ArticleJACC. Advances2026
Development of a Predictive Tool in Patients With High Pretest Probability for Transthyretin Amyloid Cardiomyopathy.
Article in JACC. Advances, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundTransthyretin amyloid cardiomyopathy (ATTR-CM) is an underdiagnosed cause of heart failure, arrhythmias, and valvular disease.
objectivesThe objectives of the study were to develop: 1) screening criteria to identify high-risk patients for ATTR-CM; and 2) a predictive tool for ATTR-CM diagnosis.
methodsThis prospective observational registry (2019-2022) at 2 Vancouver academic sites screened patients aged >60 years presenting to heart failure, atrial fibrillation, transcatheter valve clinics, and cardiologists' offices. Patients meeting high-risk criteria who underwent technetium-99m-pyrophosphate were included. Predictors of ATTR-CM were identified using univariable and multivariable logistic regression.
resultsOf 2,500 patients screened, 200 were enrolled (mean age 78.4 ± 8.3 years; 64.5% male) with a mean follow-up of 3 years. ATTR-CM was diagnosed in 46 (23.0%), and 7 (3.5%) had immunoglobulin light-chain amyloidosis amyloidosis. Compared to non-ATTR-CM, those with ATTR-CM were older (82.6 ± 7.1 years vs 77.2 ± 8.1 years; P < 0.001), male (80.4% vs 59.7%, P = 0.013), and had higher NT-proBNP (3,633 vs 2018; P = 0.027). ATTR-CM patients had greater posterior wall thickness (14.5 ± 3.2 vs 11.2 ± 1.8; P < 0.001), low QRS voltages (37.0% vs 3.9%; P < 0.001), more atrioventricular block (38.5% vs 19.2%; P = 0.018), and higher Doppler E/e' (18.7 ± 7.6 vs 14.4 ± 6.2, P = 0.001). A 5-item predictive tool (score ≥7) identified patients who had positive ATTR-CM diagnoses (sensitivity 89.1% [95% CI: 80.1%-98.1%], specificity 85.1% [95% CI: 70.4%-90.7%], area under receiver operating characteristic curve 0.931 [95% CI: 0.884-0.978]).
conclusionsBroad screening criteria of high-risk populations referred for technetium-99m-pyrophosphate yielded new ATTR-CM diagnoses in 23% of patients. Our predictive tool may guide clinicians in refining pretest probability and optimizing diagnosis for ATTR-CM. External validation in broader populations is warranted.
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