PDF(Pubmed)
Abstract：
UNASSIGNED: This study has been conducted to investigate the non-invasive diagnostic journey of patients with a transthyretin amyloid cardiomyopathy (aTTR-CM) in Turkey, identify the challenges and uncertainties encountered on the path to diagnosis from the perspectives of expert physicians, and develop recommendations that can be applied in such cases.
UNASSIGNED: This study employed a three-round modified Delphi method and included 10 cardiologists and five nuclear medicine specialists. Two hematologists also shared their expert opinions on the survey results related to hematological tests during a final face-to-face discussion. A consensus was reached when 80% or more of the panel members marked the \"agree/strongly agree\" or \"disagree/strongly disagree\" option.
UNASSIGNED: The panelists unanimously agreed that the aTTR-CM diagnosis could be established through scintigraphy (using either 99mTc-PYP, 99mTc-DPD, or 99mTc-HMPD) in a patient with suspected cardiac amyloidosis (CA) without a further investigation if AL amyloidosis is ruled out (by sFLC, SPIE and UPIE). In addition, scintigraphy imaging performed by SPECT or SPECT-CT should reveal a myocardial uptake of Grade ≥2 with a heart-to-contralateral (H/CL) ratio of ≥1.5. The cardiology panelists recommended using cardiovascular magnetic resonance (CMR) and a detailed echocardiographic scoring as a last resort before considering an endomyocardial biopsy in patients with suspected CA whose scintigraphy results were discordant/inconclusive or negative but still carried a high clinical suspicion of aTTR-CM.
UNASSIGNED: The diagnostic approach for aTTR-CM should be customized based on the availability of diagnostic tools/methods in each expert clinic to achieve a timely and definitive diagnosis.
UNASSIGNED: This study employed a three-round modified Delphi method and included 10 cardiologists and five nuclear medicine specialists. Two hematologists also shared their expert opinions on the survey results related to hematological tests during a final face-to-face discussion. A consensus was reached when 80% or more of the panel members marked the \"agree/strongly agree\" or \"disagree/strongly disagree\" option.
UNASSIGNED: The panelists unanimously agreed that the aTTR-CM diagnosis could be established through scintigraphy (using either 99mTc-PYP, 99mTc-DPD, or 99mTc-HMPD) in a patient with suspected cardiac amyloidosis (CA) without a further investigation if AL amyloidosis is ruled out (by sFLC, SPIE and UPIE). In addition, scintigraphy imaging performed by SPECT or SPECT-CT should reveal a myocardial uptake of Grade ≥2 with a heart-to-contralateral (H/CL) ratio of ≥1.5. The cardiology panelists recommended using cardiovascular magnetic resonance (CMR) and a detailed echocardiographic scoring as a last resort before considering an endomyocardial biopsy in patients with suspected CA whose scintigraphy results were discordant/inconclusive or negative but still carried a high clinical suspicion of aTTR-CM.
UNASSIGNED: The diagnostic approach for aTTR-CM should be customized based on the availability of diagnostic tools/methods in each expert clinic to achieve a timely and definitive diagnosis.
摘要：
■这项研究旨在调查土耳其甲状腺素运载蛋白淀粉样心肌病（aTTR-CM）患者的非侵入性诊断过程，从专家医生的角度识别诊断路径上遇到的挑战和不确定性，并制定可适用于此类案件的建议。
■本研究采用三轮改良德尔菲法，包括10名心脏病专家和5名核医学专家。两位血液学家还在最后的面对面讨论中分享了他们对与血液学测试相关的调查结果的专家意见。当80％或更多的小组成员标记“同意/强烈同意”或“不同意/强烈不同意”选项时，达成了共识。
■小组成员一致认为，aTTR-CM诊断可以通过闪烁显像术（使用99mTc-PYP，99mTc-DPD,或99mTc-HMPD）在疑似心脏淀粉样变性（CA）的患者中，如果排除AL淀粉样变性，则未经进一步调查（通过sFLC，SPIE和UPIE）。此外,通过SPECT或SPECT-CT进行的闪烁显像成像应显示心肌摄取≥2级，心脏与对侧（H/CL）的比率≥1.5。心脏病学专家建议使用心血管磁共振（CMR）和详细的超声心动图评分作为最后的手段，然后考虑对可疑CA的患者进行心内膜活检，其闪烁显像结果不一致/不确定或阴性，但临床上仍高度怀疑aTTR-CM。
■aTTR-CM的诊断方法应根据每个专家门诊中诊断工具/方法的可用性进行定制，以实现及时和明确的诊断。
■本研究采用三轮改良德尔菲法，包括10名心脏病专家和5名核医学专家。两位血液学家还在最后的面对面讨论中分享了他们对与血液学测试相关的调查结果的专家意见。当80％或更多的小组成员标记“同意/强烈同意”或“不同意/强烈不同意”选项时，达成了共识。
■小组成员一致认为，aTTR-CM诊断可以通过闪烁显像术（使用99mTc-PYP，99mTc-DPD,或99mTc-HMPD）在疑似心脏淀粉样变性（CA）的患者中，如果排除AL淀粉样变性，则未经进一步调查（通过sFLC，SPIE和UPIE）。此外,通过SPECT或SPECT-CT进行的闪烁显像成像应显示心肌摄取≥2级，心脏与对侧（H/CL）的比率≥1.5。心脏病学专家建议使用心血管磁共振（CMR）和详细的超声心动图评分作为最后的手段，然后考虑对可疑CA的患者进行心内膜活检，其闪烁显像结果不一致/不确定或阴性，但临床上仍高度怀疑aTTR-CM。
■aTTR-CM的诊断方法应根据每个专家门诊中诊断工具/方法的可用性进行定制，以实现及时和明确的诊断。
