Rhabdomyomas are relatively rare, cardiac tumors that are predominantly seen in infancy. Involvement of the mitral valve is considered exceptionally uncommon. We retrospectively reviewed three cases of mitral valve rhabdomyomas in patients with tuberous sclerosis complex (TSC) at a single institution, focusing on clinical presentation, imaging, and management. All patients presented with low cardiac output and mitral inflow obstruction, resulting in compromised circulatory dynamics. Echocardiography revealed mobile, hyperechoic masses on the mitral leaflets. Despite moments of hemodynamic concerns and fluctuating clinical status, all patients were managed medically with antiarrhythmics, supportive care, and one patient received sirolimus. Tumor regression and symptom improvement were observed with time, indicating a positive long-term prognosis. Neonatal mitral valve rhabdomyomas, though rare, have improved with medical therapy in these cases and surgery was avoided. In select cases, mTOR inhibitors, medical management, and echocardiographic surveillance may offer a safe alternative to surgery.

Prior authorization is a process that health insurance companies use to determine if a patient's health insurance will cover certain medical treatments, procedures, or medications. Prior authorization requests are common in adult congenital and pediatric cardiology (ACPC) due to need for advanced diagnostics, complex procedures, disease-specific medications, and the heterogeneity of the ACPC population. Prior authorizations in ACPC are rarely denied, but nonetheless, they are often accompanied by significant administrative burden on clinical care teams and delays in patient care. Prior authorizations have been implicated in worsening care inequities. The prior authorization process is insurer specific with differences between commercial and public insurers. Prior authorization rejections were previously found to be more common for women, racial minorities, those with low education, and in low-income groups. Prior authorization unduly burdens routine diagnostics, routine interventional and surgical procedures, and routine cardiac specific medication use in the ACPC population. This manuscript highlights the burdens of prior authorization and advocates for the elimination of prior authorization for ACPC patients.

Evidence from medicine and other fields has shown that gender diversity results in better decision making and outcomes. The incoming workforce of congenital heart specialists (especially in pediatric cardiology) appears to be more gender balanced, but past studies have shown many inequities. Gender‐associated differences in leadership positions, opportunities presented for academic advancement, and recognition for academic contributions to the field persist. In addition, compensation packages remain disparate if evaluated based on gender with equivalent experience and expertise. This review explores these inequities and has suggested individual and institutional changes that could be made to recruit and retain women, monitor the climate of the institution, and identify and eliminate bias in areas like salary and promotions.

Introduction: Congenital heart diseases (CHDs) are the most common congenital anomalies, and almost one-third of patients require an intervention in the neonatal period or first year of life. Unlike low- and middle-income countries (LMICs), the survival of patients with CHD in high income countries is over 90%. The aim of this study was to compare the different congenital heart surgery (CHS) outcomes databases and analyze geographical variations. Methods: We analyzed the STS-CHSD (North America), the ECHSA, the ECHSA (Only Europe), and the IQIC for CHD (LMICs) databases between 2016 and 2019. All databases include aggregated data consisting of the 10-benchmark CHS and CHS grouped by mortality risk categories. For each database and condition, we extracted the number of participants, number of operations, postoperative length of stay (LOS), and mortality, comparing regional findings. Results: Compared to ECHSA, STS-CHSD database had more operations (32066 vs 14327) despite lower participant numbers. 50% of surgeries in LMIC were VSD closure, while the STS database reported the largest number of complex interventions. Mortality was lowest in North America for all the operations. LMICs had the shortest LOS, while North America had the longest LOS for almost all the benchmark operations. (Figure) However, the relevance of LOS is limited due to lack of data on demographic findings like age at surgery and gestation. Conclusions: The analysis of these three databases, accounting for limitations of aggregate data, highlight the differences in the CHS outcomes among world regions, reflecting the geographical difference of the population. Furthermore, it emphasizes the critical need to develop collaborative databases to improve global outcomes.

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.

