Abstract：
BACKGROUND: Aortic stenosis (AS) and transthyretin (ATTR) cardiac amyloidosis (CA) share the same clinical profiles and cardiac phenotype. Amyloid deposits have been frequently reported in aortic valves of patients with severe AS referred for surgical aortic valve replacement (SAVR). The aim of this study was to determine the clinical and myocardial status of patients with aortic valve amyloidosis after aortic valve surgery.
RESULTS: We performed a retrospective descriptive study of 46 patients who underwent SAVR for severe AS with amyloid deposits upon histological analysis. All patients were screened for cardiac involvement. Amyloid deposits typing was successful in 35 (76%) patients and 28 (80%) were ATTR. Two (4%) had positive bone scintigraphy and among the 5 myocardial biopsies performed during surgery, 80% were positive for ATTR deposits.
CONCLUSIONS: ATTR is the predominant type in the presence of amyloid deposits on the aortic valve after surgery for severe AS but is only rarely accompanied by cardiac uptake on bone scintigraphy. Early stages of myocardial involvement are frequent and myocardial biopsy is more sensitive for detection of mild amyloid deposits than bone scintigraphy.
RESULTS: We performed a retrospective descriptive study of 46 patients who underwent SAVR for severe AS with amyloid deposits upon histological analysis. All patients were screened for cardiac involvement. Amyloid deposits typing was successful in 35 (76%) patients and 28 (80%) were ATTR. Two (4%) had positive bone scintigraphy and among the 5 myocardial biopsies performed during surgery, 80% were positive for ATTR deposits.
CONCLUSIONS: ATTR is the predominant type in the presence of amyloid deposits on the aortic valve after surgery for severe AS but is only rarely accompanied by cardiac uptake on bone scintigraphy. Early stages of myocardial involvement are frequent and myocardial biopsy is more sensitive for detection of mild amyloid deposits than bone scintigraphy.
摘要：
背景：主动脉瓣狭窄（AS）和甲状腺素运载蛋白（ATTR）心脏淀粉样变性（CA）具有相同的临床特征和心脏表型。经常报道淀粉样蛋白沉积在严重AS患者的主动脉瓣中，这些患者被称为外科主动脉瓣置换术（SAVR）。这项研究的目的是确定主动脉瓣手术后主动脉瓣淀粉样变性患者的临床和心肌状况。
结果：我们对46例接受SAVR治疗的严重AS伴淀粉样蛋白沉积的患者进行了回顾性描述性研究。所有患者均接受心脏受累筛查。35例（76％）患者成功进行淀粉样沉积物分型，28例（80％）为ATTR。在手术期间进行的5例心肌活检中，有2例（4％）骨闪烁显像阳性，80%对ATTR沉积物呈阳性。
结论：ATTR是严重AS手术后主动脉瓣上存在淀粉样沉积物的主要类型，但在骨闪烁显像上很少伴有心脏摄取。心肌受累的早期阶段很常见，心肌活检比骨闪烁显像对检测轻度淀粉样蛋白沉积物更敏感。骨闪烁显像未诊断。
结果：我们对46例接受SAVR治疗的严重AS伴淀粉样蛋白沉积的患者进行了回顾性描述性研究。所有患者均接受心脏受累筛查。35例（76％）患者成功进行淀粉样沉积物分型，28例（80％）为ATTR。在手术期间进行的5例心肌活检中，有2例（4％）骨闪烁显像阳性，80%对ATTR沉积物呈阳性。
结论：ATTR是严重AS手术后主动脉瓣上存在淀粉样沉积物的主要类型，但在骨闪烁显像上很少伴有心脏摄取。心肌受累的早期阶段很常见，心肌活检比骨闪烁显像对检测轻度淀粉样蛋白沉积物更敏感。骨闪烁显像未诊断。
