Priyadharshini V M Vimala, Muthukrishnan Indirani, Simon Shelley
Department of Nuclear medicine, Apollo Hospitals, Chennai, Tamil Nadu, India.
Indian J Nucl Med. 2025 Mar-Apr;40(2):79-87. doi: 10.4103/ijnm.ijnm_156_24. Epub 2025 Jun 27.
Amyloidosis is characterized by the extracellular deposition of fibrillar proteins, leading to tissue damage and dysfunction. Cardiac amyloidosis (CA) occurs when these fibrils accumulate in the heart's extracellular matrix, causing infiltrative cardiomyopathy, heart failure, and potentially death. Early diagnosis is imperative in accurate management. Transthyretin amyloidosis, either hereditary or age-related, is a common cause of CA, often presenting with left ventricular hypertrophy (LVH) and heart failure with preserved ejection fraction (HFpEF). This study investigates the utility of Tc-99 m Pyrophosphate (Tc-99 m PYP) imaging in diagnosing Transthyretin Amyloidosis ((ATTR)-CA) in patients with unexplained LVH.
This prospective observational study, conducted between January 2023 and June 2024, involved 52 patients with clinical suspicion of CA and left ventricular wall thickness >12 mm. Patients underwent Tc-99 m PYP scintigraphy, and the results were categorized as positive, negative, or equivocal.
The study population's mean age was 64.6 years, with a male predominance (69%). Of the 52 patients, 9 (17.3%) had positive, 28 (53.8%) negative, and 15 (28.8%) equivocal Tc-99 m PYP scan results. Echocardiographic features, such as septal hypertrophy and granular speckled appearance, were significantly associated with positive scan findings. Comorbidities such as coronary artery disease and dyslipidemia were also common. Cardiac biomarkers NT-proBNP and troponin I showed elevated levels in patients with positive scan results.
Tc-99 m PYP imaging is an effective noninvasive tool for diagnosing ATTR CA, particularly in patients with unexplained LVH. The presence of HFpEF, abnormal ECG, and restrictive features on echocardiography should prompt further investigation for CA. Early diagnosis allows for timely management, improving patient outcomes.
淀粉样变性的特征是纤维状蛋白在细胞外沉积,导致组织损伤和功能障碍。当这些纤维在心脏细胞外基质中积聚时,就会发生心脏淀粉样变性(CA),导致浸润性心肌病、心力衰竭,并可能导致死亡。早期诊断对于准确治疗至关重要。转甲状腺素蛋白淀粉样变性,无论是遗传性还是与年龄相关的,都是CA的常见原因,通常表现为左心室肥厚(LVH)和射血分数保留的心力衰竭(HFpEF)。本研究调查了锝-99m焦磷酸盐(Tc-99m PYP)成像在诊断原因不明的LVH患者的转甲状腺素蛋白淀粉样变性((ATTR)-CA)中的效用。
这项前瞻性观察性研究于2023年1月至2024年6月进行,纳入了52例临床怀疑患有CA且左心室壁厚度>12mm的患者。患者接受了Tc-99m PYP闪烁扫描,结果分为阳性、阴性或不确定。
研究人群的平均年龄为64.6岁,男性占主导(69%)。在52例患者中,9例(17.3%)Tc-99m PYP扫描结果为阳性,28例(53.8%)为阴性,15例(28.8%)为不确定。超声心动图特征,如室间隔肥厚和颗粒状斑点外观,与扫描阳性结果显著相关。冠状动脉疾病和血脂异常等合并症也很常见。心脏生物标志物NT-proBNP和肌钙蛋白I在扫描结果为阳性的患者中水平升高。
Tc-99m PYP成像对于诊断ATTR CA是一种有效的非侵入性工具,特别是在原因不明的LVH患者中。HFpEF、异常心电图以及超声心动图上的限制性特征的存在应促使对CA进行进一步检查。早期诊断有助于及时治疗,改善患者预后。