Details

Autor(en) / Beteiligte

• CHAN, NICHOLAS
• TERUYA, SERGIO
• MIRABAL, ALFONSINA
• WEINSAFT, ARIEL Y.
• Santos, JEFFENY DE LOS
• GUADALUPE, SAMANTHA
• JIMENEZ, MASSIEL
• RODRIGUEZ, CARLOS
• HELMKE, STEPHEN
• CUOMO, MARGARET
• SMILEY, DIA
• MAURER, MATHEW S.

Titel

Temporal Outcomes of Patients Diagnosed With Transthyretin Cardiac Amyloidosis

Ist Teil von

• Journal of cardiac failure, 2024-03

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2024

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• Transthyretin cardiac amyloidosis (ATTR-CA) is increasingly recognized. Clinical outcomes have evolved over time amid changes in the diagnostic pathway and advances in therapeutics. We sought to evaluate clinical outcomes over time of patients with ATTR-CA with access to disease-modifying therapy. This is a retrospective cohort study of 419 patients diagnosed with ATTR-CA during 2001–2021, comparing clinical characteristics across eras. The primary end point was composite all-cause mortality or orthotopic heart transplantation (OHT). Time-to-event analysis was performed using Cox proportional hazard modeling controlling for differences among cohorts. Patients diagnosed in the more recent years had higher median age (2017–2021, 78 years; 2014–2016, 75 years; 2001–2013, 74 years) and more often had wild-type ATTR (81.9% vs 82.5% vs 56.4%), but less severe phenotypes as evidenced by more individuals with Columbia stage I disease (47.6% vs 35.9% vs 22.4%), owing to lower biomarkers, more patients in New York Heart Association functional classes I and II (68.9% vs 47.6% vs 43.6%), and lower use of loop diuretics (67.0% vs 78.6% vs 89.1%). Over time, patients were treated more frequently with tafamidis (74% vs 37% vs 32%). On multivariable analysis, greater Columbia score (hazard ratio 1.42, 95% confidence interval 1.30–1.54, P < .001) was predictive of death or OHT, whereas tafamidis (hazard ratio 0.31, 95% confidence interval 0.22–0.44, P < .001) was associated with greater survival and freedom from OHT. Patients recently diagnosed with ATTR-CA have earlier stage disease and substantially lower mortality. Tafamidis is associated with significantly improved survival and freedom from OHT. Central Illustration. Temporal trends across disease eras show greater adoption of technetium pyrophosphate scan, lower prevalence of advanced stage disease, and increased prescription of tafamidis over time. A lower Columbia score, indicative of early-stage disease, and tafamidis were associated with a lower risk of the combined outcome of time to death or orthotopic heart transplantation. [Display omitted]