Jafarisis Samira, Masoumi Shahab, Khezerlouy-Aghdam Naser, Toutounchi Kia Seyed, Sarmadian Amirreza Jabbaripour, Hamzehzadeh Sina, Shariati Akram, Parizad Razieh, Rabori Venus Shahabi
Cardiovascular Research Center, Tabriz University of Medical Sciences, Daneshgah Street, Tabriz, Eastern Azerbaijan, Iran.
Department of Cardiology, School of Medicine, Urmia University of Medical Sciences, Urmia, Iran.
BMC Cardiovasc Disord. 2024 Dec 20;24(1):734. doi: 10.1186/s12872-024-04415-8.
Concomitant cardiac amyloidosis (CA) and aortic stenosis (AS) may be mistaken for isolated AS, potentially impacting the treatment strategy and patient's prognosis. Therefore, it is crucial to distinguish between these conditions, as failure to promptly diagnose CA may lead to considerable complications. The aim of this study is to investigate the diagnostic value of strain predictors in patients with concomitant CA and AS compared to isolated AS.
Forty-two patients with severe AS suspected of concomitant CA based on a comprehensive clinical evaluation were selected to undergo mTc-DPD scintigraphy. Those showing Perugini grade 2 or 3 tracer uptakes without evidence of monoclonal gammopathy were diagnosed with CA and underwent speckle-tracking echocardiography. Furthermore, strain analysis was performed to evaluate myocardial deformation, with a focus on detecting apical sparing and reduction in bull's eye mapping, resulting in the characteristic "cherry on top" sign.
Eight patients were diagnosed with CA, representing 19.0% of those suspected of concomitant CA and 7.8% of the overall cohort with severe AS. AF arrhythmia was significantly more frequent in these patients compared to those with isolated AS. Echocardiography findings revealed that E/E' ratio and RALS were significantly higher in patients with concomitant CA, while GLS and mean basal LS were significantly lower in this group. The "cherry on top" sign was detected in 19 patients (45.2%), present in 100% of those with concomitant CA and AS, versus 32.4% in isolated AS cases (P = 0.04). This sign demonstrated a sensitivity of 100% and a specificity of 67.6% for predicting concomitant CA and AS.
In conclusion, the "cherry on top" sign was significantly more prevalent in patients with concomitant CA and AS, compared to those with isolated AS, demonstrating a sensitivity of 100% and a specificity of 67.6% for predicting concomitant CA. Moreover, RALS and E/E' ratios were significantly higher in patients with concomitant CA, whereas GLS and mean basal LS were significantly lower in this group.
心脏淀粉样变性（CA）与主动脉瓣狭窄（AS）并存时可能被误诊为孤立性AS，这可能会影响治疗策略和患者预后。因此，区分这两种情况至关重要，因为未能及时诊断CA可能会导致相当多的并发症。本研究的目的是探讨应变预测指标在CA与AS并存患者与孤立性AS患者中的诊断价值。
基于全面的临床评估，选择42例疑似合并CA的重度AS患者进行99mTc-DPD心肌显像。那些显示佩鲁吉尼分级为2级或3级示踪剂摄取且无单克隆丙种球蛋白病证据的患者被诊断为CA，并接受斑点追踪超声心动图检查。此外，进行应变分析以评估心肌变形，重点是检测心尖保留和靶心图减少，从而产生特征性的“顶上樱桃”征。
8例患者被诊断为CA，占疑似合并CA患者的19.0%，占重度AS总体队列的7.8%。与孤立性AS患者相比，这些患者中房颤心律失常明显更常见。超声心动图结果显示，CA合并患者的E/E'比值和右房纵向应变（RALS）明显更高，而该组患者的整体纵向应变（GLS）和平均基底段纵向应变明显更低。19例患者（45.2%）检测到“顶上樱桃”征，在CA与AS并存的患者中100%出现，而在孤立性AS病例中为32.4%（P = 0.04）。该征预测CA与AS并存的敏感性为100%，特异性为67.6%。
总之，与孤立性AS患者相比，“顶上樱桃”征在CA与AS并存患者中明显更常见，预测合并CA的敏感性为100%，特异性为67.6%。此外，CA合并患者的RALS和E/E'比值明显更高，而该组患者的GLS和平均基底段纵向应变明显更低。
