Cardiomyopathy Unit, Cardiothoracic and Vascular Department, Careggi University Hospital, Florence, Italy; Geriatric Intensive Care Unit, Division of Geriatric Cardiology, Careggi University Hospital, Florence, Italy.
Cardiomyopathy Unit, Cardiothoracic and Vascular Department, Careggi University Hospital, Florence, Italy.
Mayo Clin Proc. 2021 Aug;96(8):2185-2191. doi: 10.1016/j.mayocp.2021.04.021.
Whether diagnostic timing in transthyretin (TTR) cardiac amyloidosis (CA) predisposes patients to worse outcomes is unresolved. We aimed to describe the long-term association of diagnostic timing (time from first onset of symptoms consistent with CA leading to medical contact to definitive diagnosis) with mortality in patients with wild-type TTR-CA (ATTRwt-CA). Overall, we reviewed the medical records of 160 patients seen at a tertiary care amyloidosis unit from January 1, 2016, to January 1, 2020 (median [interquartile range] follow-up, 21 [10 to 34] months), and compared them by survival. Median diagnostic timing was 4 (2 to 12) months and was longer in nonsurvivors (9 [3 to 15] vs 3 [1 to 7] months; P<.001). Patients diagnosed 6 or more months after symptom onset had higher mortality, with a median survival of 30 months (95% CI, 22 to 37 months). On Cox multivariable analysis, timing was independently associated with all-cause mortality (hazard ratio per month increase, 1.049 [95% CI, 1.017 to 1.083]) together with age at diagnosis, disease stage, New York Heart Association class, and coronary artery disease. In conclusion, diagnostic timing of ATTRwt-CA is associated with mortality. Timely diagnosis is warranted whenever "red flags" are present.
诊断时机是否会影响转甲状腺素蛋白（TTR）心脏淀粉样变（CA）患者的预后尚不清楚。我们旨在描述诊断时机（从首次出现符合 CA 的症状到首次医疗接触以明确诊断的时间）与野生型 TTR-CA（ATTRwt-CA）患者死亡率之间的长期关联。总的来说，我们回顾了 2016 年 1 月 1 日至 2020 年 1 月 1 日在一家三级护理淀粉样变性单位就诊的 160 例患者的病历，按生存情况进行了比较。中位诊断时机为 4（2 至 12）个月，且在死亡患者中较长（9 [3 至 15] vs 3 [1 至 7] 个月；P<.001）。症状发作后 6 个月以上诊断的患者死亡率更高，中位生存期为 30 个月（95%CI，22 至 37 个月）。Cox 多变量分析显示，时机与全因死亡率独立相关（每月增加的风险比，1.049 [95%CI，1.017 至 1.083]），同时与诊断时的年龄、疾病分期、纽约心脏协会（NYHA）分级和冠状动脉疾病相关。总之，ATTRwt-CA 的诊断时机与死亡率相关。只要出现“警示信号”，就应及时诊断。
