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Abstract：
BACKGROUND: Prevalence of both degenerative severe aortic stenosis (AS) and transthyretin cardiac amyloidosis (ATTR-CA) increases with age. Dual disease (AS+myocardial ATTR-CA) occurs in significant proportion of patients undergoing surgical aortic valve replacement (SAVR).
OBJECTIVE: This study aimed to determine the prevalence of ATTR-CA in severe AS in the Indian population, identify noninvasive predictors of its diagnosis, and understand its impact on prognosis.
METHODS: Symptomatic severe AS patients aged ≥65 years undergoing SAVR were enrolled. ATTR-CA diagnosis was based on preoperative 99m-technetium pyrophosphate (PYP) scan and intraoperatively obtained basal interventricular septum biopsy for myocardial ATTR-CA, and excised native aortic valve for isolated valvular ATTR-CA. Primary amyloidosis was excluded by serum/urine protein electrophoresis with serum immunofixation.
RESULTS: SAVR was performed in 46 AS patients (age 70 ± 5 years, 70% men). PYP scan was performed for 32 patients, with significant PYP uptake in 3 (n = 3 of 32, 9.4%), suggestive of myocardial ATTR-CA. On histopathological examination, none of the interventricular septum biopsy specimens had amyloid deposits, whereas 33 (71.7%) native aortic valves showed amyloid deposits, of which 19 (57.6%) had transthyretin deposition suggestive of isolated valvular amyloidosis. Noninvasive markers of dual disease included low myocardial contraction fraction (median [interquartile range], 28.8% [23.8% to 39.1%] vs 15.3% [9.3% to 16.1%]; P = 0.006), deceleration time (215 [144 to 236] ms vs 88 [60 to 106] ms; P = 0.009) and global longitudinal strain (-18.7% [-21.1% to -16.9%] vs -14.2% [-17.0% to -9.7%]; P = 0.030). At 1-year follow-up, 2 patients died (4.3%); 1 each in myocardial ATTR-CA negative and positive groups (3.4% vs 33.3%; P = 0.477).
CONCLUSIONS: Dual disease is not uncommon in India. Isolated valvular amyloidosis in severe AS is much more common.
OBJECTIVE: This study aimed to determine the prevalence of ATTR-CA in severe AS in the Indian population, identify noninvasive predictors of its diagnosis, and understand its impact on prognosis.
METHODS: Symptomatic severe AS patients aged ≥65 years undergoing SAVR were enrolled. ATTR-CA diagnosis was based on preoperative 99m-technetium pyrophosphate (PYP) scan and intraoperatively obtained basal interventricular septum biopsy for myocardial ATTR-CA, and excised native aortic valve for isolated valvular ATTR-CA. Primary amyloidosis was excluded by serum/urine protein electrophoresis with serum immunofixation.
RESULTS: SAVR was performed in 46 AS patients (age 70 ± 5 years, 70% men). PYP scan was performed for 32 patients, with significant PYP uptake in 3 (n = 3 of 32, 9.4%), suggestive of myocardial ATTR-CA. On histopathological examination, none of the interventricular septum biopsy specimens had amyloid deposits, whereas 33 (71.7%) native aortic valves showed amyloid deposits, of which 19 (57.6%) had transthyretin deposition suggestive of isolated valvular amyloidosis. Noninvasive markers of dual disease included low myocardial contraction fraction (median [interquartile range], 28.8% [23.8% to 39.1%] vs 15.3% [9.3% to 16.1%]; P = 0.006), deceleration time (215 [144 to 236] ms vs 88 [60 to 106] ms; P = 0.009) and global longitudinal strain (-18.7% [-21.1% to -16.9%] vs -14.2% [-17.0% to -9.7%]; P = 0.030). At 1-year follow-up, 2 patients died (4.3%); 1 each in myocardial ATTR-CA negative and positive groups (3.4% vs 33.3%; P = 0.477).
