Faculty Bibliography

OBJECTIVE:Perioperative corticosteroids have been used for pediatric cardiac surgery for decades, but the underlying evidence is conflicting. We aimed to investigate the efficacy and safety of perioperative prophylactic corticosteroids in pediatric heart surgeries. METHODS:We searched electronic databases until March 2023 to retrieve all randomized controlled trials (RCTs) that administered perioperative prophylactic corticosteroids to children undergoing heart surgery. We used RevMan 5.4 to pool risk ratios (RRs) and mean differences (MDs). RESULTS: = 0%; moderate certainty). There was no increase in adverse events except a higher risk of hyperglycemia and postoperative insulin use. CONCLUSIONS:The use of perioperative corticosteroids in pediatric heart surgeries is associated with a trend toward reduced all-cause mortality without attaining statistical significance. Corticosteroids reduced MV duration, and probably decrease the incidence of LCOS, and reoperations. The choice of corticosteroid agent and dose is highly variable and further larger studies may help determine the ideal agent, dose, and patient population for this prophylactic therapy.

BACKGROUND:Early detection of cardiac allograft rejection is crucial for post-transplant graft survival. Despite the progress made in immunosuppression strategies, acute cellular rejection remains a serious complication during and after the first post-transplant year, and there is a continued lack of consensus regarding its treatment, especially in pediatric transplant patients. METHODS:An open request was placed via the listserv to the membership of the Pediatric Heart Transplant Society (PHTS). Along with a broad literature search, numerous institutional protocols were pooled, analyzed and consolidated. A clinical approach document was generated highlighting areas of consensus and practice variation. RESULTS:The clinical approach document divides cellular rejection by International Society for Heart and Lung Transplantation grades and provides management strategies for each, including persistent cellular rejection. CONCLUSIONS:Cellular rejection treatment can be tailored to the clinical status, graft function, and the grade of cellular rejection. A case of mild and asymptomatic rejection may not require treatment, whereas a higher-grade rejection or rejection with graft dysfunction or hemodynamic compromise may require aggressive intravenous therapies, changes to maintenance immunosuppression therapy and augmented surveillance.

Mechanical circulatory support with durable continuous-flow ventricular assist devices has become an important therapeutic management strategy for patients with advanced heart failure. As more patients have received these devices and the duration of support per patient has increased, the postimplantation complications have become more apparent, and the need for approaches to manage these complications has become more compelling. Continuous-flow ventricular assist devices, including axial-flow and centrifugal-flow pumps, are the most commonly used mechanical circulatory support devices. Continuous-flow ventricular assist devices and the native heart have a constant physiological interplay dependent on pump speed that affects pressure-flow relationships and patient hemodynamics. A major postimplantation complication is cerebrovascular vascular accidents. The causes of cerebrovascular vascular accidents in ventricular assist device recipients may be related to hypertension, thromboembolic events, bleeding from anticoagulation, or some combination of these. The most readily identifiable and preventable cause is hypertension. Hypertension management in these patients has been hampered by the fact that it is difficult to accurately measure blood pressure because these ventricular assist devices have continuous flow and are often not pulsatile. Mean arterial pressures have to be identified by Doppler or oscillometric cuff and treated. Although guidelines for hypertension management after ventricular assist device implantation are based largely on expert consensus and conventional wisdom, the mainstay of treatment for hypertension includes guideline-directed medical therapy for heart failure with reduced ejection fraction because this may reduce adverse effects associated with hypertension and increase the likelihood of favorable ventricular remodeling. The use of systemic anticoagulation in ventricular assist device recipients may at a given blood pressure increase the risk of stroke.

T-wave inversions (TWI) in inferolateral electrocardiogram (ECG) leads (II, III, aVF, V5-V6) are often suggestive of cardiac pathology in adult patients. They are a common reason of additional testing in pediatric patients. The goal of the study is to determine correlation of exercise stress test T-wave response with diagnosis of cardiac pathology. This is a retrospective review of pediatric patients < 21 years with the finding of baseline TWI in inferior (II, III, avF) and/or lateral (V5-V6) leads on 12-lead ECG. All patients underwent treadmill exercise stress test (EST) and an echocardiogram within 1 year of each other. Demographics, baseline ECG findings, echocardiogram results, and EST data were recorded. T-wave reversion was considered complete if T waves demonstrated normalization during exercise, partial if there was minimal improvement, and no response if there was no change or worsening of inversions. In our cohort of 72 patients with a mean age 14.6 (± 2.9) years and 61% males, 59 (82%) had a structurally normal heart. Thirteen patients had evidence of structural or functional heart disease. Of the 59 patients, 83% had either complete or partial T-wave response. Among the 13 patients with heart disease, two patients had genetic testing consistent with risk for hypertrophic cardiomyopathy and had complete and partial response on EST. Exercise stress testing for TWI in pediatric patients has low sensitivity and specificity for the diagnosis of cardiac disease and routine use in this patient population may not be indicated.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

The field of pediatric ventricular assist device (VAD) support has expanded significantly over the past 20 years, with one third of pediatric heart transplant recipients currently being bridged to transplant with a VAD. Despite increased pediatric VAD utilization, however, there remains little formalized guidance for patient or device selection. The population of children with advanced heart failure is quite heterogeneous, and the available data suggest that VAD outcomes vary significantly based upon patient size, anatomy, level of illness, and type of device implanted. In an effort to better understand current practice patterns and identify populations for whom there does not appear to be a consensus approach to achieving optimal VAD outcomes, the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) has surveyed clinical decision-making among member sites and conducted a review of the available literature regarding patient population-specific VAD outcomes and center-specific practices related to patient and device selection. Rather than aiming to provide clinical guidelines, this document offers an overview of contemporary approaches to patient and device selection, highlighting specific populations for whom there is not a consensus approach to achieving reliably good VAD outcomes, as these populations may benefit most from future research and quality improvement efforts directed toward identifying best practice.

