Andrew Hoyer

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Scholarly Contributions

Chapters

• Seckeler, M. D., Guerrero, C. E., & Hoyer, A. (2023). 3D Printing in Congenital Heart Disease. In Magnetic Resonance Imaging of Congenital Heart Disease.

Journals/Publications

• Caryl, N. E., June, C., Culbert, M., Hellinger, R. D., Hoyer, A. W., Klewer, S. E., & Seckeler, M. D. (2024). Incidence of radiation-associated cancer in patients with congenital heart disease. American Journal of Cardiology. doi:10.1016/j.amjcard.2023.10.030
• Meziab, O., Dereszkiewicz, E., Guerrero, C., Hoyer, A., Barber, B., Klewer, S., & Seckeler, M. (2024). Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set. Pediatric Cardiology. doi:10.1007/s00246-024-03500-1
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  Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. “Low Performers” were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and “Normal Performers” were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.
• Dereszkiewicz, E., Meziab, O., Guerrero, C. E., Hoyer, A. W., Barber, B. J., Klewer, S. E., & Seckeler, M. D. (2023). NEGATIVE EFFECT OF BUNDLE BRANCH BLOCK ON EXERCISE PERFORMANCE IN PATIENTS WITH FONTAN PHYSIOLOGY: FROM THE PEDIATRIC HEART NETWORK FONTAN PUBLIC DATA SET. JACC. doi:10.1016/s0735-1097(23)01969-1
• Meziab, O., Hoyer, A. W., Fox, K. A., & Seckeler, M. D. (2023). 3-Dimensional Printing for Planning for Transvenous Pacemaker Placement in Complex Congenital Heart Disease. JACC. Clinical electrophysiology, 9(8 Pt 1), 1433-1435.
• Caryl, N. E., June, C., Culbert, H., Hellinger, R. D., Hoyer, A. W., Klewer, S. E., & Seckeler, M. D. (2022). Abstract 11061: Incidence of Cancer in Hospitalized Adults With Congenital Heart Disease. Circulation, 146(Suppl_1). doi:10.1161/circ.146.suppl_1.11061
• Seckeler, M. D., Barber, B. J., Colombo, J. N., Bernardi, A. M., Hoyer, A. W., Andrews, J. G., & Klewer, S. E. (2021). Exercise Performance in Adolescents With Fontan Physiology (from the Pediatric Heart Network Fontan Public Data Set). The American journal of cardiology, 149, 119-125.
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  In the pediatric population, exercise capacity differs between females and males and the gap widens through adolescence. However, specific age- and sex-based changes in adolescents with congenital heart disease and Fontan palliation have not been reported. The purpose of the current study is to identify age- and sex-specific changes in exercise performance at peak and ventilatory anaerobic threshold (AT) for adolescents with Fontan physiology. Retrospective review of the Pediatric Heart Network Fontan cross sectional study (Fontan 1) public use dataset. Comparisons were made for peak and AT exercise parameters for females and males at 2-year age intervals. In addition, normative values were generated by sex and age at 2-year intervals. χ test was used for comparison for categorical variables. Changes in exercise parameters between age groups by sex were compared by ANOVA with post-hoc analysis. Exercise testing was performed in 411 patients. AT was reached in 317 subjects (40% female), of whom, 166 (43% female) reached peak exercise. Peak oxygen consumption decreased 32% through adolescence in females and did not have the typical increase through adolescence for males. Oxygen consumption at AT also decreased with age in both sexes. In conclusion, age- and sex-based exercise performance for adolescents with Fontan physiology are predictably low, but there are additional significant decreases through adolescence for this population, especially in females. We have established normative exercise values for several parameters for this population which will better identify at risk patients and allow for earlier intervention.
