Prognostic Role of Hepatorenal Function Indexes in Patients With Ebstein Anomaly,Journal of the American College of Cardiology

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Prognostic Role of Hepatorenal Function Indexes in Patients With Ebstein Anomaly
Journal of the American College of Cardiology ( IF 21.7 ) Pub Date : 2020-12-01 , DOI: 10.1016/j.jacc.2020.10.035
Alexander C Egbe ₁ , William R Miranda ₁ , Joseph Dearani ₂ , Patrick S Kamath ₃ , Heidi M Connolly ₁

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BACKGROUND Hepatorenal dysfunction is a risk factor for mortality in patients with chronic tricuspid regurgitation due to acquired heart disease. Ebstein anomaly is the most common cause of primary tricuspid regurgitation in adults with congenital heart disease, but the prevalence and prognostic implications of hepatorenal dysfunction are unknown in this population. OBJECTIVES The purpose of this study was to determine the risk factors and prognostic implications of hepatorenal dysfunction, as measured primarily by the use of model for end-stage liver disease excluding international normalized ratio (MELD-XI score), as well as looking at other associated factors. METHODS This was a retrospective study of adults with Ebstein anomaly who received care at Mayo Clinic from 2003 to 2018. RESULTS Of 692 patients, the median MELD-XI score was 10.2 (interquartile range: 9.4 to 13.3); 53 (8%) died and 3 (0.4%) underwent heart transplant. MELD-XI was an independent predictor of death/transplant (hazard ratio: 1.32; 95% confidence interval: 1.11 to 2.06; p < 0.001). In the subset of patients with serial MELD-XI scores (n = 416), temporal change in MELD-XI score (ΔMELD-XI) was also a predictor of death/transplant. In the subset of patients who underwent tricuspid valve surgery (n = 344), a post-operative improvement in MELD-XI score (ΔMELD-XI) was associated with improved long-term survival. Impaired right atrial (RA) reservoir strain and elevated estimated RA pressure were associated with worse baseline MELD-XI and ΔMELD-XI scores. CONCLUSIONS Hepatorenal dysfunction is a predictor of mortality in Ebstein anomaly, and RA dysfunction and hypertension are hemodynamic biomarkers that can identify patients at risk for deterioration in hepatorenal function and mortality. These data highlight the prognostic importance of noncardiac organ-system dysfunction, and provide complementary clinical risk stratification metrics for management of these patients.

中文翻译：

肝肾功能指标在 Ebstein 异常患者中的预后作用

背景肝肾功能不全是后天性心脏病引起的慢性三尖瓣关闭不全患者死亡的危险因素。Ebstein 异常是成人先天性心脏病原发性三尖瓣关闭不全的最常见原因，但肝肾功能障碍的患病率和预后意义在该人群中尚不清楚。目的本研究的目的是确定肝肾功能不全的危险因素和预后影响，主要通过使用不包括国际标准化比率（MELD-XI 评分）的终末期肝病模型进行测量，以及查看其他相关因素。方法这是一项对 2003 年至 2018 年在梅奥诊所接受治疗的 Ebstein 异常成人的回顾性研究。结果在 692 名患者中，MELD-XI 评分中位数为 10。2（四分位距：9.4 到 13.3）；53 人（8%）死亡，3 人（0.4%）接受心脏移植。MELD-XI 是死亡/移植的独立预测因子（风险比：1.32；95% 置信区间：1.11 至 2.06；p < 0.001）。在具有连续 MELD-XI 评分（n = 416）的患者子集中，MELD-XI 评分（ΔMELD-XI）的时间变化也是死亡/移植的预测因子。在接受三尖瓣手术的患者亚组（n = 344）中，MELD-XI 评分（ΔMELD-XI）的术后改善与长期生存率的提高相关。受损的右心房 (RA) 储层应变和估计的 RA 压力升高与更差的基线 MELD-XI 和 ΔMELD-XI 评分相关。结论肝肾功能障碍是 Ebstein 异常死亡率的预测因子，RA 功能障碍和高血压是血流动力学生物标志物，可以识别有肝肾功能恶化和死亡风险的患者。这些数据突出了非心脏器官系统功能障碍的预后重要性，并为这些患者的管理提供了补充的临床风险分层指标。

更新日期：2020-12-01

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