Stan Claudiu, Mititelu Raluca, Adam Robert Daniel, Jurcuţ Ruxandra
Department of Nuclear Medicine and Ultrasonography, Fundeni Clinical Institute, 022328 Bucharest, Romania.
Department of Cardiothoracic Pathology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania.
Diagnostics (Basel). 2022 Feb 21;12(2):556. doi: 10.3390/diagnostics12020556.
Amyloidosis is a heterogeneous group of diseases caused by the extracellular deposition of amyloid insoluble fibrils in multiple organs, resulting in various clinical manifestations. Cardiac amyloidosis (CA) occurs mainly in primary light-chain (AL) amyloidosis, hereditary transthyretin (ATTRv) amyloidosis and senile or wild-type transthyretin (ATTRwt) amyloidosis. Knowing that myocardial uptake at bone scintigraphy is an essential step in the ATTR-CA diagnostic algorithm, the level of awareness among nuclear medicine physicians (NMPs) using bone tracer scintigraphy is of great importance. The objective of the study was to evaluate NMPs' awareness of scintigraphy with bisphosphonates for the detection of CA. We conducted an online survey among NMPs from Romania to assess their current awareness and state of knowledge of nuclear techniques used in CA. Among the total 65 Romanian NMPs, 35 (53%) responded to this questionnaire. Approximately three-quarters of participants (74%) found a diffuse accumulation of bisphosphonates in the heart on scintigraphy performed for bone pathology as an incidental discovery. Detection of myocardial uptake of 99mTc-labeled bisphosphonates on scintigraphy suggests CA-AL for 3% of participants and for 9% of respondents, the appearance is of uncertain cardiac amyloidosis, while 5% of participants observed cardiac uptake but did not report it as CA. Even if more than half of those who responded to this survey (54%) found abnormal cardiac uptake and interpreted it as CA-ATTR, only 14% contacted the referring physician to draw attention to the incidental discovery to refer the patient to a specialist in rare genetic cardiomyopathy. Regarding the knowledge about the categories of bisphosphonates recommended in the diagnosis of CA-ATTR, 54% answered inadequately that methylene diphosphonate (MDP) could be used. Romanian nuclear physicians are partially familiar with CA diagnosis by scintigraphy, but its diagnostic potential and standardization, recommended radiotracers and acquisition times and interpretation algorithms are known in varying proportions. Therefore, there is a need to enhance knowledge through continuing medical education programs in order to standardize the protocols for the acquisition, processing and interpretation of bisphosphonate scintigraphy for the detection of cardiac ATTR amyloidosis.
淀粉样变性是一组异质性疾病,由淀粉样不溶性纤维在多个器官中的细胞外沉积引起,导致各种临床表现。心脏淀粉样变性(CA)主要发生在原发性轻链(AL)淀粉样变性、遗传性转甲状腺素蛋白(ATTRv)淀粉样变性以及老年或野生型转甲状腺素蛋白(ATTRwt)淀粉样变性中。鉴于骨闪烁显像时心肌摄取是ATTR-CA诊断算法中的关键步骤,使用骨显像剂进行闪烁显像的核医学医师(NMPs)的认知水平至关重要。本研究的目的是评估NMPs对双膦酸盐闪烁显像检测CA的认知情况。我们对罗马尼亚的NMPs进行了一项在线调查,以评估他们对CA中使用的核技术的当前认知和知识水平。在罗马尼亚的65名NMPs中,35名(53%)回复了这份问卷。约四分之三的参与者(74%)在因骨病变进行的闪烁显像中偶然发现心脏有双膦酸盐的弥漫性积聚。在闪烁显像中检测到99mTc标记的双膦酸盐的心肌摄取,3%的参与者认为提示CA-AL,9%的受访者认为表现为心脏淀粉样变性情况不明,而5%的参与者观察到心脏摄取但未将其报告为CA。即使在回复此调查的人中,超过一半(54%)发现心脏摄取异常并将其解释为CA-ATTR,但只有14%的人联系转诊医生,提请注意这一偶然发现,以便将患者转诊给罕见遗传性心肌病专家。关于CA-ATTR诊断中推荐的双膦酸盐类别知识,54%的回答不充分,认为可以使用亚甲基二膦酸盐(MDP)。罗马尼亚核医学医师对通过闪烁显像诊断CA有一定了解,但对其诊断潜力和标准化、推荐的放射性示踪剂和采集时间以及解读算法的了解程度各不相同。因此,需要通过继续医学教育项目来加强知识,以便规范用于检测心脏ATTR淀粉样变性的双膦酸盐闪烁显像的采集、处理和解读方案。