cardiac Surgery (SOCCS). The SOCCS currently has a membership of approximately 1,000. Among charter members were such luminaries as Forrest Adams, Donald Cassels, Mary Allen Engle, Benjamin Gasul, Dan McNamara, Alexander Nadas, Abraham Rudolph, Helen Taussig, and Ruth Whittemore.

The mission of the SOCCS is to improve the lives of infants, children, adolescents, and young adults with heart disease through research, advocacy, education, and interdisciplinary collaboration and to engage, educate, and support providers who care for these patients and families. It is our vision that infants, children, adolescents, and young adults with heart conditions will receive optimal care and will have the opportunity to live healthy and fulfilling lives and that the professionals who provide care for these patients will be supported, valued, and resourced to deliver excellent care.

During our review of publications in Pediatrics, we sought to identify landmark articles that brought new and important information to pediatric primary care and specialty physicians that informed their cardiac care of neonates, children, adolescents, and young adults. We also chose publications that served a critical role in advancing the quality and breadth of services that characterize the best pediatric heart centers today.

Landmark Articles in the World of Pediatric Cardiology and Pediatric Cardiac Surgery

Stuart Berger, MD, FAAP

Affiliation: Division Head Lurie Children’s Hospital, Co-Director Heart Center, Professor of Pediatrics and Medicine, Feinberg School of Medicine Northwestern University

First Quarter Century (1948 - 1973)

Highlighted Article From Pediatrics

• Bruce RA, Blackmon JR, Jones JW, Strait G. Exercise testing in adult normal subjects and cardiac patients. Pediatrics. 1963;32(4):742-756

At the time of this publication by Bruce et al, clinicians used exercise tests primarily to evaluate the status of the coronary circulation and to quantitate working capacity. Interpretation of findings required the acquisition of much normative data as a function of age, sex, physical status, and the circumstances of the test. To be effective, exercise stresses must involve large muscle masses, and the patient or subject must be ambulatory, cooperative, and properly motivated. This article reviewed some of the approaches to testing and presented preliminary observations on a new technique of exercise testing (the Bruce protocol). To this day, the Bruce protocol remains the standard by which clinical exercise testing is performed.

Second Quarter Century (1973 - 1998)

Highlighted Article From Pediatrics

• Section on Cardiology. Guidelines for pediatric diagnostic and treatment centers. Pediatrics. 1991;87(4):576-80. Updated in 2002 as Section on Cardiology and Cardiac Surgery. Guidelines for pediatric cardiovascular centers. Pediatrics. 2002;109(3):544-549

This article provided a leapfrog update to prior guidelines from 1978. For the first time, the AAP developed these guidelines in concert with those promulgated by the Joint Commission on Accreditation of Health Care Organizations with the intent that health planning agencies and health service organizations would use the guidelines to evaluate existing pediatric cardiac centers and to establish the need for new centers as well the necessary components of pediatric cardiac centers. The AAP again updated these guidelines in 2002 in the article entitled, “Guidelines for Pediatric Cardiovascular Centers.” This 2002 update encouraged organizations to incorporate diverse critical elements of cardiac diagnosis and care, including noninvasive diagnostic modalities, cardiac catheterization, cardiovascular surgery, and cardiovascular intensive care, into one integrated center so that pediatric cardiac physician specialists and specialized pediatric staff could work together to achieve the highest quality outcomes.

