Cardiovascular Division, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street Boston, MA 02115, USA.
Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Clinical Research, Innovation and Education Center, Tohoku University Hospital, Seiryo-machi 1-1 Aobaku Sendai, Miyagi 980-8574, Japan.
Eur Heart J Cardiovasc Imaging. 2017 Oct 1;18(10):1128-1137. doi: 10.1093/ehjci/jex097.
Although cardiac amyloidosis (CA) is characterized by significant left atrial (LA) dilatation, the characteristics of LA function remain to be fully investigated.
We assessed LA function by speckle-tracking echocardiography in 124 patients with CA and sinus rhythm: 68 with light chain (AL), 29 with mutant (ATTRm), 27 with wild-type (ATTRwt) transthyretin amyloidosis. Conventional and strain-derived parameters, including LA peak longitudinal strain (LS) and strain rate (peak LSR: reservoir function; early LSR: conduit function; late LSR: active function), were assessed compared between CA patients and 20 healthy controls of similar age and gender. All LA function phases, including LA longitudinal strain, peak LSR, early and late LSR were significantly impaired in CA compared to healthy controls after adjusting for LA size, LV ejection fraction and LV filling pressures (E/E') (all P < 0.05). Peak LA LS was moderately correlated with LV global LS (R = -0.60, P < 0.001); late LSR was correlated with A wave at the level of LV inflow (R = -0.69, P < 0.001). Among the different CA subtypes, peak LS and LA active emptying fraction were worse in ATTRwt than AL and ATTRm [P < 0.05 after adjustment for age, sex, body mass index, systolic blood pressure, heart rate, LA volume index, severity of mitral regurgitation, left ejection fraction, and left ventricular end-diastolic pressure (E/E')].
In CA, LA function was severely impaired and highly correlated with LV deformation. Differences in LA function between amyloid subtypes suggest that amyloid aetiology plays a role in the pathophysiology of cardiac dysfunction in CA.
尽管心脏淀粉样变性（CA）以显著的左心房（LA）扩张为特征，但 LA 功能的特征仍有待充分研究。
我们通过斑点追踪超声心动图评估了 124 例 CA 伴窦性节律患者的 LA 功能：68 例轻链（AL），29 例突变（ATTRm），27 例野生型（ATTRwt）转甲状腺素蛋白淀粉样变性。与 20 名年龄和性别相匹配的健康对照相比，评估了包括 LA 峰值纵向应变（LS）和应变率（峰值 LSR：储备功能；早期 LSR：输送功能；晚期 LSR：主动功能）在内的常规和应变衍生参数。在调整 LA 大小、LV 射血分数和 LV 充盈压（E/E'）后，与健康对照组相比，所有 CA 患者的 LA 功能各阶段，包括 LA 纵向应变、峰值 LSR、早期和晚期 LSR 均显著受损（均 P<0.05）。峰值 LA LS 与 LV 整体 LS 中度相关（R=-0.60，P<0.001）；晚期 LSR 与 LV 流入水平的 A 波相关（R=-0.69，P<0.001）。在不同的 CA 亚型中，ATTRwt 的峰值 LS 和 LA 主动排空分数比 AL 和 ATTRm 差[调整年龄、性别、体重指数、收缩压、心率、LA 容积指数、二尖瓣反流严重程度、左射血分数和左心室舒张末期压（E/E'）后 P<0.05]。
在 CA 中，LA 功能严重受损，与 LV 变形高度相关。淀粉样变亚型之间 LA 功能的差异表明，淀粉样变病因在 CA 心脏功能障碍的病理生理学中起作用。
