Frequency of and Prognostic Significance of Cardiac Involvement at Presentation in Hereditary Transthyretin-Derived Amyloidosis and the Value of N-Terminal Pro-B-Type Natriuretic Peptide
Key result
Baseline cardiac involvement in ATTRm amyloidosis linked to ~6-fold higher mortality.
- HR 5.95
- 95% CI 2.12-16.7
- n=77
Why the study?
The prevalence of cardiac involvement at diagnosis in hereditary transthyretin-derived amyloidosis and the diagnostic value of NT-proBNP were not well established.
Population
77 consecutive patients with hereditary transthyretin-derived amyloidosis
Comparison
Patients with and without cardiac involvement at baseline
Design
Prospective cohort study at a national amyloidosis center
Authors
Discussion
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Member takes
Implication
Cardiac involvement at ATTRm diagnosis signals higher mortality; leaves open whether early NT-proBNP screening alters management in observational cohorts.
Study Design
Type
Cohort (n=77)
Multicenter
No
Structured PICO
P
Population
77 consecutive patients with hereditary transthyretin-derived (ATTRm) amyloidosis followed prospectively.
O
Outcome
Overall mortality and diagnostic sensitivity of NT-proBNP for cardiac involvementhard clinical
Main Result
Hazard Ratio: 5.95 (95% CI 2.12–16.7)
Cardiac involvement is present in half of patients with ATTRm amyloidosis at diagnosis and strongly predicts mortality, with NT-proBNP serving as a highly sensitive screening marker.
Cite This Study
Klaassen et al. (2017) conducted a cohort in Hereditary transthyretin-derived (ATTRm) amyloidosis (n=77). Cardiac involvement at baseline vs. No cardiac involvement at baseline was evaluated on Overall mortality (HR 5.95, 95% CI 2.12-16.7). Cardiac involvement at baseline in patients with ATTRm amyloidosis was associated with significantly increased overall mortality (HR 5.95; 95% CI 2.12-16.7).