Dataset Information

Multimodality assessments of wild-type transthyretin cardiac amyloidosis with no ventricular hypertrophy.

ABSTRACT: While patients with transthyretin cardiac amyloidosis (ATTR-CA) typically present with concentric or asymmetric hypertrophy, a small percentage of ATTR-CA is known to present with 'atypical' cardiac morphologies such as eccentric hypertrophy or even no hypertrophy. However, detailed report of multimodality assessments of ATTR-CA with no ventricular hypertrophy is lacking. Herein, we report detailed multimodality assessments of an 81-year-old Japanese woman with heart failure and history of carpal tunnel syndrome and lumbar canal stenosis, presenting no ventricular hypertrophy and negative ^99m technetium-pyrophosphate scintigraphy, who was eventually diagnosed as having wild-type ATTR-CA. Our case highlights the role of multimodality assessments for early diagnosis of ATTR-CA in patients with atypical cardiac morphologies and also emphasizes the limitations of bone scintigraphy and the importance of considering ATTR-CA in patients with non-cardiac manifestations of ATTR amyloidosis.

SUBMITTER: Iwaya T

PROVIDER: S-EPMC10053176 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature

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Multimodality assessments of wild-type transthyretin cardiac amyloidosis with no ventricular hypertrophy.

Iwaya Takuma T Okada Atsushi A Tateishi Emi E Ohta Yasutoshi Y Morita Yoshiaki Y Ohta-Ogo Keiko K Izumi Chisato C

ESC heart failure 20221106 2

While patients with transthyretin cardiac amyloidosis (ATTR-CA) typically present with concentric or asymmetric hypertrophy, a small percentage of ATTR-CA is known to present with 'atypical' cardiac morphologies such as eccentric hypertrophy or even no hypertrophy. However, detailed report of multimodality assessments of ATTR-CA with no ventricular hypertrophy is lacking. Herein, we report detailed multimodality assessments of an 81-year-old Japanese woman with heart failure and history of carpa ...[more]

PMID: 36335980

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Project description:Background and aimsIn real-world, wild-type transthyretin cardiomyopathy is increasingly diagnosed in patients ≥ 80 years old (octogenarians), although being underrepresented in randomized clinical trials. Specific data on natural course and outcome under tafamidis treatment in octogenarians are therefore scarce. The impact of tafamidis treatment on mortality in real-world wild-type transthyretin cardiomyopathy octogenarians was studied.MethodsAn international, multicentre cohort study of 710 consecutive wild-type transthyretin cardiomyopathy patients with mean follow-up of 2.2 ± 1.8 years for all-cause mortality endpoint was performed.ResultsThe cohort consisted of 58.5% (415/710) octogenarians (85 ± 4 years, 74.2% male). Before tafamidis availability, natural course in octogenarians (148/257) vs. non-octogenarians (109/257) was poor, with 16% 1-year and 71% 5-year mortality vs. 8% and 47%, respectively (P < .001). Since tafamidis availability, 70.1% (253/361) octogenarians were initiated on tafamidis vs. 83.7% (231/276) non-octogenarians (P < .001). Tafamidis discontinuation was similar (octogenarians 10.3% and non-octogenarians 7.4%; P = .260). Overall tafamidis treated vs. untreated octogenarians had better unadjusted survival (P < .001), with 5% 1-year and 24% 3-year mortality. Tafamidis treatment associated with lower mortality after propensity score matching on baseline variables, including age, National Amyloidosis Centre stage, and New York Heart Association class in on average 394 subjects [hazard ratio (HR) = 0.53, 95% confidence interval (CI) 0.34-0.84, P = .007], also in octogenarians (HR = 0.57, 95% CI 0.33-1.01, P = .053). Neither age at diagnosis (P = .217) nor at treatment initiation (P = .154) interacted with tafamidis mortality benefit. Octogenarians had poorer survival despite tafamidis, when initiated at ≥90 years (HR = 3.3, 95% CI 1.10-9.53, P = .033) and National Amyloidosis Centre Stage ≥3 (HR = 2.4, 95% CI 0.87-6.46, P = .090).ConclusionsReal-world tafamidis treatment improves survival without age affecting treatment efficacy, although mortality remains considerable in octogenarians.

Dataset Information

Multimodality assessments of wild-type transthyretin cardiac amyloidosis with no ventricular hypertrophy.

Publications

Multimodality assessments of wild-type transthyretin cardiac amyloidosis with no ventricular hypertrophy.

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