Assessment of Left Ventricular Myocardial Fibrosis in Adult Patients With Ebstein Anomaly: A Retrospective Cohort Study Based on Cardiac Magnetic Resonance and Histopathological Samples.

BACKGROUND: The association between Ebstein anomaly and myocardial fibrosis, particularly in the left ventricle, has been controversial. We aimed to assess the prevalence of replacement fibrosis with a focus on the left ventricle (LV) using cardiac magnetic resonance (CMR), make a histopathological association between LV fibrosis and CMR findings, and explore whether LV fibrosis is an independent risk factor for cardiovascular disease mortality using a derived risk score. METHODS: We performed a 12-year (2009-2021) retrospective cohort of adult patients with Ebstein anomaly who underwent CMR. The CMR evaluation included a comprehensive assessment of myocardial fibrosis by late gadolinium enhancement (LGE). Four postmortem samples were obtained from our cohort and stained using Masson trichrome to characterize LV fibrosis. We used Cox-regression analysis to identify and derive a prediction score that associated LV fibrosis with cardiovascular disease mortality. RESULTS: We included 57 adults with Ebstein anomaly (52% men; median age, 29.52 [interquartile range, 21.24-39.17] years), of whom 12 died during follow-up. LGE prevalence by CMR was observed in 52.6% in any chamber; LV-LGE in 29.8%. Histopathological findings revealed a mid-wall pattern with predominantly interstitial fibrosis and minimal replacement fibrosis. LV-LGE was associated with increased risk of cardiovascular disease mortality (hazard ratio, 6.02 [95% CI, 1.22-19.91]) attributable to lateral and mid-wall LV segment involvement. Our mortality score achieved an overall good prediction capacity (R2, 0.435; C statistic, 0.93; Dxy, 0.86). CONCLUSIONS: There is a high prevalence of LV fibrosis replacement in adults with Ebstein anomaly, characterized by specific CMR and histological patterns. Furthermore, LV-LGE fibrosis is an independent predictor of cardiovascular disease mortality, which could be integrated into risk assessment in clinical management.

Cardiac computed tomography assessment of congenital aortic diseases: a case series.

Background: Congenital aortic diseases (CAoD) encompass a wide variety of disorders that range from asymptomatic findings to life-threatening conditions. Multiple imaging techniques are available for the assessment of CAoD. Case summary: We present seven case reports of congenital aortic diseases, including obstructions in the aortic arch (coarctation, hypoplasia, and interruption) and vascular rings, in which the clinical manifestations throughout the cases are discussed, highlighting the heterogeneity of the symptoms. Discussion: Multi-imaging techniques are indispensable for the assessment of CAoD, where cardiac computed tomography angiography is the main modality for rapid acquisition of three-dimensional volume-rendered images for optimal surgical planning.

Case Report: Unusual Cause of Chest Pain: A Multi-Image Assessment of a Cardiac Mass.

Myxomas represent the most common benign primary cardiac tumor, they usually grow out of the interatrial septum into the left atrium with a pedunculated base. Intracardiac masses can be found incidentally on imaging studies, but symptomatology may arise secondary to the mass effect, embolization, and valvular function impairment. We present the case of a 75-year-old woman who arrived at the emergency department with atrial fibrillation and NSTEMI segment elevation myocardial infarction (NSTEMI) secondary to a highly vascularized neoplasm visible by coronary angiography and angiotomography. Scarce reports show high quality multi-imaging assessment of significantly vascularized myxomas with such atypical presentation. High-definition imaging studies played a fundamental role in the surgeon's management of a mass with a complex neovascularization.