• Strah, D., Kowalek, K., Weinberger, K., Mendelson, J., Hoyer, A. W., Klewer, S. E., & Seckeler, M. D. (2021). Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease. Pediatric Cardiology. doi:10.1007/s00246-021-02751-6
• Mauro, D. M., Flors, L., Hoyer, A. W., Norton, P. T., & Hagspiel, K. D. (2015). Development of bilateral coronary artery aneurysms in a child with Noonan syndrome. Pediatric Radiology. doi:10.1007/s00247-015-3472-z
• Minette, M. S., Hoyer, A. W., Pham, P. P., DeBoer, M. D., Reller, M. D., & Boston, B. A. (2013). Cardiac Function in Congenital Adrenal Hyperplasia: A Pattern of Reversible Cardiomyopathy. Journal of Pediatrics. doi:10.1016/j.jpeds.2012.11.086
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  To evaluate cardiac function in infants with congenital adrenal hyperplasia (CAH) before and after corticosteroid replacement therapy.This prospective, case-control study included 9 infants with CAH. Cardiac function was assessed by echocardiography at presentation and after corticosteroid replacement therapy. Six term infants underwent 2 echocardiograms each and served as the control group. Data on fractional shortening (FS), rate-corrected velocity of circumferential fiber shortening (Vcf), wall stress, tissue Doppler indices, myocardial performance index, left ventricular mass, and Vcf/wall stress were obtained.The infants with CAH exhibited myocardial dysfunction at baseline and lower systolic blood pressure (SBP) compared with the control group. FS, a measure of systolic contractility, differed significantly from before to after corticosteroid treatment (mean, 32.3%±4.7% pretreatment, 39.9%±5.0% posttreatment). Vcf, a preload-independent measure of cardiac contractility, also differed significantly before and after treatment (mean, 1.23±0.16 circumferences/second pretreatment, 1.45±0.22 circumferences/second posttreatment). SBP was also lower (mean, 84±9.3 mmHg) and improved with treatment (mean, 95±4.8 mmHg). The control group demonstrated no statistically significant changes in FS, Vcf, or SBP. There was a change in left ventricular mass in the control group between the 2 studies.Newborns with CAH have evidence for cardiac dysfunction at baseline that reverses with corticosteroid replacement therapy. These data suggest that corticosteroids play a direct role in modulating cardiac function in the newborn.
• Minette, M., Hoyer, A., Pham, P., Deboer, M., Reller, M., & Boston, B. (2013). Cardiac function in congenital adrenal hyperplasia: A pattern of reversible cardiomyopathy. Journal of Pediatrics, 162(6). doi:10.1016/j.jpeds.2012.11.086
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  Objective To evaluate cardiac function in infants with congenital adrenal hyperplasia (CAH) before and after corticosteroid replacement therapy. Study design This prospective, case-control study included 9 infants with CAH. Cardiac function was assessed by echocardiography at presentation and after corticosteroid replacement therapy. Six term infants underwent 2 echocardiograms each and served as the control group. Data on fractional shortening (FS), rate-corrected velocity of circumferential fiber shortening (Vcf), wall stress, tissue Doppler indices, myocardial performance index, left ventricular mass, and Vcf/wall stress were obtained. Results The infants with CAH exhibited myocardial dysfunction at baseline and lower systolic blood pressure (SBP) compared with the control group. FS, a measure of systolic contractility, differed significantly from before to after corticosteroid treatment (mean, 32.3% ± 4.7% pretreatment, 39.9% ± 5.0% posttreatment). Vcf, a preload- independent measure of cardiac contractility, also differed significantly before and after treatment (mean, 1.23 ± 0.16 circumferences/second pretreatment, 1.45 ± 0.22 circumferences/second posttreatment). SBP was also lower (mean, 84 ± 9.3 mmHg) and improved with treatment (mean, 95 ± 4.8 mmHg). The control group demonstrated no statistically significant changes in FS, Vcf, or SBP. There was a change in left ventricular mass in the control group between the 2 studies. Conclusion Newborns with CAH have evidence for cardiac dysfunction at baseline that reverses with corticosteroid replacement therapy. These data suggest that corticosteroids play a direct role in modulating cardiac function in the newborn. Copyright © 2013 Mosby Inc.
• Seckeler, M., D'Souza, M., Gangemi, J., Hoyer, A., & Anitha Jayakumar, K. (2013). Thrombotic occlusion of extracardiac conduit: 4 months after fontan surgery. Texas Heart Institute Journal, 40(3).