Cardiac disease has emerged as a leading cause of mortality in Duchenne muscular dystrophy in the current era. This survey sought to identify the diagnostic and therapeutic approach to DMD among pediatric cardiologists in Advanced Cardiac Therapies Improving Outcomes Network. Pediatric cardiology providers within ACTION (a multi-center pediatric heart failure learning network) were surveyed regarding their approaches to cardiac care in DMD. Thirty-one providers from 23 centers responded. Cardiac MRI and Holter monitoring are routinely obtained, but the frequency of use and indications for ordering these tests varied widely. Angiotensin converting enzyme inhibitor and aldosterone antagonist are generally initiated prior to onset of systolic dysfunction, while the indications for initiating beta-blocker therapy vary more widely. Seventeen (55%) providers report their center has placed an implantable cardioverter defibrillator in at least 1 DMD patient, while 11 providers (35%) would not place an ICD for primary prevention in a DMD patient. Twenty-three providers (74%) would consider placement of a ventricular assist device (VAD) as destination therapy (n = 23, 74%) and three providers (10%) would consider a VAD only as bridge to transplant. Five providers (16%) would not consider VAD at their institution. Cardiac diagnostic and therapeutic approaches vary among ACTION centers, with notable variation present regarding the use of advanced therapies (ICD and VAD). The network is currently working to harmonize medical practices and optimize clinical care in an era of rapidly evolving outcomes and cardiac/skeletal muscle therapies.

To understand the genetic contribution to primary pediatric cardiomyopathy, we performed exome sequencing in a large cohort of 528 children with cardiomyopathy. Using clinical interpretation guidelines and targeting genes implicated in cardiomyopathy, we identified a genetic cause in 32% of affected individuals. Cardiomyopathy sub-phenotypes differed by ancestry, age at diagnosis, and family history. Infants < 1 year were less likely to have a molecular diagnosis (p < 0.001). Using a discovery set of 1,703 candidate genes and informatic tools, we identified rare and damaging variants in 56% of affected individuals. We see an excess burden of damaging variants in affected individuals as compared to two independent control sets, 1000 Genomes Project (p < 0.001) and SPARK parental controls (p < 1 × 10^-16). Cardiomyopathy variant burden remained enriched when stratified by ancestry, variant type, and sub-phenotype, emphasizing the importance of understanding the contribution of these factors to genetic architecture. Enrichment in this discovery candidate gene set suggests multigenic mechanisms underlie sub-phenotype-specific causes and presentations of cardiomyopathy. These results identify important information about the genetic architecture of pediatric cardiomyopathy and support recommendations for clinical genetic testing in children while illustrating differences in genetic architecture by age, ancestry, and sub-phenotype and providing rationale for larger studies to investigate multigenic contributions.

BACKGROUND:Since the first heart transplant in 1967, there has been significant progress in this field of cardiac transplantation. Approximately 600 pediatric heart transplants are performed every year worldwide. With the increasing number of pediatric heart transplant patients, and given the few tertiary care pediatric transplant centers, adult and pediatric emergency department (ED) providers are increasingly engaged in the care of pediatric heart transplant recipients in the ED. OBJECTIVE:The aim of this article is to review common ED scenarios pertinent to the pediatric heart transplant patients. DISCUSSION:There are complications unique to this population, such as rejection, opportunistic infections, and medication side effects, that require special considerations, and it is helpful for the emergency medicine (EM) provider to have knowledge about them. CONCLUSIONS:The unique immunological challenges in these patients, including rejection and medication side effects and opportunistic infections, make this population fragile, and the knowledge of these challenges is helpful for EM providers.

Cardiovascular diseases and cancer continue to be the two leading causes of death in the United States. While innovations in artificial intelligence, digital health, and telemedicine may revolutionize cardio-oncology clinical practice, barriers to widespread adoption continue to exist. The most effective way to advance these technologies is through a broad range of stakeholders sharing a common vision. Additionally, as we enter the digital era in healthcare, we must help lead this charge for the benefit of our cardiology and oncology patients. Bolstering collaborations in cardiology and oncology is key, in partnership with technology firms, industry, academia, and private practice, with an emphasis on various forms of innovation. The ultimate goal is to connect our patients and their health to informatics-based opportunities to advance cardiovascular disease prevention in cancer patients. We have established the Cardiology Oncology Innovation Network in accordance with this vision, to develop new care delivery options through the use of innovative technological strategies. Our tripartite mission - innovation, collaboration, and education - aims to increase access to and expertise in digital transformation to prevent cardiovascular diseases in cancer patients. Here we describe network initiatives, early accomplishments, and future milestones.