JACC. Advances · 2025
Impact of Increasing Referral for 99mTc-DPD Scintigraphy on Prognosis Across the Phenotypic Spectrum of Restrictive Cardiomyopathy.
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Choy Chern Hsiang, Kwok Chun Shing, Wahid Yasmin, Liu Boyang, Turvey-Haigh Lauren, Parker Kenneth, Cullis James, Parekh Poonam, Hussain Khalid, Akhtar Akshanna, Bradlow William M, Zaphiriou Alex, Baig Shanat, Gillmore Julian D, Fontana Marianna, Pinney Jennifer, Steeds Richard P, Moody William E
Аннотация
BACKGROUND: Although bone scintigraphy has been widely accepted as integral to the non-biopsy diagnostic algorithm for transthyretin amyloid cardiomyopathy (ATTR-CM), its prognostic role remains uncertain.
OBJECTIVES: The authors aimed to characterize changes in the referral pattern for 99mTc-DPD scintigraphy and its influence on the clinical phenotype and prognosis of patients diagnosed with ATTR-CM, and among those individuals in whom ATTR-CM is excluded.
METHODS: Retrospective cohort study of all-comers referred for 99mTc-DPD scintigraphy to the Midlands Amyloidosis Service over 15 years.
RESULTS: Of 528 patients referred for 99mTc-DPD scintigraphy, 477 underwent echocardiography suggestive for ATTR-CM and were included in the study. A heightened demand for 99mTc-DPD scintigraphy was linked to increasing proportions of referrals from cardiologists over 5-year periods (47% vs 87% vs 96%, P < 0.001). Nearly half of patients (216/477, 45%) were diagnosed with ATTR-CM: 186 had ATTRwt-CM and 30 had ATTRv-CM; the commonest TTR variant was V142I (25/30, 83%). Compared to ATTR-CM, patients with nonamyloid CM (261/477, 55%) were younger and more often female yet with similar 5-year mortality rates (40% vs 31%, P = 0.044). In an age- and sex-adjusted Cox-proportional hazards model, there was no significant difference in survival between patients with ATTRwt-CM and nonamyloid CM (HR 0.99, 95% CI: 0.98-1.38, P = 0.96). There was also no difference in mortality according to Perugini grading.
CONCLUSIONS: There is strikingly high mortality among patients with negative 99mTc-DPD scintigraphy, equivalent to that of ATTR-CM. This finding serves as a call for future studies to better characterise this seemingly overlooked cohort, with the aim of developing targeted therapies and improving outcomes.
OBJECTIVES: The authors aimed to characterize changes in the referral pattern for 99mTc-DPD scintigraphy and its influence on the clinical phenotype and prognosis of patients diagnosed with ATTR-CM, and among those individuals in whom ATTR-CM is excluded.
METHODS: Retrospective cohort study of all-comers referred for 99mTc-DPD scintigraphy to the Midlands Amyloidosis Service over 15 years.
RESULTS: Of 528 patients referred for 99mTc-DPD scintigraphy, 477 underwent echocardiography suggestive for ATTR-CM and were included in the study. A heightened demand for 99mTc-DPD scintigraphy was linked to increasing proportions of referrals from cardiologists over 5-year periods (47% vs 87% vs 96%, P < 0.001). Nearly half of patients (216/477, 45%) were diagnosed with ATTR-CM: 186 had ATTRwt-CM and 30 had ATTRv-CM; the commonest TTR variant was V142I (25/30, 83%). Compared to ATTR-CM, patients with nonamyloid CM (261/477, 55%) were younger and more often female yet with similar 5-year mortality rates (40% vs 31%, P = 0.044). In an age- and sex-adjusted Cox-proportional hazards model, there was no significant difference in survival between patients with ATTRwt-CM and nonamyloid CM (HR 0.99, 95% CI: 0.98-1.38, P = 0.96). There was also no difference in mortality according to Perugini grading.
CONCLUSIONS: There is strikingly high mortality among patients with negative 99mTc-DPD scintigraphy, equivalent to that of ATTR-CM. This finding serves as a call for future studies to better characterise this seemingly overlooked cohort, with the aim of developing targeted therapies and improving outcomes.