• Clarke, C. J., Gurka, M. J., Norton, P. T., Kramer, C. M., & Hoyer, A. W. (2012). Assessment of the Accuracy and Reproducibility of RV Volume Measurements by CMR in Congenital Heart Disease. JACC: Cardiovascular Imaging. doi:10.1016/j.jcmg.2011.05.007
• Clarke, C., Gurka, M., & Hoyer, A. (2012). Reply. JACC: Cardiovascular Imaging, 5(6). doi:10.1016/j.jcmg.2012.04.003
• Vergales, J., West, S. C., & Hoyer, A. W. (2012). Pulmonary Vein Atresia with Severe Contralateral Pulmonary Vein Stenosis in a Child. Pediatric Cardiology. doi:10.1007/s00246-012-0178-y
• Clarke, C. J., Jayakumar, K., & Hoyer, A. W. (2010). Anatomically Corrected Malposition of the Great Arteries. Pediatric Cardiology. doi:10.1007/s00246-009-9618-8
• Law, Y. M., Hoyer, A. W., Reller, M. D., & Silberbach, M. (2010). Reply. JACC. doi:10.1016/j.jacc.2009.12.017
• Law, Y., Hoyer, A., Reller, M., & Silberbach, M. (2010). Reply. Journal of the American College of Cardiology, 55(11). doi:10.1016/j.jacc.2009.12.017
• Law, Y., Hoyer, A., Reller, M., & Silberbach, M. (2009). Accuracy of Plasma B-Type Natriuretic Peptide to Diagnose Significant Cardiovascular Disease in Children. The Better Not Pout Children! Study. Journal of the American College of Cardiology, 54(15). doi:10.1016/j.jacc.2009.06.020
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  Objectives: The purpose of this study was to assess the ability of plasma B-type natriuretic peptide (BNP) to diagnose significant cardiovascular disease (CVD) in the pediatric population. Background: BNP has been shown to be reliable in detecting ventricular dysfunction and heart failure in adults. Timely and accurate identification of significant pediatric heart disease is important but challenging. A simple blood test could aid the front-line physician in this task. Methods: Subjects without a history of heart disease with findings possibly attributable to significant CVD in the acute care setting requiring a cardiology consult were enrolled. Clinicians were blinded to the BNP result, and confirmation of disease was made by cardiology consultation. Results: Subjects were divided into a neonatal (n = 42, 0 to 7 days) and older age group (n = 58, >7 days to 19 years). CVD was present in 74% of neonates and 53% of the older age group. In neonates with disease, median BNP was 526 pg/ml versus 96 pg/ml (p < 0.001) for those without disease. In older children with disease, median BNP was 122 pg/ml versus 22 pg/ml in those without disease (p < 0.001). Subjects with disease from an anatomic defect, a longer hospital stay, or who died had higher BNP. A BNP of 170 pg/ml yielded a sensitivity of 94% and specificity of 73% in the neonatal group and 87% and 70% in the older age group, respectively, using a BNP of 41 pg/ml. Conclusions: BNP is a reliable test to diagnose significant structural or functional CVD in children. Optimal cutoff values are different from adult values. © 2009 American College of Cardiology Foundation.
• Hoyer, A., & Balaji, S. (2007). The safety and efficacy of ibutilide in children and in patients with congenital heart disease. PACE - Pacing and Clinical Electrophysiology, 30(8). doi:10.1111/j.1540-8159.2007.00799.x
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  Background: The safety and efficacy of ibutilide in the cardioversion of atrial flutter and atrial fibrillation in children and in patients with congenital heart disease (CHD) is unknown. Methods: Data from 19 patients (age 6 months to 34 years, median 16 years) who received ibutilide for atrial flutter or atrial fibrillation between 1996 and 2005 was retrospectively reviewed. There were 15 patients with CHD (14 had prior heart surgery); four children had normal heart structure. Results: There were 74 episodes of atrial flutter and four episodes of atrial fibrillation (median episodes per patient was one, range 1-31). Ibutilide converted 55 of all the episodes (71%). Ibutilide was successful during its first-ever administration in 12 of 19 patients (63%). Fourteen episodes in six patients required electrical cardioversion after ibutilide failed. There were no episodes of symptomatic bradycardia. One patient went into torsade de pointes and one patient had nonsustained ventricular tachycardia. Conclusion: With careful monitoring, ibutilide can be an effective tool in selected patients for cardioversion of atrial flutter. © 2007, The Authors.
