Ladefoged Bertil, Dybro Anne, Povlsen Jonas Agerlund, Vase Henrik, Clemmensen Tor Skibsted, Poulsen Steen Hvitfeldt
Department of Cardiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, 8200 Aarhus, N, Denmark.
Department of Cardiology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, 8200 Aarhus, N, Denmark.
Int J Cardiol. 2020 Apr 1;304:138-143. doi: 10.1016/j.ijcard.2019.12.063. Epub 2020 Jan 25.
To determine the diagnostic delay in patients with wild-type transthyretin cardiac amyloiodosis (ATTRwt). To determine the clinical and echocardiogtraphic characteristics of patients with an early and a late diagnosis and to study the suspected diagnoses and identification of diagnostic "red flags" before the ATTRwt diagnosis was established.
In 50 consecutive patients with ATTRwt diagnosed from 2017 to 2019, clinical and echocardiographic patient characteristics were investigated based on electronic patient charts and echocardiographic database review at Aarhus University Hospital, Denmark.
The median diagnostic delay was 13 months (2-47 months) and a diagnostic delay above 3 months was associated with more advanced symptoms and left ventricular (LV) diastolic dysfunction at the time of the diagnosis. Thirty patients (60%) were investigated for at least two non-ATTRwt diagnoses during the time period from the first cardiac examination to the time of the confirmed diagnosis. ATTR red flags were significantly less used in patients with the longest diagnostic delay (p < 0.001). Abormal LV global longitudinal strain (LV-GLS < 18%) and apical sparring ratio (APSR ≥ 1.5) were present in 96% and 94% of the ATTRwt patients, respectively.
The diagnostic delay in ATTRwt was substantial and a prolonged diagnostic delay was associated with more advanced symptoms and LV diastolic dysfunction at the time of the diagnosis. Established ATTR red flags are poorly utilized in the diagnostic process. Echocardiographic analysis of LV-GLS and APSR contributes significantly to the evaluation of LV myocardial performance and helps raise the suspicion of ATTRwt.
确定野生型转甲状腺素蛋白心脏淀粉样变性（ATTRwt）患者的诊断延迟情况。确定早期诊断和晚期诊断患者的临床及超声心动图特征，并研究在ATTRwt诊断确立之前的疑似诊断及诊断“红旗”标识的识别情况。
对2017年至2019年连续诊断的50例ATTRwt患者，基于丹麦奥胡斯大学医院的电子病历和超声心动图数据库回顾，研究患者的临床和超声心动图特征。
中位诊断延迟为13个月（2 - 47个月），诊断延迟超过3个月与诊断时更严重的症状及左心室（LV）舒张功能障碍相关。从首次心脏检查到确诊期间，30例患者（60%）至少接受过两种非ATTRwt诊断的检查。诊断延迟最长的患者中，ATTR红旗标识的使用显著减少（p < 0.001）。分别有96%和94%的ATTRwt患者存在异常的左心室整体纵向应变（LV-GLS < 18%）和心尖 sparing 比率（APSR≥1.5）。
ATTRwt的诊断延迟时间较长，诊断延迟时间延长与诊断时更严重的症状及LV舒张功能障碍相关。在诊断过程中，既定的ATTR红旗标识未得到充分利用。对LV-GLS和APSR进行超声心动图分析有助于显著评估LV心肌功能，并有助于提高对ATTRwt的怀疑。
