Division of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Austria (C.N., M.K., C.D., R. Radun, K.M., A.K., G.H., V.D., G.S., K.H., M.-P.W., R. Rosenhek, P.B., C.H., J.M., G.G.).
Division of Nuclear Medicine (R.C., M.H.), Medical University of Vienna.
Circ Cardiovasc Imaging. 2022 Jul;15(7):e014115. doi: 10.1161/CIRCIMAGING.122.014115. Epub 2022 Jul 8.
Dual pathology of severe aortic stenosis (AS) and transthyretin cardiac amyloidosis (ATTR) is increasingly recognized. Evolution of symptoms, biomarkers, and myocardial mechanics in AS-ATTR following valve replacement is unknown. We aimed to characterize reverse remodeling in AS-ATTR and compared with lone AS.
Consecutive patients referred for transcatheter aortic valve replacement (TAVR) underwent ATTR screening by blinded 99mTc-DPD bone scintigraphy (Perugini Grade-0 negative, 1-3 increasingly positive) before intervention. ATTR was diagnosed by DPD and absence of monoclonal protein. Reverse remodeling was assessed by comprehensive evaluation before TAVR and at 1 year.
One hundred twenty patients (81.8±6.3 years, 51.7% male, 95 lone AS, 25 AS-ATTR) with complete follow-up were studied. At 12 months (interquartile range, 7-17) after TAVR, both groups experienced significant symptomatic improvement by New York Heart Association functional class (both <0.001). Yet, AS-ATTR remained more symptomatic (New York Heart Association ≥III: 36.0% versus 13.8; =0.01) with higher residual NT-proBNP (N-terminal pro-brain natriuretic peptide) levels (<0.001). Remodeling by echocardiography showed left ventricular mass regression only for lone AS (=0.002) but not AS-ATTR (=0.5). Global longitudinal strains improved similarly in both groups. Conversely, improvement of regional longitudinal strain showed a base-to-apex gradient in AS-ATTR, whereas all but apical segments improved in lone AS. This led to the development of an apical sparing pattern in AS-ATTR only after TAVR.
Patterns of reverse remodeling differ from lone AS to AS-ATTR, with both groups experiencing symptomatic improvement by TAVR. After AS treatment, AS-ATTR transfers into a lone ATTR cardiomyopathy phenotype.
严重主动脉瓣狭窄（AS）和转甲状腺素蛋白心脏淀粉样变性（ATTR）的双重病变越来越受到关注。在接受瓣膜置换术后，AS-ATTR 的症状、生物标志物和心肌力学的演变尚不清楚。我们旨在对 AS-ATTR 的逆向重构进行特征描述，并与单纯 AS 进行比较。
连续接受经导管主动脉瓣置换术（TAVR）的患者在介入前通过盲法 99mTc-DPD 骨闪烁显像进行 ATTR 筛查（佩鲁基尼分级-0 为阴性，1-3 级逐渐阳性）。通过 DPD 和无单克隆蛋白诊断 ATTR。在 TAVR 前和 1 年后通过综合评估来评估逆向重构。
共研究了 120 例患者（81.8±6.3 岁，51.7%为男性，95 例为单纯 AS，25 例为 AS-ATTR），且均完成了随访。在 TAVR 后 12 个月（四分位间距，7-17），两组患者的纽约心功能分级（均<0.001）均有明显改善。然而，AS-ATTR 的症状仍更为严重（纽约心功能分级≥III：36.0%比 13.8%；=0.01），且残余 NT-proBNP（N 端脑钠肽前体）水平更高（<0.001）。超声心动图显示，只有单纯 AS 出现左心室质量的回归（=0.002），而 AS-ATTR 没有（=0.5）。两组的整体纵向应变均得到改善。相反，在 AS-ATTR 中，区域性纵向应变的改善呈基底到心尖的梯度，而在单纯 AS 中，除心尖段外，所有节段均得到改善。这导致仅在 TAVR 后，AS-ATTR 出现心尖部保留模式。
与单纯 AS 相比，AS-ATTR 的逆向重构模式不同，两组患者在接受 TAVR 后症状均有改善。在 AS 治疗后，AS-ATTR 转化为单纯的 ATTR 心肌病表型。