• Pham, P. P., Hoyer, A. W., Shaughnessy, R., & Law, Y. M. (2006). A Novel Approach Incorporating Sildenafil in the Management of Symptomatic Neonates with Ebstein’s Anomaly. Pediatric Cardiology. doi:10.1007/s00246-006-1203-9
• Hoyer, A. W., & Silberbach, M. (2005). Infective Endocarditis. Pediatrics In Review. doi:10.1542/pir.26.11.394
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  DefinitionsEndarteritis:Infection of the lining of blood vessels (eg, in a patient who has postoperative coarctation of the aorta)Endocarditis:Inflammation of the valvular or mural endocardium, whether from an infection or from a noninfectious inflammatory processInfective endocarditis:Endocarditis caused by microorganisms (bacteria or fungi) involving either the heart or the great vessels (ie, endarteritis); the pathology also may include abscess formationMycotic aneurysm:A term applied to fungal or bacterial infection within the wall of a vesselMycotic endocarditis:Endocarditis caused by fungusAfter completing this article, readers should be able to: Apreviouslyhealthy 4-year-old boy refuses to walk. He has a 7-day history of fever and fatigue. His temperature is 101.3°F (38.5°C), and his heart rate is 120 beats/min. He has a new systolic heart murmur. His right knee is warm and mildly swollen. He is admitted to the hospital for evaluation and treatment of possible septic arthritis and osteomyelitis. A blood culture is drawn the first day. The orthopedic surgeon drains fluid from the right knee. The next day, he remains febrile, the murmur persists, and gram-positive cocci grow from his blood and joint fluid. A cardiology consultation is requested to rule out endocarditis.A 12-year-old boy who has cyanosis and tetralogy of Fallot and last underwent surgery at the age of 4 years presents to the emergency department with a fever and appearing acutely ill. He has a narrowed conduit from the right ventricle to the pulmonary artery and an unrepaired ventricular septal defect. One week prior to admission, his mother pierced his ear at home. The ear subsequently became inflamed and swollen. After his initial presentation, his condition rapidly deteriorates, and he requires intubation and inotropic support. An echocardiogram demonstrates a mobile mass within the homograft conduit. Multiple blood cultures are obtained before broad-spectrum antibiotics are initiated. Staphyloccocus aureus grows from the blood in fewer than 12 hours. Despite blood cultures obtained on subsequent days being sterile, his condition worsens. He is taken to the operating room, and the conduit is replaced. His recovery is uneventful.Few problems in pediatrics cause as much consternation as heart murmurs or positive blood cultures; their nexus raises concern for infective endocarditis (IE). This review provides the pediatrician with an evidence-based and practical framework for the evaluation and management of the febrile child who has a murmur.The epidemiology of endocarditis has changed in the modern era. Although it remains a rare diagnosis, the rate of IE may be increasing, and it is a frequent concern among pediatricians. The prevalence of rheumatic heart disease has decreased, and a population of patients who survive beyond infancy with severe congenital cardiac malformations has emerged. Importantly, 90% of IE cases occur in individuals who have heart disease, usually congenital. In fact, IE may be the presenting sign of a bicuspid aortic valve. The increased use of invasive procedures in neonatal and pediatric intensive care units, however, has placed individuals whose hearts are structurally normal at risk.Although most cases of IE are not procedure-related, certain procedures, such as dental extraction, rigid bronchoscopy, and tonsillectomy/adenoidectomy, are believed to increase the risk for IE in susceptible individuals, as do atopic dermatitis, acne, and poor dental hygiene. The American Heart Association considers a number of procedures to be low risk for IE, including restorative dentistry (when bleeding of the gums does not occur), local anesthetic injection in the mouth, tympanostomy tube insertion, endotracheal intubation, gastrointestinal endoscopy, urethral catheterization in uninfected tissue, cardiac catheterization, and circumcision. (A complete list is available at http://circ.ahajournals.org/cgi/content/full/96/1/358.)Bacteremia is relatively common; IE is not. IE tends to occur when bacteremia that has a causative organism is present in the setting of damaged cardiac or vascular endothelium. Bacteremia occurs in the postoperative setting; in immunocompromised patients; and in nonhospital settings such as after tooth-brushing, tattooing, body