Abstract Background Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with Coronavirus Disease 2019 present similarly with mucocutaneous symptoms and fever. Both syndromes can progress to shock. Successful treatments for MIS-C are largely based on proven KD management. As more patients with MIS-C are treated, protocols are adjusted. Infectious Diseases (ID) specialists are often early consultants in these cases. Understanding differences in how body systems are affected in MIS-C versus KD is essential for management. Figure 1. Cardiac changes among patients with Kawasaki Disease shock syndrome (KDSS) and Muti-system Inflammatory Syndrome (MIS-C) Methods This is a single hospital comparison of 25 cases of MIS-C with mucocutaneous presentation and symptoms of shock and 25 consecutive cases of KD Shock Syndrome (KDSS). Cases were compared for demographics, symptoms, cardiac abnormalities, medical treatments, and cardiac recovery. Results Patients with MIS-C develop symptoms of shock including sustained hypotension and tachycardia at 3 times the rate of patients with KD (45% vs 13%; p< 0.001). On echocardiogram, left ventricular myocardial dysfunction, assessed by ejection fraction, is more commonly noted in cases of MIS-C than KDSS (fig 1). About half of patients with MIS-C show left ventricular myocardial dysfunction initially with normalization by 6 months post-presentation in the majority (96%). Conclusion Cardiac changes and shock events related to KD and MIS-C are thought to be caused by differing inflammatory mediators. By comparing these two syndromes, we can determine ways to manage each optimally. MIS-C often results in left ventricular myocardial dysfunction, which is rarer in KD cases. Fluid resuscitation with multiple fluid boluses followed by inotropes to treat hypotension in cases of in MIS-C puts increased strain on the already weakened myocardium. Early intravenous immunoglobulin (IVIG) administration, even in the presence of mild hypotension, can simultaneously provide the patient with additional fluid and decrease the underlying inflammatory process. This prompt treatment might reduce the need for pressor support while protecting the myocardium from further damage. As early consultants in MIS-C, ID providers should be educated regarding the unique cardiac challenges of MIS-C and avoid delay in IVIG treatment and cardiologist and intensivist consultation. Disclosures All Authors: No reported disclosures

The congenital heart care community faces a myriad of public health issues that act as barriers toward optimum patient outcomes. In this article, we attempt to define advocacy and policy initiatives meant to spotlight and potentially address these challenges. Issues are organized into the following 3 key facets of our community: patient population, health care delivery, and workforce. We discuss the social determinants of health and health care disparities that affect patients in the community that require the attention of policy makers. Furthermore, we highlight the many needs of the growing adults with congenital heart disease and those with comorbidities, highlighting concerns regarding the inequities in access to cardiac care and the need for multidisciplinary care. We also recognize the problems of transparency in outcomes reporting and the promising application of telehealth. Finally, we highlight the training of providers, measures of productivity, diversity in the workforce, and the importance of patient-family centered organizations in advocating for patients. Although all of these issues remain relevant to many subspecialties in medicine, this article attempts to illustrate the unique needs of this population and highlight ways in which to work together to address important opportunities for change in the cardiac care community and beyond. This article provides a framework for policy and advocacy efforts for the next decade.

PURPOSE: The Mednax Cardiology Quality Collaborative (MCQC) joined the ACC Adult Congenital and Pediatric Cardiology (ACPC) Quality Network (QNet) in the fall of 2016. MCQC leadership began projects with established goals by 2018 of: (1) 50% voluntary practice enrollment in ACPC QNet, and (2) 5 metrics reported per practice on the ACPC QNet quarterly reports. METHODS: Mednax Cardiology practices were asked to designate a physician as the practice MCQC lead. Two face to face meetings are held annually to collaborate on quality improvement. Monthly MCQC conference calls follow up …

Purpose: Our aim was to expand Quality Metric (QM) documentation and data collection while creating a platform to facilitate reporting for the Mednax Cardiology Quality Collaborative (MCQC). Goals included optimizing our existing EMR tools to capture denominator and numerator elements while leveraging a workflow conducive to enhance outcomes. Long-term goals included: (1) 100% documentation within the EMR platform, (2) 100% semi-automated reporting of QM';s (3) QM compliance within parameters set by the American College of Cardiology (ACC) Adult Congenital and Pediatric Cardiology (ACPC) section Quality Network (ACPC QNet) collaborative. …

Objectives. This report describes the results of the Food and Drug Administration's phase 1 and 2 clinical trials of intravascular stents at Texas Children's Hospital,Background. Since the late 1980s, intravascular stent implantation for the treatment of arterial and venous stenoses in congenital heart disease has been highly successful.Methods. Stents were placed in postoperative pulmonary artery (PA) stenoses, congenital PA stenoses or stenoses of systemic veins/venous anastomoses, Prospective collection of data according to protocol was done before intervention, after stent implantation and at follow-up catheterization.Results. At stent implantation, pressure gradients decreased significantly in all three groups (mean +/- SD): from 46 +/- 25 to 10 +/- 13 mm Hg in postoperative PA stenoses (p < 0.001); from 71 +/- 45 to 15 +/- 21 mm Hg in congenital PA stenoses (p < 0.001); and from 7 +/- 6 to 1 +/- 2 mm Hg in stenoses of systemic veins/venous anastomoses stenoses (p < 0.001). Vessel diameters markedly increased: from 6 +/- 3 to 12 +/- 3 mm in postoperative PA stenoses (p < 0.001); from 3 +/- 1 to 9 +/- 1 mm in congenital PA stenoses (p < 0.001); and from 3 +/- 4 to 12 +/- 4 mm in stenoses of systemic veins/venous anastomoses (p < 0.001). In the postoperative and congenital PA stenoses groups, right ventricular pressure decreased (right ventricular pressure indexed to femoral artery pressure ratio): from 0.63 +/- 0.2 to 0.41 +/- 0.02 (p < 0.001) and from 0.71 +/- 0.3 to 0.55 +/- 0.35 (p = 0.04), respectively, Perfusion to a single affected lung increased from 31 +/- 17% to 46 +/- 14% (p < 0.001). On recatheterization (mean 14 months), results varied minimally, Repeat angioplasty of residual stent stenoses was safe and effective. Complications included four early patients with stent migration, three with stent thrombosis and two deaths, There were no late complications, Significant restenosis occurred in only three patients.Conclusions. Intravascular stents for the treatment of vascular stenoses in congenital heart disease provide excellent immediate and long-term results. (C) 1998 by the American College of Cardiology.