Third Quarter Century (1998 - 2023)

Highlighted Articles From Pediatrics

1. American College of Cardiology Foundation, American Heart Association, American Academy of Pediatrics. ACC/AHA/AAP recommendations for training in pediatric cardiology. Pediatrics. 2005;116(6):1574-1575
2. Markenson D, Pyles L, Neish S, Committee on Pediatric Emergency Medicine, Section on Cariology and Cardiac Surgery. Pediatrics. Ventricular fibrillation and the use of automated external defibrillators on children. 2007;120(5):e1368-1379
3. Section on Cardiology and Cardiac Surgery. Pediatric sudden cardiac arrest. Pediatrics. 2012;129(4):e1094-e1102
4. Erickson CC, Salerno JC, Berger S, et al. Sudden death in the young: information for the primary care provider. Pediatrics. 2021;148(1):e2021052044
5. Perrin JM, Friedman RA, Knilans TK, Black Box Working Group, Section on Cardiology and Cardiac Surgery. Cardiovascular monitoring and stimulant drugs for attention-deficit/hyperactivity disorder. Pediatrics. 2008;122(2):451-453
6. Daniels SR, Greer FR, Committee on Nutrition. Lipid screening and cardiovascular health in childhood. Pediatrics. 2008;122(1):198-208
7. Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents. Expert panel on integrated guidelines for cardiovascular health and risk reduction in children and adolescents: summary report. Pediatrics. 2011;128(suppl 5):S213-S256
8. Beekman RH 3rd, Duncan BW, Hagler DF, et al. Pathways to approval of pediatric cardiac devices in the United States: challenges and solutions. Pediatrics. 2009;124(1):e155-e162
9. Section on Cardiology and Cardiac Surgery, Section on Orthopedics. Off-label use of medical devices in children. Pediatrics. 2017;139(1):e20163439
10. Mahle WT, Newburger JW, Matherne GP, et al. Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the AHA and AAP. Pediatrics. 2009;124(2):823-836
11. Section on Cardiology and Cardiac Surgery Executive Committee. Endorsement of Health and Human Services recommendation for pulse oximetry screening for critical congenital heart disease. Pediatrics.  2012;129(1):190-192
12. Madrigal VN, Feltman DM, Leuthner SR, et al. Bioethics for neonatal cardiac care. Pediatrics. 2022;150(suppl 2):e2022056415N

Pediatric Cardiology Training

In 2005, the AAP published pediatric cardiology training recommendations jointly with the American College of Cardiology and the American Heart Association.¹ This article set forth clear curriculum standards for training categorical fellows in pediatric cardiology.

Sudden Cardiac Death in Children

In 2007, David Markelson published an important article entitled, “Ventricular Fibrillation and the Use of Automated External Defibrillators in Children.”² At the time, pediatricians were being consulted on whether AED programs should be implemented that addressed sudden cardiac arrest in children, and if so, where. This article concluded that as AED programs expand, pediatricians must advocate on behalf of children to address their unique needs. The article conveyed the necessary knowledge for pediatricians to provide guidance about, and to ensure that children are included in, AED programs. The article explained how AEDs work, included up-to-date literature regarding pediatric fibrillation and energy delivery, and provided pediatric providers with understanding of the role of AEDs as life-saving interventions for children.

In 2012, Robert Campbell was the lead author on the AAP statement “Pediatric Sudden Cardiac Arrest” that was endorsed by the American College of Cardiology, the American Heart Association, and the Heart Rhythm Society.³ The article reviewed the fact that pediatric sudden cardiac arrest (SCA), defined as the abrupt and unexpected loss of heart function, can cause sudden cardiac death (SCD) if not treated within minutes. It is well known that this entity has a profound effect on everyone: children, parents, family members, communities, and health care providers. Preventing the tragedy of pediatric SCA remained a concern to all, and the goal of this statement was to increase the knowledge base of pediatricians (including primary care providers and specialists) about the incidence of pediatric SCA, the spectrum of causes of pediatric SCA, disease-specific presentations, the role of patient and family screening, the rapidly evolving role of genetic testing, and finally, important aspects of secondary SCA prevention.

In 2021, Chris Erickson et al published updated practice guidelines for the primary care provider about SCA.⁴ This updated statement identified multiple conditions that increased risk for SCA in children. Although efforts had been made by multiple organizations to screen children for cardiac conditions, the emphasis had been on screening before athletic competition. This update recommended 4 main screening questions not just for athletes, but for all children. It also included a discussion about how to manage post-SCA and SCD situations, as well as discussion about genetic testing.

