遗传性出血性毛细血管扩张症伴肝动静脉畸形患者心力衰竭症状的预测因素。

Predictors of heart failure symptoms in hereditary hemorrhagic telangiectasia patients with hepatic arteriovenous malformations.

机构信息

Division of Interventional Radiology, Department of Radiology, David Geffen School of Medicine at UCLA, 757 Westwood Plaza, 2nd Floor, Room 2125, Los Angeles, CA, 90095, USA.

Department of Biostatistics, Fielding School of Public Health, University of California, Los Angeles, CA, USA.

出版信息

Orphanet J Rare Dis. 2021 Nov 18;16(1):478. doi: 10.1186/s13023-021-02109-4.

DOI:10.1186/s13023-021-02109-4

PMID:34794458

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8600745/

Abstract

BACKGROUND

Hepatic arteriovenous malformations (AVMs) in hereditary hemorrhagic telangiectasia (HHT) patients are most commonly hepatic artery to hepatic venous shunts which can result in high-output heart failure. This condition can be debilitating and is a leading cause of liver transplantation in HHT patients. However, it is not known what characteristics can discriminate between asymptomatic patients and those who will develop heart failure symptoms.

RESULTS

176 patients with HHT were evaluated with computed tomography angiography (CTA) between April 2004 and February 2019 at our HHT Center of Excellence. 63/176 (35.8%) patients were found to have hepatic AVMs on CTA. 18 of these patients were excluded because of the presence of another condition which could confound evaluation of heart failure symptoms. In the remaining 45 patients included in our cohort, 25/45 (55.6%) patients were classified as asymptomatic and 20/45 (44.4%) were classified as symptomatic, and these groups were compared. In symptomatic patients, mean common hepatic artery (CHA) diameter was significantly higher (11.1 versus 8.4 mm) and mean hemoglobin levels were significantly lower (10.7 vs 12.6 g/dL). A stepwise multiple logistic regression analysis demonstrated that both CHA diameter and hemoglobin level were independent predictors of heart failure symptoms with ORs of 2.554 (95% CI 1.372-4.754) and 0.489 (95% CI 0.299-0.799), respectively. The receiver operator characteristic (ROC) curve of our analysis demonstrated an AUC of 0.906 (95% CI 0.816-0.996), sensitivity 80.0% (95% CI 55.7-93.4%), and specificity 75.0% (95% CI 52.9-89.4%).

CONCLUSIONS

CTA is an effective and easily reproducible method to evaluate hepatic involvement of HHT. Utilizing CTA, clinical, and laboratory data we determined CHA diameter and hemoglobin level were independent predictors of heart failure symptoms.

摘要

背景

遗传性出血性毛细血管扩张症（HHT）患者的肝动静脉畸形（AVM）最常见的是肝动脉至肝静脉分流，可导致高输出心力衰竭。这种情况可能使人虚弱，是 HHT 患者肝移植的主要原因。然而，目前尚不清楚有哪些特征可以区分无症状患者和出现心力衰竭症状的患者。

结果

我们的 HHT 卓越中心在 2004 年 4 月至 2019 年 2 月期间对 176 例 HHT 患者进行了计算机断层血管造影（CTA）评估。176 例患者中有 63 例（35.8%）在 CTA 上发现肝 AVM。其中 18 例因存在另一种可能影响心力衰竭症状评估的疾病而被排除在外。在我们的队列中，剩余的 45 例患者中，25/45（55.6%）例患者被归类为无症状，20/45（44.4%）例患者被归类为有症状，对这两组进行了比较。在有症状的患者中，平均肝总动脉（CHA）直径明显较高（11.1 毫米比 8.4 毫米），平均血红蛋白水平明显较低（10.7 克/分升比 12.6 克/分升）。逐步多变量逻辑回归分析表明，CHA 直径和血红蛋白水平均是心力衰竭症状的独立预测因子，比值比（OR）分别为 2.554（95%置信区间 1.372-4.754）和 0.489（95%置信区间 0.299-0.799）。分析的受试者工作特征（ROC）曲线显示曲线下面积（AUC）为 0.906（95%置信区间 0.816-0.996），灵敏度为 80.0%（95%置信区间 55.7-93.4%），特异性为 75.0%（95%置信区间 52.9-89.4%）。