University of Chicago Medical Center, Chicago, IL, United States.
Medical College of Milwaukee Medical Center, Milwaukee, IL, United States.
Curr Probl Cardiol. 2024 Apr;49(4):102417. doi: 10.1016/j.cpcardiol.2024.102417. Epub 2024 Jan 25.
Limited data exists on the prognostic impact of valvular heart disease in cardiac amyloidosis (CA). We therefore sought to define the prevalence of valvular disease in patients with CA and assess the effects of significant valve disease on survival.
This multi-center retrospective cohort study included consecutive patients with confirmed transthyretin (TTR) or light chain (AL) amyloidosis. Echocardiographic data closest to the date of amyloid diagnosis was reviewed, and severity was graded according to ASE guidelines. Kaplan-Meier survival analysis was performed to compare survival between patients with moderate or greater valve disease against those with mild or less disease.
We included 345 patients (median age 76 years; 73 % men; 110 AL, 235TTR). The median survival for the total patient cohort with cardiac amyloidosis was 2.92 years, with 30 % of patients surviving at five years after their diagnosis. Median survival comparing AL vs ATTR was 2.58 years vs 2.82 years (p = 0.67) The most common valvular abnormalities in the total cohort were mitral (62 %) and tricuspid (66 %).regurgitation There was a statistically significant difference in median survival between patients with no or mild MR compared to those with moderate or severe MR (2.92 years vs 3.35 years, p = 0.0047) (Fig. 5). There was a statistically significant difference in median survival in patients with no or mild TR compared to those with moderate or severe TR (3.35 years vs 2.3 years, p = 0.015).
Our study demonstrates a significant prevalence of mitral and tricuspid regurgitation in CA, with patients with moderate to severe MR and TR having a poorer prognosis.
心脏淀粉样变性（CA）中瓣膜性心脏病的预后影响数据有限。因此，我们旨在确定 CA 患者中瓣膜疾病的患病率，并评估严重瓣膜疾病对生存的影响。
这项多中心回顾性队列研究纳入了经证实的转甲状腺素（TTR）或轻链（AL）淀粉样变性患者。回顾了最接近淀粉样变性诊断日期的超声心动图数据，并根据 ASE 指南对严重程度进行分级。采用 Kaplan-Meier 生存分析比较中度或更严重瓣膜疾病患者与轻度或更轻瓣膜疾病患者的生存情况。
共纳入 345 例患者（中位年龄 76 岁；73%为男性；110 例 AL，235 例 TTR）。整个 CA 患者队列的中位总生存时间为 2.92 年，30%的患者在诊断后 5 年内存活。AL 与 ATTR 相比，中位生存时间分别为 2.58 年和 2.82 年（p=0.67）。总队列中最常见的瓣膜异常是二尖瓣（62%）和三尖瓣（66%）反流。在无或轻度 MR 患者与中度或重度 MR 患者之间，中位生存时间存在统计学显著差异（2.92 年 vs 3.35 年，p=0.0047）（图 5）。在无或轻度 TR 患者与中度或重度 TR 患者之间，中位生存时间也存在统计学显著差异（3.35 年 vs 2.3 年，p=0.015）。
我们的研究表明 CA 中二尖瓣和三尖瓣反流的患病率显著，中重度 MR 和 TR 的患者预后更差。
