Neighborhood socioeconomic status and length of stay after congenital heart disease surgery.

Background and Objectives: Socioeconomic factors are associated with health outcomes and can affect postoperative length of stay after congenital heart disease (CHD) surgery. The hypothesis of this study is that patients from neighborhoods with a disadvantaged socioeconomic status (SES) have a prolonged length of hospital stay after CHD surgery. Methods: Pre- and postoperative data were collected on patients who underwent CHD surgery at the University of Maryland Medical Center between 2011 and 2019. A neighborhood SES score was calculated for each patient using data from the United States Census Bureau and patients were grouped by high vs. low SES neighborhoods. The difference of patient length of stay (LOS) from the Society for Thoracic Surgeons median LOS for that surgery was the primary outcome measure. Linear regression was performed to examine the association between the difference from the median LOS and SES, as well as other third variables. Results: The difference from the median LOS was -4.8 vs. -2.2 days in high vs. low SES groups (p = 0.003). SES category was a significant predictor of LOS in unadjusted and adjusted regression analyses. There was a significant interaction between Norwood operation and SES-patients with a low neighborhood SES who underwent Norwood operation had a longer LOS, but there was no difference in LOS by SES in patients who underwent other operations. Conclusions: Neighborhood SES is a significant predictor of the LOS after congenital heart disease surgery. This effect was seen primarily in patients undergoing Norwood operation.

MRI Findings in Desmoplakin-related Arrhythmogenic Left Ventricular Cardiomyopathy in a Pediatric Patient: A Case Report.

Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disorder that cannot be explained by ischemic, hypertensive, or valvular heart disease and often results in sudden cardiac death. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is the best-characterized ACM and can be diagnosed using the revised task force criteria. In contrast, there are no accepted clinical diagnostic criteria for arrhythmogenic left ventricular cardiomyopathy (ALVC), another subtype of ACM. Cardiac MRI aids in ARVC diagnosis by delineating biventricular structural and functional abnormalities and can be instrumental in diagnosing ALVC. This report presents a pediatric case of desmoplakin cardiomyopathy, a distinct subtype of ALVC, with findings overlapping myocarditis and LV noncompaction. Keywords: Pediatrics, Heart, Cardiomyopathies Supplemental material is available for this article. © RSNA, 2023.

Challenges and opportunities for integrating genetic testing into a diagnostic workflow: heritable long QT syndrome as a model.

BACKGROUND: An increasing number of diagnostic evaluations incorporate genetic testing to facilitate accurate and timely diagnoses. The increasing number and complexity of genetic tests continue to pose challenges in deciding when to test, selecting the correct test(s), and using results to inform medical diagnoses, especially for medical professionals lacking genetic expertise. Careful consideration of a diagnostic workflow can be helpful in understanding the appropriate uses of genetic testing within a broader diagnostic workup. CONTENT: The diagnosis of long QT syndrome (LQTS), a life-threatening cardiac arrhythmia, provides an example for this approach. Electrocardiography is the preferred means for diagnosing LQTS but can be uninformative for some patients due to the variable presentation of the condition. Family history and genetic testing can augment physiological testing to inform a diagnosis and subsequent therapy. Clinical and laboratory professionals informed by peer- reviewed literature and professional recommendations constructed a generalized LQTS diagnostic workflow. This workflow served to explore decisions regarding the use of genetic testing for diagnosing LQTS. SUMMARY AND OUTLOOK: Understanding the complexities and approaches to integrating genetic testing into a broader diagnostic evaluation is anticipated to support appropriate test utilization, optimize diagnostic evaluation, and facilitate a multidisciplinary approach essential for achieving accurate and timely diagnoses.

Approach to syncope and altered mental status.

Children who present with an episode of altered mental status, whether transient or persistent, present a diagnostic challenge for practitioners. This article describes some of the more common causes of altered mental status and delineates a rational approach to these patients. This will help practitioners recognize the life-threatening causes of these frightening presentations as well as help avoid unnecessary testing for the more benign causes.

Unusually high association of hypertrophic cardiomyopathy and complex heart defects in children with fasciculoventricular pathways.