CONCLUSIONS: Dual disease is not uncommon in India. Isolated valvular amyloidosis in severe AS is much more common.
摘要：
背景：退行性严重主动脉瓣狭窄（AS）和甲状腺素运载蛋白心脏淀粉样变性（ATTR-CA）的患病率随年龄增加而增加。在接受外科主动脉瓣置换术（SAVR）的患者中，有很大一部分发生双重疾病（AS心肌ATTR-CA）。
目的：本研究旨在确定印度人群中严重AS中ATTR-CA的患病率，确定其诊断的非侵入性预测因子，了解其对预后的影响。
方法：纳入≥65岁接受SAVR的有症状的重度AS患者。ATTR-CA的诊断基于术前99m焦磷酸tech（PYP）扫描和术中获得的心肌ATTR-CA的基底室间隔活检，并切除天然主动脉瓣用于分离的瓣膜ATTR-CA。通过血清免疫固定的血清/尿蛋白电泳排除了原发性淀粉样变性。
结果：在46例AS患者中进行了SAVR（年龄70±5岁，70%的男性）。对32例患者进行了PYP扫描，在3个（32个中的n=3，9.4％）中具有显着的PYP摄取，提示心肌ATTR-CA。在组织病理学检查中，室间隔活检标本均无淀粉样蛋白沉积，而33（71.7％）天然主动脉瓣显示淀粉样沉积物，其中19例（57.6％）有甲状腺素运载蛋白沉积，提示孤立的瓣膜淀粉样变性。双重疾病的非侵入性标志物包括低心肌收缩分数(中位数[四分位距]，28.8%[23.8%至39.1%]对15.3%[9.3%至16.1%]；P=0.006)，减速时间(215[144至236]msvs88[60至106]ms;P=0.009)和全球纵向应变(-18.7%[-21.1%至-16.9%]vs-14.2%[-17.0%至-9.7%];P=0.030)。在1年的随访中，死亡2例（4.3％）；心肌ATTR-CA阴性和阳性组各1例（3.4％vs33.3％；P=0.477）。
结论：双重疾病在印度并不少见。在严重的AS中，孤立的瓣膜淀粉样变性要常见得多。
目的：本研究旨在确定印度人群中严重AS中ATTR-CA的患病率，确定其诊断的非侵入性预测因子，了解其对预后的影响。
方法：纳入≥65岁接受SAVR的有症状的重度AS患者。ATTR-CA的诊断基于术前99m焦磷酸tech（PYP）扫描和术中获得的心肌ATTR-CA的基底室间隔活检，并切除天然主动脉瓣用于分离的瓣膜ATTR-CA。通过血清免疫固定的血清/尿蛋白电泳排除了原发性淀粉样变性。
结果：在46例AS患者中进行了SAVR（年龄70±5岁，70%的男性）。对32例患者进行了PYP扫描，在3个（32个中的n=3，9.4％）中具有显着的PYP摄取，提示心肌ATTR-CA。在组织病理学检查中，室间隔活检标本均无淀粉样蛋白沉积，而33（71.7％）天然主动脉瓣显示淀粉样沉积物，其中19例（57.6％）有甲状腺素运载蛋白沉积，提示孤立的瓣膜淀粉样变性。双重疾病的非侵入性标志物包括低心肌收缩分数(中位数[四分位距]，28.8%[23.8%至39.1%]对15.3%[9.3%至16.1%]；P=0.006)，减速时间(215[144至236]msvs88[60至106]ms;P=0.009)和全球纵向应变(-18.7%[-21.1%至-16.9%]vs-14.2%[-17.0%至-9.7%];P=0.030)。在1年的随访中，死亡2例（4.3％）；心肌ATTR-CA阴性和阳性组各1例（3.4％vs33.3％；P=0.477）。
结论：双重疾病在印度并不少见。在严重的AS中，孤立的瓣膜淀粉样变性要常见得多。