Cardiac Effects of Stimulant Medications for ADHD

 In 2008, James Perrin et al published a much referenced article on the subject of whether children with attention-deficit/hyperactivity disorder (ADHD) on stimulant medications should be screened for cardiovascular abnormalities.⁵ This article recommended that clinicians carefully assess all children for cardiac abnormalities, including those in whom ADHD treatment is being considered, by using history and physical assessment. This AAP policy statement did not recommend the routine use of ECGs before initiating stimulant therapy for ADHD and provided an algorithm developed by the SOCCS. This report was written in response to and in conjunction with an AHA article. The article stated that the AAP shared the concern of the AHA about improving the diagnosis of silent but clinically significant cardiac conditions in children and adolescents and urged additional research into effective methods to detect these conditions and reduce the incidence of SCD.

Childhood Precursors of Adult Cardiac Disease

In 2008, Steve Daniels et al published an article that reviewed the role of lipids in pediatric precursors to adult cardiac disease and the indications for lipid screening during childhood.⁶ This clinical report replaced the 1998 AAP policy on cholesterol in childhood. The report has taken on heightened urgency given the current epidemic of childhood obesity with the subsequent increasing risk of type 2 diabetes mellitus, hypertension, and cardiovascular disease in older children and adults. The approach to screening children and adolescents with a fasting lipid profile became a targeted approach as defined by that article. The article stated that overweight children belong to a special risk category of children and were in need of cholesterol screening regardless of family history or other risk factors. The report reemphasized the need for prevention of cardiovascular disease by following Dietary Guidelines for Americans and increasing physical activity. The report also included an important review of the pharmacologic agents and indications for treating dyslipidemia in children.

In 2011, Pediatrics published a summary of the deliberations of an expert panel that sought to develop comprehensive evidence-based guidelines that addressed the known risk factors for cardiovascular disease to assist all primary pediatric care providers in both the promotion of cardiovascular health and the identification and management of specific risk factors from infancy into young adult life.⁷ The article strongly argued for innovative approaches to prevention, because the antecedents of remote adult diseases (eg, atherosclerosis) begin in childhood. The recommendations of this article were designed to address the following 2 goals: the prevention of risk-factor development (primordial prevention) and the prevention of future cardiovascular disease by the effective management of identified risk factors (primary prevention). This comprehensive article offered important recommendations with regard to risk reduction.

Pediatric Cardiac Devices

In 2009, Rob Beekman et al published on the broad topic of facilitating federal approval for the use of novel pediatric cardiac devices.⁸ Patients treated by pediatric interventional cardiologists and cardiac surgeons often have unmet medical device needs that pose a challenge to the current regulatory evaluation and approval process in the United States. This report reviewed the US FDA regulatory processes, reviewed some unique aspects of pediatric cardiology and cardiac surgery that pose challenges to these processes, and discussed possible alternate pathways to cardiac device evaluation and approval for children. The authors, representing the pediatric cardiology community, advanced strong arguments that children deserved to benefit from new and refined cardiac devices and technology designed explicitly for their unique conditions.

In 2017, Kathy Jenkins et al published a study on the off-label use of medical devices in children.⁹ The study showed that despite widespread therapeutic needs, the majority of medical and surgical devices used in children did not have approval or clearance from the FDA for use in pediatric populations. The clinical need for devices to diagnose and treat diseases or conditions occurring in children had led to the widespread and necessary practice in pediatric medicine and surgery of using approved devices for “off-label” or “physician-directed” applications that were not included in FDA-approved labeling. This practice was common and often appropriate, even with the highest-risk (class III) devices. The legal and regulatory framework used by the FDA for devices was complex, and economic or market barriers to medical and surgical device development for children were quite significant. Given the need for pediatric medical and surgical devices and the challenges to pediatric device development, off-label use was and is a necessary and appropriate part of care. In addition, as discussed in this article, because of the relatively uncommon nature of pediatric conditions, FDA clearance or approval often required other regulatory pathways (eg, Humanitarian Device Exemption), which often caused confusion among pediatricians and payers about whether a specific use, even of an approved device, is considered experimental. This policy statement described the appropriateness of off-label use of devices in children and the use of devices approved or cleared through the FDA regulatory processes, including through the Humanitarian Device Exemption. It also discussed the important need to increase pediatric device labeling information for all devices and especially those that posed the highest risk to children.

