Objectives: This study sought to investigate the regional uptake of technetium 99m-pyrophosphate (TcPYP) in transthyretin cardiac amyloidosis (ATTR) and its association with mortality.
Background: TcPYP nuclear scintigraphy is a diagnostic and prognostic tool in ATTR. Echocardiography has identified a pattern of regional variation in longitudinal strain (LS) with a gradient of improved strain from base to apex in ATTR.
Methods: Consecutive patients with ATTR were evaluated who underwent TcPYP nuclear scintigraphy with planar and attenuation corrected single-photon emission computed tomography (SPECT). Heart-to-contralateral lung (H/CL) ratio was calculated on planar images, and left ventricular (LV) uptake was determined in each of the 17 segments using SPECT. A measure of apical-sparing of myocardial TcPYP uptake, termed the apical-sparing ratio (ASR), was calculated as basal + mid / apical counts.
Results: Overall, 54 patients with ATTR (age 78 ± 9 years, 76% male, 31% hereditary ATTR) were analyzed. There was increased TcPYP uptake in basal and mid relative to apical LV segments, and an apical-sparing LS pattern on echocardiography. The right ventricle similarly showed greater uptake in basal segments. There were 26 deaths over 1.8 years median follow-up. The ASR of TcPYP uptake was associated with age-adjusted all-cause mortality (p = 0.013) with worse prognosis seen at levels <2.75. Global LS was also prognostic (p = 0.01), whereas H/CL ratio and total LV uptake indexed to blood pool were not (p = 0.772 and p = 0.850, respectively). The prognostic utility of the ASR persisted in multivariable modeling (p = 0.003), whereas global LS did not.
Conclusions: There is decreased TcPYP uptake in apical as compared to mid and basal segments in the LV, mimicking apical-sparing LS seen on echocardiography. Regional distribution of LV TcPYP uptake is associated with mortality, whereas overall amount of uptake as measured by H/CL ratio and indexed LV SPECT uptake is not.
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http://dx.doi.org/10.1016/j.jcmg.2017.06.020 | DOI Listing |
Eur J Nucl Med Mol Imaging
January 2025
Department of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
Objective: Tafamidis has shown potential in slowing disease progression in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). This study aimed to evaluate serial changes on [Tc]Tc-pyrophosphate (PYP) scintigraphy during tafamidis treatment for hereditary ATTR-CM.
Methods: We retrospectively analyzed a prospectively collected cohort of Ala97Ser (A97S) hereditary ATTR-CM patients treated with tafamidis (61 mg/day) and a control group comprising A97S hereditary ATTR-CM patients who had not received disease-modifying medications.
J Card Fail
December 2024
Cardiology Division, Montefiore-Einstein Center for Heart and Vascular Care, Montefiore Medical Center, Albert Einstein College of Medicine. Bronx, NY, USA. Electronic address:
Background: Two diagnostic clinical scoring systems, the ATTR-CM Score and the T-AMYLO Score, have been proposed but not validated in diverse populations, despite Black race being an important risk factor for transthyretin amyloid cardiomyopathy (ATTR-CM). The aim of this study was to evaluate their performance in diagnosing ATTR-CM in a diverse patient cohort.
Methods: This retrospective single-center study analyzed patients who underwent a 99mTc-pyrophosphate single photon emission computed tomography scan (Tc-PYP) for workup of suspected ATTR-CM.
Clin Nucl Med
November 2024
From the Departments of Nuclear Medicine.
Amyloidosis is a misfolded protein deposition disorder within the extracellular matrix, leading to dysfunction in the affected organ. Primary amyloidosis manifests as AL and ATTR subtypes, wherein AL is associated with plasma cell dyscrasias. Herein we present a case of a patient who underwent investigation due to the presence of bilateral reticulonodular lung infiltrates, suggestive of miliary tuberculosis.
View Article and Find Full Text PDFClin Nucl Med
December 2024
Department of Nuclear Medicine, Fuwai Hospital, Chinese Academy of Medical Sciences, Shenzhen.
A 54-year-old woman with a history of end-stage renal disease was found to have infiltrative cardiomyopathy by echocardiography. 99m Tc-pyrophosphate ( 99m Tc-PYP) scintigraphy was positive with a remarkable myocardial uptake. Gene test found a mutation of AGXT , confirming a final diagnosis of primary hyperoxaluria.
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