INTRODUCTION: Fasciculoventricular pathways (FVPs) are rare causes of preexcitation that do not mediate tachycardias. We report a two-center experience of pediatric patients with FVP and an unexpectedly high association of complex congenital heart defects (CHDs), chromosomal anomalies, and hypertrophic cardiomyopathy. METHODS: A retrospective review of the electrophysiology database at two institutions was performed to identify patients with FVP from January 2000 to January 2011. Medical records of these patients were reviewed for clinical history and course, presence of comorbidities, and details of intracardiac electrophysiology (EP) study. RESULTS: A total of 17 patients were identified. The primary indication for EP study was a preexcitation pattern on electrocardiogram. The majority of patients, 12/17 (71%), were found to have associated cardiac and genetic anomalies. Hypertrophic cardiomyopathy was found in 5/17 (29%) patients, with genetic testing in two patients demonstrating the lysosomal-associated membrane protein 2 mutation (Danon syndrome). Underlying complex CHDs were present in 3/17 (18%) patients. One patient (6%) was status post (s/p) cardiac transplant, one patient had hypertension, and another had Trisomy 21. Other electrophysiologic substrates mediating tachycardia were found in 3/17 (18%) patients. Only 5/17 patients (29%) were otherwise healthy with structurally normal hearts. CONCLUSIONS: In this largest reported series of FVP in children, there is an unusually high association of FVP with complex CHDs, chromosomal anomalies, and hypertrophic cardiomyopathy. Any patient with such disorders and manifest preexcitation should be evaluated with a high index of suspicion for a FVP.

Acute myocarditis in children: current concepts and management.

The spectrum of presentation of pediatric myocarditis ranges from minor flu-like illness with chest pain to acute cardiogenic shock in a previously healthy child. A major change in the diagnostic evaluation of myocarditis is a shift in focus away from endomyocardial biopsy and histologic confirmation toward cardiac MRI for noninvasive diagnosis and prognostication of acute myocarditis. Cardiac MRI may be particularly useful in pediatric patients, considering the risks associated with biopsy in children. Some of the MRI characteristics seen in pediatric patients with acute myocarditis also may serve as predictors of outcome. The approach to managing myocarditis varies according to the severity of presentation; it is primarily supportive while spontaneous recovery of cardiac function is awaited. For subacute heart failure, the mainstay of therapy is still supportive management with oral heart failure medications such as angiotensin-converting enzyme inhibitors, beta-blockers, and diuretics. Acute myocarditis presenting with severe symptomatology, termed fulminant myocarditis, has a high recovery rate. Aggressive supportive care, including mechanical circulatory support, is indicated in fulminant myocarditis. With the increasing availability of and experience with paracorporeal pulsatile mechanical assist devices for children, more and more young patients are being bridged to transplantation or recovery. Despite promising results from several uncontrolled pediatric studies using immunosuppressive and/or immunomodulating therapy with intravenous gamma-globulin, the translation of these results into a recommended, routine therapy for pediatric myocarditis has been complicated by the high rate of spontaneous improvement of myocarditis with supportive care and the lack of demonstrable benefit for such therapies in blinded, randomized, placebo-controlled trials in adult myocarditis. Further clinical studies are necessary to define the possible utility of immunosuppressive, immunomodulatory, and antiviral therapy. Heart transplantation remains the final therapeutic option for children with myocarditis and intractable severe heart failure.

Improvement of cardiac auscultation skills in pediatric residents with training.

Cardiac murmurs, most of which are harmless, are present in more than 50% of children. Good auscultation skills are required to prevent unnecessary referrals. The auscultation skills of a group of 21 pediatric residents were assessed. Based on their identification of key features such as S1, S2, timing, grade, location, quality of the murmur, and any extra sounds, residents were asked to make a clinical diagnosis. The overall diagnostic accuracy for all residents was 30% and improved with years of training. The average score for correctly identified key features was 46% with no significant improvement with year of training. Residents who had completed a cardiology rotation scored better than the others (41% vs 18% for correct diagnosis). Clinical auscultation skills of pediatric residents in our institution showed significant improvement with training, especially in the ability to recognize a harmless heart murmur.