临床评分系统在不同患者队列中诊断转甲状腺素蛋白淀粉样心肌病的性能。
Performance of Clinical Scoring Systems in the Diagnosis of Transthyretin Amyloid Cardiomyopathy in a Diverse Patient Cohort.
作者信息
Greenberg Garred S, Onuegbu Chinwendu, Espiche Carlos, Scotti Andrea, Ippolito Paul, Dwaah Henry, Gilman Jake, Tauras James, Schenone Aldo L, Slomka Piotr J, Kittleson Michelle M, Di Carli Marcelo F, Garcia Mario J, Travin Mark, Slipczuk Leandro
机构信息
Cardiology Division, Montefiore-Einstein Center for Heart and Vascular Care, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York.
Departments of Medicine and Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, California; Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center. Los Angeles, California.
出版信息
J Card Fail. 2025 Aug;31(8):1139-1148. doi: 10.1016/j.cardfail.2024.11.016. Epub 2024 Dec 17.
BACKGROUND
Two diagnostic clinical scoring systems, the ATTR-CM Score and the T-AMYLO Score, have been proposed but not validated in diverse populations despite Black race being an important risk factor for transthyretin amyloid cardiomyopathy (ATTR-CM). The aim of this study was to evaluate their performance in diagnosing ATTR-CM in a diverse patient cohort.
METHODS
This retrospective single-center study analyzed patients who underwent a 99mTc-pyrophosphate single-photon emission computed tomography scan (Tc-PYP) for workup of suspected ATTR-CM. ATTR-CM was considered present in those exhibiting Perugini scores of 2 or 3, confirmed by myocardial radiotracer uptake via single-photon emission computed tomography. The diagnostic performance of a multivariate regression model and the two scoring systems was tested against Tc-PYP as the gold standard.
RESULTS
Our cohort included 476 patients, of which 308 (65%) were non-Hispanic Black, 93 (20%) were Hispanic, and 215 (45%) were female. A total of 164 (34%) had a positive Tc-PYP result. Age ≥74 years, male sex, history of carpal tunnel, left ventricular ejection fraction <55%, posterior wall thickness over 12 mm, and relative wall thickness over 0.57 were independent predictors of positive Tc-PYP results in our cohort, and hemoglobin level <10 mg/dL, glomerular filtration rate <30 mL/min/1.73 m, and coronary artery disease were independent predictors of negative Tc-PYP. The multivariate model had an area under the curve (AUC) of 0.92 (95% CI 0.90-0.95). The ATTR-CM Score (AUC, 0.86; 95% CI 0.83-0.90) had better diagnostic accuracy than the T-AMYLO Score (AUC, 0.75; 95% CI 0.71-0.80; P < .001).
CONCLUSIONS
Two simple clinical scoring systems, derived to identify patients at high risk of having ATTR-CM necessitating further diagnostic evaluation, showed good predictive accuracy in our diverse patient cohort. The ATTR-CM Score was superior to the T-AMYLO Score in our cohort.
背景
已经提出了两种诊断临床评分系统，即ATTR-CM评分和T-淀粉样蛋白评分，但尽管黑人种族是转甲状腺素蛋白淀粉样心肌病（ATTR-CM）的重要危险因素，但尚未在不同人群中进行验证。本研究的目的是评估它们在不同患者队列中诊断ATTR-CM的性能。
方法
这项回顾性单中心研究分析了因疑似ATTR-CM而接受99mTc-焦磷酸盐单光子发射计算机断层扫描（Tc-PYP）的患者。如果患者的佩鲁吉尼评分为2或3，并通过单光子发射计算机断层扫描显示心肌放射性示踪剂摄取得到证实，则认为存在ATTR-CM。以Tc-PYP作为金标准，测试多变量回归模型和两种评分系统的诊断性能。
结果
我们的队列包括476名患者，其中308名（65%）为非西班牙裔黑人，93名（20%）为西班牙裔，215名（45%）为女性。共有164名（34%）患者的Tc-PYP结果为阳性。年龄≥74岁、男性、腕管综合征病史、左心室射血分数<55%、后壁厚度超过12 mm以及相对壁厚度超过0.57是我们队列中Tc-PYP结果为阳性的独立预测因素，而血红蛋白水平<10 mg/dL、肾小球滤过率<30 mL/min/1.73 m²以及冠状动脉疾病是Tc-PYP结果为阴性的独立预测因素。多变量模型的曲线下面积（AUC）为0.92（95%CI 0.90-0.95）。ATTR-CM评分（AUC，0.86；95%CI 0.83-0.90）的诊断准确性优于T-淀粉样蛋白评分（AUC，0.75；95%CI 0.71-0.80；P<.001）。
结论
两种简单的临床评分系统旨在识别有ATTR-CM高风险需要进一步诊断评估的患者，在我们的不同患者队列中显示出良好的预测准确性。在我们的队列中，ATTR-CM评分优于T-淀粉样蛋白评分。
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