Cardiac atrial pathology in Duchenne muscular dystrophy.

INTRODUCTION/AIMS: Duchenne muscular dystrophy (DMD) is characterized by fibrofatty replacement of muscle. This has been documented in the ventricular myocardium of DMD patients, but there is limited description of atrial involvement. The purpose of this study is to examine the arrhythmia and ectopy burden in patients with DMD and non-DMD dilated cardiomyopathy (DCM) and to characterize the cardiac histopathologic changes in DMD patients across the disease spectrum. METHODS: This was a retrospective analysis of age-matched patients with DMD and non-DMD DCM who received a Holter monitor and cardiac imaging within 100 days of each other between 2010 and 2020. Twenty-four-hour Holter monitors were classified based on the most recent left ventricular ejection fraction at the time of monitoring. Cardiac histopathologic specimens from whole-heart examinations at the time of autopsy from three DMD patients and one DCM patient were reviewed. RESULTS: A total of 367 patients with 1299 Holter monitor recordings were included over the study period, with 94% representing DMD patients and 6% non-DMD DCM. Patients with DMD had more atrial ectopy across the cardiac function spectrum (p < 0.05). There was no difference in ventricular ectopy. Four DMD patients developed symptomatic atrial arrhythmias. Autopsy specimens from DMD patients demonstrated fibrofatty infiltration of both atrial and ventricular myocardium. DISCUSSION: The atrial myocardium in patients with DMD is unique. Autopsy specimens reveal fibofatty replacement of the atrial myocardium, which may be a nidus for both ectopy and arrhythmias in DMD patients.

Noncontrast free-breathing ECG-gated 3D balanced steady-state free precession in congenital heart disease and aortopathy evaluation.

BACKGROUND: High-fidelity cardiac magnetic resonance (MR) imaging plays a pivotal role in the surveillance of congenital heart disease (CHD) and aortopathy. OBJECTIVE: We aimed to evaluate the quality and accuracy of free breathing, ECG-gated noncontrast three-dimensional (3D) balanced steady-state free precession (bSSFP) in cases of CHDs and aortopathies using contrast-enhanced 3D bSSFP as a reference. We also used one of our routinely used non-ECG-gated 2D-single-shot (SSh) bSSFP sequence as an adjunct to noncontrast 3D bSSFP. MATERIALS AND METHODS: Institutional review board approval was obtained to perform a systematic retrospective analysis of image quality and vascular measurements. Patients with CHD and aortopathy, who were undergoing clinically indicated contrast-enhanced 3D bSSFP, were prospectively identified to also undergo additional noncontrast 3D bSSFP and 2D SSh bSSFP imaging as part of a clinical quality improvement initiative aimed at reducing the use of contrast when feasible. Two readers, blinded to each other's evaluations, graded image quality on a 5-point Likert scale and performed vascular measurements in separate sessions for both 3D bSSFP images. They also reported the visibility of various mediastinal great vessels on 2D SSh bSSFP images. Raw agreement, the weighted kappa statistic, and intra-class correlation coefficients (ICCs) were computed to assess the consistency and agreement between the two readers. Comparative analysis of noncontrast and contrast-enhanced 3D bSSFP imaging was performed in adult and pediatric patients using a two-sided paired t-test and Bland-Altman analysis. A P-value < 0.05 was considered significant for all inference testing. RESULTS: A total of 29 patients (17 males, median age 20.3 years, interquartile range (IQR) 12.5, age range 7-39 years), including 11 pediatric patients under the age of 18 years (6 males, median age 14.5 years, IQR 4.0, age range 7-17 years), underwent retrospective analysis. The overall image quality score for contrast-enhanced 3D bSSFP was significantly higher (P < 0.0001) than that of noncontrast 3D bSSFP for both all subjects (4.4 ± 0.2, range 4.0-4.9 vs 3.7 ± 0.4, range 3.1-4.7) and only pediatric subjects (4.3 ± 0.3, range 4.0-4.9 vs 3.6 ± 0.5, range 3.1-4.4). By combining noncontrast 3D bSSFP and 2D bSSFP, reader 1 and reader 2 rated 423 and 420 vessels diagnostic, respectively, in a total of 435 vessel segments. All landmarks showed similar mean vessel diameters without significant differences between noncontrast and contrast-enhanced 3D bSSFP MR angiography (r = 0.99, bias -0.31 mm, 95% limits of agreement -2.04 mm to 1.43 mm). CONCLUSIONS: Although contrast-enhanced images had better overall image quality, an imaging protocol consisting of noncontrast 2D SSh bSSFP and 3D bSSFP whole-chest images provides diagnostically adequate image quality, and accurate vascular measurements, comparable to free-breathing contrast-enhanced 3D bSSFP in both children and adults with CHD and aortopathies.

Baja incidencia de cardiopatía clínicamente significativa en niños en edad escolar tras padecer COVID-19 / The low incidence of clinically significant heart disease in school-age children following COVID-19

No es clara la carga de morbimortalidad de la lesión cardíaca clínicamente evidente secundaria a la enfermedad por coronavirus de 2019 (COVID-19) en los niños en edad escolar. A lo largo de 12 meses, en un importante hospital pediátrico académico en la región del medio oeste de Estados Unidos, hubo 1481 casos de COVID-19 sin hospitalización en niños en edad escolar por lo demás sanos, en quienes se hicieron 195 pruebas cardíacas. Si bien aparecieron hallazgos fortuitos, no se descubrió ninguna patología cardíaca relacionada con la COVID-19. Además, ~3 % de los niños solamente tuvieron síntomas cardíacos agudos que requirieron una evaluación por el área de cardiología pediátrica. Los niños que no fueron hospitalizados por COVID-19 tienen un riesgo muy bajo de desarrollar daño cardíaco clínicamente significativo y son más propensos a presentar hallazgos fortuitos.

The burden of clinically-apparent cardiac injury secondary to coronavirus disease 2019 (COVID-19) in school-age children is unclear. Over 12 months at a large academic pediatric hospital in the Midwestern portion of the United States, there were 1481 COVID-19 positive non-hospitalized otherwise healthy schoolaged children with 195 having cardiac testing performed. While incidental findings occurred, no definitive COVID-19 related cardiac pathology was discovered. Additionally, only ~3 % of children had acute cardiac symptoms necessitating evaluation by pediatric cardiology. School-age children who were not hospitalized for COVID-19 have a very low risk of having clinically significant cardiac damage and are more likely to discover incidental findings.