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转甲状腺素蛋白淀粉样变性病男女患者的左心室壁厚度与心脏疾病严重程度

Left ventricular wall thickness and severity of cardiac disease in women and men with transthyretin amyloidosis.

作者信息

Aimo Alberto, Tomasoni Daniela, Porcari Aldostefano, Vergaro Giuseppe, Castiglione Vincenzo, Passino Claudio, Adamo Marianna, Bellicini Maria Giulia, Lombardi Carlo Mario, Nardi Matilde, Palamara Gloria, Varrà Guerino Giuseppe, Saro Riccardo, Allegro Valentina, Merlo Marco, Sinagra Gianfranco, Metra Marco, Emdin Michele, Rapezzi Claudio

机构信息

Interdisciplinary Center for Health Sciences, Scuola Superiore Sant'Anna, Pisa, Italy.

Cardiology Division, Fondazione Toscana Gabriele Monasterio, Pisa, Italy.

出版信息

Eur J Heart Fail. 2023 Apr;25(4):510-514. doi: 10.1002/ejhf.2824. Epub 2023 Mar 15.

Abstract

AIMS

Cardiac amyloidosis (CA) is due to a deposition of amyloid fibrils in the heart causing an increase in wall thickness. A left ventricular (LV) wall thickness ≥12 mm plus at least one red flag should raise the suspicion of CA. As normal values of LV wall thickness are lower in women, the adoption or the same cut-off values for men and women could lead to underdiagnosis or delayed diagnosis in women. We investigated the relationship between LV wall thickness and the severity of cardiac involvement in women and men with transthyretin (ATTR) CA.

METHODS AND RESULTS

We evaluated 330 consecutive patients diagnosed with ATTR-CA at three centres (Pisa, n = 232; Brescia, n = 69; Trieste, n = 29). Interventricular septum (IVS) and posterior wall (PW) thickness values were lower in women (n = 53, 16%) than men, but most differences were abolished when indexing by body surface area (BSA), height, or height , suggesting similar disease severity when accounting for the smaller body size of women. PW thickness indexed for height was even higher in women. We also searched for correlations between IVS and PW thickness and other indicators of the severity of cardiac disease. IVS values indexed by height displayed tighter associations with N-terminal pro-B-type natriuretic peptide values than non-indexed IVS values. Similarly, indexed values displayed closer relationships with relative wall thickness, E/e' ratio, and tricuspid annular plane systolic excursion.

CONCLUSIONS

Indexed LV wall thickness values, particularly by height , reflect more accurately the severity of cardiac involvement than non-indexed values.

摘要

目的

心脏淀粉样变性(CA)是由于淀粉样纤维在心脏沉积导致心肌厚度增加。左心室(LV)壁厚度≥12mm加上至少一个警示信号应引起对CA的怀疑。由于女性LV壁厚度的正常值较低,采用相同的男女临界值可能导致女性诊断不足或诊断延迟。我们研究了转甲状腺素蛋白(ATTR)CA患者中男女LV壁厚度与心脏受累严重程度之间的关系。

方法与结果

我们评估了在三个中心连续诊断为ATTR-CA的330例患者(比萨,n = 232;布雷西亚,n = 69;的里雅斯特,n = 29)。女性(n = 53,16%)的室间隔(IVS)和后壁(PW)厚度值低于男性,但按体表面积(BSA)、身高或身高指数化后,大多数差异消失,这表明考虑到女性较小的体型,疾病严重程度相似。女性按身高指数化的PW厚度甚至更高。我们还寻找了IVS和PW厚度与其他心脏病严重程度指标之间的相关性。按身高指数化的IVS值与N末端B型利钠肽前体值的关联比未指数化的IVS值更紧密。同样,指数化值与相对壁厚度、E/e'比值和三尖瓣环平面收缩期位移显示出更密切的关系。

结论

指数化的LV壁厚度值,尤其是按身高指数化的值,比未指数化的值更准确地反映心脏受累的严重程度。

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