Details

Autor(en) / Beteiligte

• Prasad, Mark R.
• Chandrashekar, Pranav
• Al-Rashdan, Lana
• Burton, Yunwoo
• Zhao, Yuanzi
• Coleman, Abby
• Silbermann, Rebecca
• Medvedova, Eva
• Masri, Ahmad

Titel

Survival Of Patients With Monoclonal Gammopathy Of Undetermined Significance And Transthyretin Amyloid Cardiomyopathy

Ist Teil von

• Journal of cardiac failure, 2024-01, Vol.30 (1), p.132-133

Ort / Verlag

Elsevier Inc

Erscheinungsjahr

2024

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• Monoclonal gammopathy of undetermined significance (MGUS) is a precursor plasma cell dyscrasia that has been reported to be present at higher rates in individuals with transthyretin amyloid cardiomyopathy (ATTR-CM) when compared to age-matched individuals in the general population. Whether MGUS in ATTR-CM is associated with worse outcomes is unknown. Baseline clinical characteristics and outcomes were compared between ATTR-CM (n=116), ATTR-CM+MGUS (n=27), and light chain amyloid cardiomyopathy (AL-CM) patients (n=122) in a single-center cohort. The primary outcome was all-cause mortality. Median age was 75, 74, and 65 years in the ATTR-CM only, ATTR-CM+MGUS, and AL-CM cohorts respectively (p<0.001). The ATTR-CM only and ATTR-CM+MGUS cohorts had higher proportions of male subjects when compared to the AL-CM cohort (86%, 100%, and 57% respectively; p<0.001). Patients across all three cohorts had similar baseline renal function (serum creatinine: p=0.813; eGFR: p=0.161) and cardiac biomarkers (NT-proBNP: p=0.793; Troponin I: p=0.078). On echocardiography, left ventricular size (diastolic diameter: p=0.02; systolic diameter: p<0.001) and thickness (septum thickness: p<0.001; posterior wall thickness: p<0.001) were greater in patients with ATTR-CM (both with and without MGUS) than in those with AL-CM. Over a median follow up of 3.73 years (2.18, 5.78), 28 (24.1%) patients with ATTR-CM only, 5 (18.5%) patients with ATTR-CM+MGUS, and 47 (38.5%) patients with AL-CM cohort died. There was no significant difference in survival at 3 (p=0.099) and 5 years (p=0.324) between all three groups. In the ATTR-CM patients, after adjusting for age, sex, NAC (National Amyloidosis Centre) stage, and tafamidis treatment (time-dependent covariate), the presence of MGUS in patients with ATTR-CM was not associated with increased mortality at 3 (HR 0.428; 95% CI 0.054, 3.374; p=0.42) or 5 years (HR 0.81; 95% CI 0.29, 2.262; p=0.458). The presence of MGUS in patients with ATTR-CM is not associated with worse outcomes over a median follow up of 3.73 years. Larger studies are needed to confirm our findings which have significant implications with the aging population and the increasing number of patients living with ATTR-CM.