Annals of Nuclear Cardiology
Online ISSN : 2424-1741
Print ISSN : 2189-3926
ISSN-L : 2189-3926
Advance online publication
Displaying 1-2 of 2 articles from this issue
  • Ken-ichi Hirano, Keita Kodama, Hideyuki Miyauchi, Yasuyuki Nagasawa, Y ...
    Article type: Letter to Editor
    Article ID: 23-00014
    Published: 2023
    Advance online publication: December 22, 2023
    JOURNAL OPEN ACCESS FULL-TEXT HTML ADVANCE PUBLICATION
    Triglyceride deposit cardiomyovasculopathy (TGCV) is an emerging rare heart disease with high mortality, characterized by defective intracellular lipolysis of triglycerides (TG). We developed diagnostic criteria for TGCV, in which low washout rate of BMIPP (BMIPP-WR) is a key factor. The working group of the Japan Society of Nuclear Cardiology recently published practice recommendations for measuring BMIPP-WR. We reported that hemodialysis (HD) patients with TGCV exhibited a markedly higher cardiovascular risk than those without TGCV. Secondary carnitine deficiency is common in patients undergoing HD, as carnitine is removed from the circulation. However, clinical evidence linking carnitine levels to BMIPP-WR is limited. Here we report the effect of L-carnitine administration on the BMIPP-WR in 9 chronic HD patients with TGCV in a retrospective cohort. The mean age at TGCV diagnosis was 59 years. Following standard doses of oral L-carnitine administration, plasma free carnitine levels significantly increased. However, BMIPP-WR was not changed. In normal condition, most BMIPP taken up were esterified/incorporated into TG pool, hydrolyzed by intracellular lipases, and then transported by carnitine shuttle to mitochondria. In TGCV, intracellular TG lipolysis is defective. During the intracellular metabolism of BMIPP, carnitine shuttling occurs downstream of TG lipolysis. Therefore, even when carnitine levels were increased, BMIPP-WR did not change in patients with TGCV who underwent chronic HD. A phase IIb/III clinical trial for TGCV, is underway (jRCT2051210177). Increased awareness of the disease concept of TGCV, along with its diagnostic principles and procedures using BMIPP scintigraphy, is warranted.
  • A Dual SPECT Study using 99mTc Pyrophosphate and 201Thallium
    Hiroki Suenaga, Kenji Fukushima, Shiro Ishii, Osamu Hasegawa, Yuuki Mu ...
    Article type: Original Article
    Article ID: 23-00009
    Published: 2023
    Advance online publication: December 15, 2023
    JOURNAL OPEN ACCESS FULL-TEXT HTML ADVANCE PUBLICATION
    Supplementary material
    Purpose: We aimed to clarify the clinical characteristics of global and regional myocardial perfusion in patients with transthyretin type of cardiac amyloidosis (ATTR) using dual single-photon emission computed tomography (SPECT) with 99mTc pyrophosphate (PYP) and 201TL (TL).
    Methods: Consecutive 178 (mean age 78 ± 12, male 79) patients known or suspect of ATTR who underwent PYP-TL dual SPECT were retrospectively enrolled. Patients were categorized according to the visual grading for planar PYP uptake using Perugini grading, and the patients with grade greater than or equal to 2 were analyzed. In planar analysis, the heart/ contralateral ratio (H/CL) for PYP, and heart/lung ratio (H/L) for TL were obtained to evaluate global myocardial uptake. In TL-SPECT polar map analysis, the heterogeneity of myocardial uptake was evaluated using segmental mean %uptake. Cardiac function and left ventricular function and end-diastolic ventricular mass (LVmass) were measured by echocardiography.
    Results: Among 178 patients, 39 patients showed PYP uptake with grade 2 or 3 and H/CL >1.3 (81 ± 5 ys, male 28). Of those, 4 patients showed significant perfusion defect in TL scan. Among 35 patients without perfusion defect, H/L showed a significant inverse correlation to H/CL, and LVmass (r=-0.3, p=0.02; r=-0.4, p=0.03. 95% confidence interval -0.4 to 0.2, and -0.7 to -0.04 for H/CL and LVmass). Polar map analysis demonstrated significantly lower mean %uptake for TL in septum compared to lateral (79.4 ± 8.4 vs. 84.3 ± 6.2, p=0.006 for TL in septum vs. lateral, respectively).
    Conclusion: In ATTR, TL uptake surrogated the reduction of global myocardial perfusion. A significant regional heterogeneity was observed with a notable reduction in septum despite the diffuse hypertrophy.
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