Clinical InvestigationCongestive Heart FailureProspective evaluation of the morbidity and mortality of wild-type and V122I mutant transthyretin amyloid cardiomyopathy: The Transthyretin Amyloidosis Cardiac Study (TRACS)
Section snippets
Study patients
Patients (n = 29) with ATTR cardiac amyloidosis associated with the V122I mutation (ATTRm; n = 11) or wild-type TTR (ATTRwt; n = 18) were identified after referral to tertiary care outpatient cardiology/amyloid clinics at participating sites. Eligibility and exclusion criteria are described more specifically in the supplemental materials to this study (see online Appendix, Study Patients).
Patients were studied at baseline and then at 6-month intervals for up to 2 years.
Biochemical and functional analysis
Blood samples were
Patient characteristics
Baseline characteristics of patients with ATTRm and ATTRwt demonstrated a high degree of similarity between the 2 groups in terms of gender, comorbidities, and baseline ECG findings (Table I). As expected, all patients with ATTRm were African American. Patients with ATTRwt tended to be older upon enrollment (76 ± 6 years vs 71 ± 5 years, P = .06), at the time of diagnosis (75 ± 6 years vs 70 ± 6 years), and upon symptom onset (73 ± 5 years vs 70 ± 6 years). Diastolic blood pressure was
Discussion
To our knowledge, TRACS represents the largest prospective, longitudinal study specifically designed to assess the natural history and disease progression of TTR cardiac amyloidosis due to both wild-type and, specifically, the V122I mutation. The principal finding of this report is that ATTR cardiomyopathy was associated with significant morbidity and mortality over a relatively short follow-up period of 15 months despite suggestions that heart disease in patients with ATTR, regardless of
Acknowledgements
The authors wish to acknowledge the General Clinical Research Unit (NIH M01 RR00533) of Boston University. Editorial support was provided by Xian Cao, MD, PhD at Scientific Strategy Partners and was funded by Pfizer Inc.
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