piercing, and intravenous street-drug use.Gram-positive cocci are the most likely pathogens, although gram-negative rods and fungi can cause IE. Gram-positive cocci have a predilection for subendocardial connective tissue, especially fibronectin, that is exposed when endocardium is disrupted. Virulence factors and a unique “cytokine milieu,” however, can cause other bacterial species to adhere to intact endocardium.Congenital heart disease (postsurgical or unrepaired) is a major risk factor for IE. For individuals undergoing evaluation for IE who do not have previously known heart disease, bicuspid aortic valve is the most common newly discovered cardiac malformation. Jets of blood spurting through a regurgitant mitral valve, a patent ductus arteriosus, or a ventricular septal defect can damage the endothelium and deposit bacteria at the site of injury. For unknown reasons, disrupted endocardium usually occurs at the low-pressure side of the jet (except in aortic stenosis, in which the vegetations occur on the ventricular side of the leaflets).Foreign materials such as homograft tissue and Gore-Tex® also can serve as infectious loci in postoperative patients. Central lines that protrude into the right atrium or cross the tricuspid valve annulus can damage endocardial tissue.The current theory of pathogenesis is that exposed fibronectin at the site of injury induces the clotting cascade and fibrin deposition. The blood stream delivers microbes into the clot, where they thrive in a relatively avascular environment. The original nidus of infection grows into a vegetation that encases the bacteria inside an organized, often calcified, mass. Vegetations that occur on valve leaflets can be very destructive, causing valve regurgitation and heart failure. Pieces of bacteria-containing vegetation may embolize to the lungs, brain, kidney, or extremities. Bacteria can infiltrate deeper tissues of the heart or arteries, causing abscesses.Noncardiac manifestations of IE, originally believed to be due to vegetative emboli, now are recognized as immunologic phenomena. Circulating immune complexes, rheumatoid factor, and other evidence of inflammation persist even after the blood is sterilized, and their disappearance can serve as a marker of successful therapy.In one prospective study of patients who had congenital heart disease, Streptococcus viridans and S aureus both were found in 23% of the total of culture-positive IE cases, followed by beta-hemolytic streptococci (9%) and Staphylococcus epidermidis (9%). Gram-negative organisms cause IE less commonly, probably due to their inability to bind fibronectin. The gram-negative rods that cause IE are the so-called “HACEK” organisms (Haemophilus sp, Actinobacillus [Haemophilus] actinomycetemcomitans, Cardiobacterium hominis, Eikenella sp, and Kingella kingae). Fungi also can cause infective endocarditis. The most common fungus is Candida, followed by Aspergillus.The diagnosis of IE should be considered in any child who has unexplained fever and is known to have heart disease. IE, outside of the immediate postoperative period, usually presents as an indolent disease, so-called “subacute bacterial endocarditis.” Fulminant IE, as presented in Case 2, has been called acute bacterial endocarditis. These terms are employed less commonly as physicians have sought to describe the specific cause of the infection rather than a general syndrome. Besides fever, common symptoms include myalgia, arthralgia, headache, and generalized malaise. A history of anorexia and weight loss is common. The classic signs, such as Roth spots, Janeway lesions, and Osler nodes, are very rare in children (Table 1). Splinter hemorrhages also are rare and nonspecific. Almost all patients who have IE have a murmur, although it may be an innocent murmur caused by turbulent flow through a left ventricular outflow tract during the high-output state associated with a severe systemic illness. (See Table 1 for an estimated frequency of presenting signs.)The definitive diagnosis of IE is elusive and often requires input from pediatricians, infectious disease specialists, laboratory microbiologists, and cardiologists. A variety of worrisome factors affects the clinician’s attempts to make this diagnosis: heightened concern for missing or not treating a condition that has a potentially fatal outcome, the potential need for weeks or months of intravenous therapy, the uncertainty in diagnosing culture-negative endocarditis, and the high prevalence of innocent murmurs in children during illness.The lack of a consistent, evidence-based method to establish the diagnosis of IE prompted the creation of the Duke Criteria (Tables 2 