Department of Nuclear Cardiology (V.F.-B., J.S.-R., A.C.-G., A.E.-G., E.S.-P., E.A.-R., N.E.-Z.), National Institute of Cardiology Ignacio Chavez, Mexico City.
Department of Endocrinology (N.E.A.-V.), National Institute of Cardiology Ignacio Chavez, Mexico City.
Circ Cardiovasc Imaging. 2023 May;16(5):e015011. doi: 10.1161/CIRCIMAGING.122.015011. Epub 2023 May 8.
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.
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.
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 (R, 0.435; C statistic, 0.93; D, 0.86).
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.
Ebstein 畸形与心肌纤维化之间的关联，特别是在左心室，一直存在争议。我们旨在使用心脏磁共振（CMR）评估左心室（LV）的替代纤维化的患病率，重点关注左心室纤维化与 CMR 结果之间的组织病理学关联，并探讨 LV 纤维化是否是心血管疾病死亡率的独立危险因素，使用衍生风险评分。
我们进行了一项为期 12 年（2009-2021 年）的回顾性成年 Ebstein 畸形患者队列研究，这些患者接受了 CMR。CMR 评估包括使用晚期钆增强（LGE）对心肌纤维化进行全面评估。从我们的队列中获得了 4 个尸检样本，并使用 Masson 三色染色对 LV 纤维化进行染色。我们使用 Cox 回归分析来识别和推导一个预测评分，该评分将 LV 纤维化与心血管疾病死亡率相关联。
我们纳入了 57 名 Ebstein 畸形成人患者（52%为男性；中位年龄 29.52 [四分位距 21.24-39.17] 岁），其中 12 人在随访期间死亡。CMR 观察到任何腔室的 LGE 患病率为 52.6%；LV-LGE 为 29.8%。组织病理学发现呈现出中壁模式，主要为间质纤维化和最小的替代纤维化。LV-LGE 与心血管疾病死亡率增加相关（危险比 6.02 [95%CI 1.22-19.91]），归因于侧壁和中壁 LV 节段受累。我们的死亡率评分达到了总体良好的预测能力（R 为 0.435；C 统计量为 0.93；D 值为 0.86）。
Ebstein 畸形成人中存在高患病率的 LV 纤维化替代，其特征为特定的 CMR 和组织学模式。此外，LV-LGE 纤维化是心血管疾病死亡率的独立预测因子，可纳入临床管理中的风险评估。