Recent studies have reported the expanding use of transplantation as the definitive option for pediatric patients with inoperable congenital heart disease. This study compares perioperative risk factors and outcomes in pediatric patients who received heart transplants for congenital heart disease with those in pediatric patients who received heart transplants for cardiomyopathy. Retrospective data collected on 40 consecutive pediatric patients undergoing cardiac transplantation from 1 January 1990 through 31 January 1995 provided the following results: 26 patients with cardiomyopathy (mean age, 7.6 years) and 14 patients with congenital heart disease (mean age, 7.2 years) underwent heart transplantation. Between groups, no significant difference was detected in waiting time for a donor heart (cardiomyopathy = 85 days, range = 2 to 409; congenital heart disease = 126 days, range = 9 to 396; P = NS); in donor/recipient weight ratio (1.27 +/- 0.34 vs 1.27 +/- 0.28, P = NS); or in ischemic times (209 +/- 92 minutes vs 248 +/- 70 minutes, P = NS). Cardiopulmonary bypass times accounted for the only significant difference (73 +/- 21 minutes vs 102 +/- 29 minutes, P = 0.003). No significant difference was found in the number of infection episodes, total days hospitalized, rejection episodes, or incidence of transplant coronary artery disease. Forty-month actuarial survival was 88% +/- 6% and 92% +/- 7% for cardiomyopathy and congenital heart disease transplant recipients, respectively (P = NS). We conclude that post-transplantation morbidity and mortality in patients with previous congenital heart disease are not significantly different from morbidity and mortality in patients with cardiomyopathy. Transplantation should be considered an acceptable therapeutic option for patients with congenital heart disease when surgical repair of the native heart is not possible.

OBJECTIVETo determine the diagnostic accuracy of clinical assessment of heart murmurs in children and specific clinical features that are predictive of cardiac disease.DESIGNConcurrent case series with pretest-posttest assessment.SETTINGPediatric cardiology outpatient clinic.PARTICIPANTSFive full-time academic pediatric cardiologists.MEASURESFor each of 222 consecutive patients who were seen for first-time evaluation of a heart murmur, the clinical findings and diagnostic impressions were recorded after clinical assessment. The results of electrocardiograms and echocardiograms were then reviewed, and changes in diagnostic impressions were recorded and compared with the original impressions.RESULTSThe prevalence of cardiac disease was 33%. Clinical assessment differentiated those patients with pathologic murmurs with a sensitivity of 92%, specificity of 94%, positive predictive value of 88%, and negative predictive value of 96%. If diagnostic uncertainty was considered an indication for echocardiography, then sensitivity and specificity increased to 97% and 98%, respectively. Missed disease included only trivial or minor lesions. Clinical features that were independently predictive of the presence of disease included murmurs that were pansystolic (odds ratio [OR], 54.0), grade 3 or more in intensity (OR, 4.84), heard best at the left upper sternal border (OR, 4.24) and harsh in quality (OR, 2.37), and the presence of an abnormal second heart sound (OR, 4.09) and an early or midsystolic click (OR, 8.35).CONCLUSIONSClinical assessment by a pediatric cardiologist is sufficient to distinguish pathologic from innocent heart murmurs. A genetic approach by using specific clinical features that are independently associated with disease may have some practical utility to noncardiologists.

This study sought to determine the extent of anxiety and misperceptions about heart murmurs for consenting parents of 182 children referred for first-time pediatric cardiology assessment (including echocardiography) of a heart murmur. From questionnaires completed before assessment, 22% of parents indicated that they were extremely concerned, and only 16% could define a heart murmur as a sound made by the heart. From 1-month follow-up questionnaires obtained from parents of children without heart disease at assessment, 10% continued to believe that their child had a heart problem. Cardiology assessment may not provide complete reassurance to all families and additional interventions may be necessary.