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Transthyretin (TTR) amyloidosis is a fatal disease characterized by progressive polyneuropathy and cardiomyopathy. Here we describe the relationships between patient clinical characteristics and several routinely measured cardiac biomarkers from the Transthyretin Amyloidosis Survey (THAOS) patient registry.
Symptomatic patients were categorized into one of three phenotypes: (a) Cardiac: patients with heart failure, dyspnea or cardiac rhythm disturbance, with minimal or absent neurologic or gastrointestinal symptoms; (b) Neurologic: those with neurologic symptoms, including walking disability, or gastrointestinal symptoms of any severity, who do not meet the cardiac phenotype criteria; (c) Mixed: those not meeting either cardiac or neurologic phenotype criteria. LV wall thickness, pacemaker use, and biomarker data (brain natriuretic peptide [BNP], N-terminal pro-BNP [NTproBNP], troponin I) were evaluated according to phenotype.
The cardiac data of 146 cardiac, 421 neurologic and 168 mixed phenotype patients were analyzed. A higher proportion of patients of the cardiac phenotype had a left ventricular (LV) septum wall thickness > 12 mm (93.2%) compared with neurologic (29.5%) and mixed (80.5%) phenotype groups. Pacemaker use was highest among patients of mixed phenotype (mixed, 44.1%; cardiac 27.0%; neurologic 13.3%). The cardiac and mixed phenotype groups had elevated median BNP values compared with those of neurologic phenotype patients (BNP: cardiac, 542.0 [43.0-11999.0] pg/mL; mixed, 500.9 [35.5-32434.0] pg/mL; neurologic, 93.0 [35.0-2790.0] pg/mL). NT-proBNP demonstrated similar trends (cardiac, 4276.5 [180.02-213350.8] pg/mL; mixed, 3144.0 [125.0-296450.0] pg/mL; neurologic, 387.0 [135.0-14047.0] pg/ml), as did troponin I values (cardiac, 0.10 [0.02, 0.73]; mixed 0.08 [0.03, 0.86]; neurologic 0.03 [0.01, 0.21]).
Many patients with TTR amyloidosis demonstrate abnormal biomarkers, suggestive of cardiac involvement, while some cardiac data show the potential to distinguish between phenotypes. Data for this abstract are derived from the THAOS registry, which is sponsored by Pfizer Inc.
Poster Sessions, Expo North
Saturday, March 09, 2013, 3:45 p.m.-4:30 p.m.
Session Title: What Is Unfolding in Cardiac Amyloidosis Research?
Abstract Category: 23. Pericardial/Myocardial Disease
Presentation Number: 1163-152
- 2013 American College of Cardiology Foundation