and 3). This system employs a combination of clinical, microbiologic, and echocardiographic criteria to determine the likelihood of IE similar to the use of the Jones Criteria in the diagnosis of rheumatic fever. The utility of the Duke criteria in diagnosing IE in pediatric patients has been established. Effective blood culturing technique is key to the successful diagnosis of IE using the Duke Criteria (Table 4).The echocardiographic findings that constitute IE in the Duke scheme are: 1) vegetation on a valve or supporting structure (FigureF1), 2) abscess, or 3) new partial dehiscence of a prosthetic valve. Because these findings are relatively rare in children, the diagnosis often must be considered in the absence of echocardiographic evidence. Indeed, when the overall suspicion for IE is low, echocardiography is of little value.Transesophageal echocardiography (TEE) is of proven utility in adults, in whom acoustic penetration often is limited by a thick chest wall or by the air-filled lungs. Such problems are less common in children, in whom TEE should be reserved for those who have poor echo windows, in whom prosthetic valves cause “shadowing,” and in whom the transthoracic echocardiography (TTE) view appears normal despite a high index of suspicion for IE.Retrospective studies suggest that blood cultures may be persistently negative in approximately 5% of cases of IE. In such a situation, numerous blood cultures, prolonged culture periods, studies of surgically isolated vegetations or emboli, and serologic investigations (antibody titers and polymerase chain reaction tests) fail to identify the offending organism. Potential reasons for false-negative blood cultures are many: The diagnosis of culture-negative endocarditis depends on a high degree of suspicion and indirect evidence. Therefore, a patient who has congenital heart disease and suggestive symptoms should be studied aggressively. Cardiac magnetic resonance imaging, radiolabeled white blood cell scans, or other types of nuclear imaging may be helpful.The Duke Criteria were designed to standardize diagnosis, but they provide little guidance for management. Although antibiotics are recommended for individuals who have “definite” IE, the need for treating “possible” or even “rejected” IE is not excluded in that scheme. The diagnosis and treatment of IE must be determined case by case. Management depends on assessing the reliability of the blood cultures, searching for underlying heart disease, and occasionally on determining, rather arbitrarily by consensus, the presence or absence of IE.If the patient who presumably has IE is ill and the pediatrician feels that antibiotics should be started before blood culture results are available, empiric treatment should be directed at the most common offenders: streptococci and staphylococci. A standard regimen is penicillin or ampicillin (or vancomycin, if allergic to penicillin) plus gentamicin. If an organism is isolated from the blood, susceptibilities should be determined. Emerging resistance patterns or elusive bacteria often complicate antibiotic choice, and help from infectious disease specialists is needed, especially for enterococcal infections. Therapy must be intravenous to attain persistently high bactericidal concentrations in relatively avascular valve leaflets and to penetrate infected thrombi. The usual course of therapy is 4 to 6 weeks, but infection of prosthetic valves and tissue may require longer treatment. New, as yet unvalidated, therapies include cyclosporine, linezolid, and teicoplanin (similar to vancomycin). There are intriguing reports of the successful use of recombinant tissue plasminogen activator to lyse intracardiac vegetations in severely ill infants. The investigators offered the hope of avoiding surgery in persistently symptomatic neonates.Indications for surgery during the acute phase of IE are continued bacteremia after 2 weeks of appropriate therapy, fungal vegetations, abscess formation, worsening heart failure (due to valvular regurgitation caused by ruptured leaflets or chordae), or systemic emboli. Patients eventually may require surgical intervention for chronic valvular stenosis or regurgitation caused by previous IE. IE related to a hemodynamically trivial ventricular septal defect warrants surgical repair following successful treatment of the infection (FigureF1).In Case 1, a boy has a new murmur and bacteremia. In this setting, the Duke Criteria help assess the likelihood of IE. The murmur represents a major criterion if it signifies valve disease. It may, however, be an innocent flow murmur brought about by a fever-caused hyperdynamic state. Fever and a single positive