Pulse Oximetry for Screening of CCHD

In 2009, Bill Mahle et al published a truly seminal article entitled, “Role of Pulse Oximetry in Examining Newborns for Congenital Heart Disease: A Scientific Statement From the AHA and AAP.”¹⁰ This article made the very important point that critical congenital heart disease in the newborn was sometimes not detected until after hospital discharge, which increased the risk for mortality and significant morbidity. In addition, they argued that routine pulse oximetry performed on apparently healthy newborns after 24 hours of life, but before hospital discharge, could detect some forms of critical cyanotic congenital heart disease (CCHD). Routine pulse oximetry performed after 24 hours in hospitals that have on-site pediatric cardiovascular services incurs very low cost and risk of harm. This article advocated for future studies in larger populations and across a broad range of newborn delivery systems to determine whether this neonatal pulse oximetry screening should become standard of care in the routine assessment of the neonate.

Three years later, in 2012, Bill Mahle et al published a follow-up article that provided AAP endorsement of the HHS recommendation to add CCHD screening to the uniform screening panel.¹¹ This recommendation stemmed from large studies that demonstrated the proof of concept of screening for detecting CCHD in newborns. The AAP had published strategies for the implementation of pulse oximetry screening, which addressed critical issues such as necessary equipment, personnel, and training, and also provided specific recommendations for assessment of saturation by using pulse oximetry as well as appropriate management of a positive screening result. Publication of this article again demonstrated that the AAP and Pediatrics are committed to the safe and effective implementation of pulse oximetry screening and will be working with other advocacy groups and governmental agencies to promote pulse oximetry and to support widespread surveillance for CCHD. In addition, the article stressed the importance for AAP chapters to partner with state health departments to implement the new screening strategy for CCHD and ensure that there will be an adequate system for referral for echocardiographic and pediatric cardiology evaluation after a positive screening result. This article encouraged AAP members to engage with state and local policy makers to adopt and fund the recommendations made by the Secretary of HHS.

Bioethics

Vanessa Madrigal was the lead author of an important 2022 article entitled, “Bioethics for Neonatal Cardiac.”¹² The care of the fetus and neonate with congenital heart disease (CHD) has become a rapidly expanding field in ways that improve survival and long-term outcomes. Much like the evolving therapeutic and prognostic challenges that accompany conditions such as congenital diaphragmatic hernia or myelomeningocele, fetal and perinatal care for CHD invokes ethical implications and dilemmas. This article discusses good ethical practice including a thorough understanding of the details and narrative of each individual case. The article reviews the 4 classic and critical principles of bioethics: autonomy, beneficence, nonmaleficence, and justice. These translate into the practices of general respect for persons, good quality shared decision-making, informed consent, transparent communication, universal approach, and recognition of bias. The aim of this article was to share recommendations that highlight practical approaches solidly grounded in ethics principles. We are increasingly cognizant that parent experiences and preferences are critical components that inform ethical processes. Therefore, the article included a parent voice to the authorship of the writing group.

Closing Thought

Although not necessarily comprehensive, and without going all the way back to 1948, this retrospective provides many examples of the importance of Pediatrics as a conduit for dissemination of critically important information to generalists as well as to pediatric cardiologists. It is the hope of the SOCCS that this collaboration will become stronger as the escalating complexity of pediatric cardiology and cardiac surgery require more policies and guidelines and timely communication of new information to all pediatric providers who care for neonates, children, and adolescents with cardiac conditions.