Intensive Cardiac Care Unit, Heart and Vessel Department, Azienda Ospedaliero Universitaria Careggi, University of Florence, Largo Brambilla 3, 50127 Florence, Italy.
Eur Heart J Cardiovasc Imaging. 2012 May;13(5):416-22. doi: 10.1093/ejechocard/jer289. Epub 2011 Dec 16.
The importance of right ventricle (RV) dysfunction in AL amyloidosis has been underestimated. This study was designed to comprehensively evaluate RV function and its prognostic role in patients with AL amyloidosis with and without echocardiographic evidence of cardiac involvement.
Fifty-two biopsy-proven AL amyloidosis patients underwent a thorough echocardiographic evaluation. Twenty-seven patients (CA) met the international echocardiographic criteria for cardiac involvement [left ventricular (LV) wall thickness ≥ 12 mm] and 25 patients had no cardiac amyloidosis features (NCA). Patients were compared with a sex- age-matched control group. Patients and controls underwent traditional, tissue Doppler (TDI), speckle-tracking left and RV echocardiographic evaluation. No difference was observed between groups in RV diastolic diameter, whereas CA patients showed increased RV free wall thickness (P< 0.0001). Compared with controls and NCA patients, traditional echocardiography, TDI, and speckle-tracking evaluation detected significantly (P< 0.0001) depressed RV longitudinal systolic function in CA patients. No difference was observed between groups at Doppler diastolic evaluation, whereas at tricuspidal annulus TDI analysis, CA subject showed significantly lower E' and A' values with increased E/E' ratio (P< 0.0001). Over a 19 months median follow-up period, 18 patients died. Cox multivariate analysis showed that N-terminal pro-Brain natriuretic peptide and RV longitudinal strain were the strongest death predictor.
Our data show that in patients with AL amyloidosis, RV involvement develops later than LV amyloid deposition but when it occurs, prognosis dramatically worsens. Moreover RV longitudinal strain was the only echocardiographic predictor of prognosis. We suggest that RV function analysis should be performed routinely as a part of echocardiographic evaluation in these patients.
右心室（RV）功能障碍在 AL 淀粉样变中的重要性被低估了。本研究旨在全面评估 RV 功能及其在有和无超声心动图心脏受累证据的 AL 淀粉样变患者中的预后作用。
52 例经活检证实的 AL 淀粉样变患者接受了全面的超声心动图评估。27 例患者（CA）符合国际超声心动图心脏受累标准[左心室（LV）壁厚度≥12mm]，25 例患者无心脏淀粉样变特征（NCA）。将患者与性别、年龄匹配的对照组进行比较。患者和对照组接受了传统、组织多普勒（TDI）、斑点追踪左室和 RV 超声心动图评估。RV 舒张直径在各组之间无差异，而 CA 患者的 RV 游离壁厚度增加（P<0.0001）。与对照组和 NCA 患者相比，传统超声心动图、TDI 和斑点追踪评估检测到 CA 患者 RV 纵向收缩功能明显（P<0.0001）降低。各组在多普勒舒张评估方面无差异，而在三尖瓣环 TDI 分析中，CA 患者的 E'和 A'值明显较低，E/E'比值增加（P<0.0001）。在 19 个月的中位随访期间，18 例患者死亡。Cox 多变量分析显示，N 末端脑钠肽前体和 RV 纵向应变是死亡的最强预测因素。
我们的数据表明，在 AL 淀粉样变患者中，RV 受累的发生晚于 LV 淀粉样物质沉积，但当发生时，预后显著恶化。此外，RV 纵向应变是唯一的超声心动图预后预测因子。我们建议，在这些患者中，应常规进行 RV 功能分析，作为超声心动图评估的一部分。