blood culture both are minor criteria. These findings, however, may be sufficient to establish the diagnosis of “possible” IE by the Duke criteria if the murmur is, in fact, significant. Consultation with a pediatric cardiologist is warranted to help determine if the murmur represents structural heart disease. TTE should be obtained if findings on the physical examination suggest valvular disease. Continued antibiotic therapy for the septic joint and possible osteomyelitis is warranted. If the murmur is found to be innocent, the pediatrician, cardiologist, and infectious disease specialist need to reassess the patient if the fever persists or if any other signs or symptoms of IE develop.In Case 2, the presentation of a patient who has congenital heart disease, has a fever, and appears ill suggests fulminant IE, which is in contrast to the usual indolent presentation. Patients at high risk for IE include those who have tetralogy of Fallot or other complex cyanotic congenital heart diseases, prosthetic cardiac valves (including bioprosthetic and homograft valves), and flow jets through narrowed areas. Unsterile body piercing is dangerous for anyone; in the setting of congenital heart disease, however, it is potentially lethal. Ear piercing, often performed with suspect sterile technique in shopping malls, should be avoided in at-risk individuals. In this case, TTE quickly demonstrated the vegetation, verifying the diagnosis under the Duke Criteria, and appropriate treatment was initiated. The decision to proceed to surgery is always difficult and usually requires considerable effort by the nonsurgical caregivers to convince the surgeons to take an acutely ill and obviously infected patient to the operating room. In this child, continued deterioration despite appropriate medical therapy warranted a surgical approach. Evidence is substantial that in specific circumstances, surgical intervention in acute IE can be lifesaving.The rationale behind antibiotic prophylaxis for IE is that certain medical or dental procedures can cause bacteremia and lead to endocarditis in patients who have heart disease and that antibiotics administered at the time of the procedure can prevent the disease. The guidelines published by the American Heart Association (AHA) for prophylaxis serve as the basis for treatment decisions by caregivers. It should be recognized, however, that direct evidence to support these recommendations is limited. According to the AHA, procedures that are likely to produce bacteremia involving IE-causing pathogens in susceptible patients should be targeted for antibiotic prophylaxis. Surgery that involves any mucosal surface or infected tissue is considered a risk. An important exception is heart surgery itself, in which antibiotic prophylaxis typically is given. The antibiotic regimen for prophylaxis depends on the procedure. Incision of oral, respiratory, or esophageal mucosa requires an antibiotic that is effective against viridans streptococci, making amoxicillin the first choice. Incision of intestinal or genitourinary mucosa requires a regimen effective against enterococcus; thus, intravenous ampicillin and gentamicin are used. The AHA’s specfic recommendations have been published by Dajani and associates (see Suggested Reading).The course of IE can be complicated by embolization to virtually any organ, depending on whether the disease involves the right or left side of the heart. Other problems include abscess formation, heart failure, heart block, and mycotic aneurysms. In one relatively recent study of pediatric IE, 13 of 73 patients died. Complications included valvular insufficiency (18%), surgical interventions (15%), pulmonary embolus (10%), arrhythmia (8%), stroke (8%), congestive heart failure (7%), seizures (4%), renal abscess (3%), and osteomyelitis (1%). Patients who have the highest risk for complications include those who have prosthetic valves, left-sided IE, S aureus or fungal IE, prior episodes of IE, duration of symptoms greater than 3 months, cyanotic heart disease, systemic artery-to-pulmonary artery shunts, and poor clinical response to antibiotics.IE is an often considered, yet rare, diagnosis in children. The Duke criteria help guide the diagnosis and initial evaluation, but a high index of suspicion in persistently febrile patients is necessary. Pediatric cardiologists, infectious disease specialists, and cardiothoracic surgeons can aid in management.

Proceedings Publications

• Chandra, S., Meziab, O., Caryl, N. E., Hoyer, A. W., Chatterjee, A., & Seckeler, M. D. (2023). O-1 | 3D Printing of Patient-Specific Intravascular Stents for Congenital Heart Disease – Proof of Concept. In SCAI.
