心脏淀粉样变性合并脑梗死的单中心诊疗经验

Single-center experience of diagnosis and strategy in patients with cardiac amyloidosis and cerebral infarction

目的探讨心脏淀粉样变性(CA)合并脑梗死患者的临床特征及预后。方法 收集2010年1月至2017年1月在北京协和医院住院的46例CA患者,其中23例合并脑梗死为观察组(脑梗死组),其他为对照组(无脑梗死组)。结果 46例CA患者年龄57.0(51.5,64.0)岁,其中男性26例(56.5%),淀粉样轻链蛋白型淀粉样变性32例(69.7%),合并心房颤动/心房扑动/房性心动过速18例(39.1%),限制型心肌病14例(30.4%)。脑梗死组患者收缩压[(98.4±23.9)mmHg比(121.7±19.2)mmHg(1 mmHg=0.133 kPa),P=0.014]、κ型游离轻链(3/23比14/23,P=0.010)显著低于无脑梗死组,而沙利度胺(16/23比7/23,P =0.042)、螺内酯(15/23比5/23,P =0.026)及袢利尿药(19/23比9/23,P=0.035)显著高于无脑梗死组,差异均有统计学意义。46例患者随访10.5(3.0,30.8)个月,死亡26例(56.5%),其中脑梗死组21例。Cox多因素回归分析显示,脑梗死(OR12.7,95% CI 2.6~62.8,P=0.002)为全因死亡的独立预测因子,而口服抗凝药物(OR 0.3,95% CI0.1~0.9,P=0.036)则能显著降低全因死亡率。Kaplan-Meier生存分析示脑梗死组1、5、10年生存率为(91.3±5.9)%、(78.3±8.6)%及(47.8±10.4)%,脑梗死组生存率及生存时间显著低于无脑梗死组(Log-Rank χ2=22.8,P<0.001)。结论低灌注压、心房颤动、左心室腔大、左心功能下降、服用沙利度胺是CA合并脑梗死的可能易感因素。脑梗死增加CA的全因死亡风险。

Objective To explore the clinical features and prognosis of patients with cardiac amyloidosis (CA) and cerebral infarction. Methods A case-control study, 46 patients with CA were collected in Peking union medical college hospital from December 2010 to December 2018, including 23 patients with cerebral infarction(case group) and the rest as the control group. Results The median age of total cases was 57.0 (51.5,64.0) years,including 26(56.5%)of males,32(69.7%) with AL amyloidosis,18 (39.1%) with atrial fibrillation/atrial fl utter/atrial velocity,and 14(30.4%)with restricted cardiomyopathy. Compared with the control group, case group manifestated with decreased systolic blood pressure[(98.4±23.9) mmHg vs.(121.7±19.2)mmHg(1 mmHg=0.133 kPa), P=0.014],more free light-chain proteins of λ type but less κ type (3/23 vs. 14/23, P=0.010), and more prescriptions of thalidotamine(16/23 vs. 7/23,P=0.042),spironolactone(15/23 vs. 5/23, P=0.026)and loop diuretics (19/23 vs. 9/23,P=0.035). During a median follow-up of 10.5 (3.0, 30.8) months,26 patients died, including 21 patients with cerebral infarction. Multivariant Cox regression analysis discovered that cerebral infarction was an independent predictor of all-cause mortality (OR 12.7,95%CI 2.6–62.8,P=0.002),and anticoagulants signifi cantly reduced all-cause mortality (OR 0.3,95%CI 0.1–0.9,P=0.0036). Kaplan-Meier analysis showed that the 1-year, 5-year and 10-year survival rates were(91.3±5.9)%,(78.3±8.6)% and (47.8±10.4)% in patients with CA and cerebral infarction, repectively. Survival rate and survival time in the case group were signifi cantly decreased than those in the control group(Log-Rank χ2= 22.8, P<0.001). Conclusions Decreased systemic perfusion pressure, atrial fibrillation, enlarged left ventricle, impaired left ventricular function and thalidomide are possible risk factors of cerebral infarction in patients with CA. Cerebral infarction increases the risk of all-cause death in CA cases.