The Heart Center, University Hospital of Copenhagen, Rigshospitalet, Copenhagen, Denmark.
The Heart Center, University Hospital of Copenhagen, Rigshospitalet, Copenhagen, Denmark.
J Am Coll Cardiol. 2022 Sep 6;80(10):967-977. doi: 10.1016/j.jacc.2022.06.026.
Bilateral carpal tunnel syndrome (CTS) is a common extracardiac manifestation of amyloidosis and usually predates overt cardiac amyloidosis (CA) by several years. Screening studies on patients undergoing CTS surgery have shown a low yield of CA (2.0%), but high prevalence of amyloid in the carpal ligament. The proportion of patients with amyloid in the carpal ligament who later develop CA is unknown.
The authors sought to investigate the prevalence of undiagnosed CA 5 to 15 years after surgery for bilateral CTS.
Using national registries, the authors identified subjects aged 60 to 85 years with prior CTS surgery, where the first procedure on the second wrist was performed 5 to 15 years earlier. Invitations to participate in the study were sent by mail. Per international recommendations, the initial cardiac evaluation included echocardiography, technetium-pyrophosphate scintigraphy, and assessment of monoclonal proteins in serum and urine.
A total of 250 subjects (35.7% of those invited) participated in the study. The median age was 70.4 years, and 50% were female. CA was diagnosed in 12 patients (4.8%; 95% CI: 2.5%-8.2%), and all cases were wild-type transthyretin amyloidosis (ATTRwt). The prevalence of ATTRwt in men was 8.8% (95% CI: 4.5%-15.2%; n = 11), and 21.2% (95% CI: 11.1%-34.7%) in male subjects ≥70 years with a BMI <30 kg/m. All but 2 patients diagnosed with ATTRwt were in the lowest disease severity score (Mayo score).
Screening for CA in patients with prior surgery for bilateral CTS finds approximately 5% with early-stage transthyretin CA. The clinical yield was higher (>1 in 5) when focusing on nonobese men ≥70 years, showing potential for systematic screening.
双侧腕管综合征(CTS)是淀粉样变性的一种常见心脏外表现,通常比明显的心脏淀粉样变性(CA)早数年出现。对接受 CTS 手术的患者进行筛查研究表明,CA 的检出率较低(2.0%),但腕韧带中的淀粉样物质检出率较高。腕韧带中有淀粉样物质但后来发展为 CA 的患者比例尚不清楚。
作者旨在调查双侧 CTS 手术后 5 至 15 年时未确诊 CA 的患病率。
作者利用国家登记处,确定了年龄在 60 至 85 岁之间、首次双侧第二腕部手术 5 至 15 年前的患者。通过邮件向患者发送了参加研究的邀请。根据国际建议,初始心脏评估包括超声心动图、焦磷酸盐放射性核素扫描以及血清和尿液中单克隆蛋白的评估。
共有 250 名患者(受邀者的 35.7%)参加了研究。中位年龄为 70.4 岁,50%为女性。12 名患者(4.8%;95%CI:2.5%-8.2%)诊断为 CA,所有病例均为野生型转甲状腺素蛋白淀粉样变性(ATTRwt)。男性中的 ATTRwt 患病率为 8.8%(95%CI:4.5%-15.2%;n=11),年龄≥70 岁且 BMI<30kg/m 的男性中患病率为 21.2%(95%CI:11.1%-34.7%)。除 2 名患者外,其余所有诊断为 ATTRwt 的患者的 Mayo 评分均最低。
对双侧 CTS 手术后的患者进行 CA 筛查发现,约有 5%的患者患有早期转甲状腺素蛋白 CA。当重点关注年龄≥70 岁且 BMI<30kg/m 的非肥胖男性时,临床检出率更高(1/5 以上),表明有进行系统筛查的潜力。