• Hoyer, A. W., Ratchford, T. L., & Yu, J. (2011). Strain and strain rate analysis of the right ventricle in patients with hypoplastic left heart syndrome. In SCMR.
• Saunders, C., Jayakumar, K., & Hoyer, A. W. (2010). OP12.07: Velocity vector imaging of longitudinal function, strain and strain rate in the right ventricle of the fetus with hypoplastic left heart syndrome. In 20th World Congress on Ultrasound in Obstetrics and Gynecology.
• Yu, J., Salerno, M., & Hoyer, A. W. (2010). Vector Velocity Imaging can be used to analyze function and dyssynchrony of the right ventricle in patients with tetralogy of Fallot. In Society for Cardiovascular Magnetic Resonance.
• Hoyer, A. W., Shen, I., Ungerleider, R. M., Reed, R. D., Welke, K. F., Pham, P. P., Sahn, D. J., & Balaji, S. (2005, May). Dual-site versus single-site ventricular pacing in patients with single ventricle physiology. In Heart Rhythm Society.

Presentations

• Seckeler, M., Chatterjee, A., Meziab, O., Camarena, M., Chandra, S., Yaddanapudi, K., Hoyer, A., & Caryl, N. (2023, May). Not every TIA is a PFO – the importance of other shunts. The Society for Cardiovascular Angiography and Interventions 2023 Scientific Sessions. Phoenix, Arizona.
• Seckeler, M., Klewer, S. E., Barber, B. J., Hoyer, A., Guerrero, C. E., Meziab, O., & Dereszkiewicz, E. (2023, March). Negative Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology (from the Pediatric Heart Network Fontan Public Data Set) . American College of Cardiology ACC.23/WCC Scientific Sessions. New Orleans, Louisiana.
• Seckeler, M., Hoyer, A., Kowalek, K., Weinberger, K., & Strah, D. D. (2021, September). Enalapril improves pulmonary artery pressure for infants with single systemic right ventricles – Analysis of the Pediatric Heart Network Infant Single Ventricle public use dataset. Pediatric and Adult Interventional Cardiac Symposium 2021. Las Vegas, Nevada.

Poster Presentations

• Maxfield, K., Melcher, L., Bottrill, K., Provencio-Dean, N., Hoyer, A., Seckeler, M., Klewer, S. E., Edgin, J., & Combs, D. A. (2024, June). Insomnia, Mood, and Quality of Life in Children and Young Adults with Congenital Heart Disease. SLEEP 2024. Houston.
• Seckeler, M., Hoyer, A., Klewer, S. E., Barber, B. J., & Chatterjee, A. (2024, September). 3D modelling to plan transcatheter closure of an inferior sinus venosus defect. Pediatric and Adult Interventional Cardiac Symposium 2024. San Diego.
• Yatsenko, V., Mathena, S., Andrews, J. G., Barber, B. J., Hoyer, A., Fox, K., Meziab, O., Guerrero, C., Klewer, S. E., & Seckeler, M. (2024, November). Healthcare utilization trends for adolescents and young adults with moderate and severe congenital heart disease. American Heart Association 2024 Scientific Sessions. Chicago.
• Caryl, N., Culbert, H., June, C., Hellinger, R., Hoyer, A., Klewer, S. E., & Seckeler, M. (2022, November). Incidence of Cancer in Hospitalized Adults with Congenital Heart Disease. Arizona American College of Cardiology 2022 Annual Meeting. Scottsdale, AZ.
• Hoyer, A., Pariury, H., Seckeler, M., Goeken, B., & Shah, M. (2022, October). Evaluation of change in QTc interval in children and young adults with cancer. First International Pediatric Cardio-Oncology Conference. Cincinnati, OH.
• Seckeler, M., Klewer, S. E., Hoyer, A., Hellinger, R., June, C., Culbert, H., & Caryl, N. (2022, November). Incidence of Cancer in Hospitalized Adults with Congenital Heart Disease. American Heart Association 2022 Scientific Sessions. Chicago, IL.