Revisiting morphological features of cardiac amyloid with cardiac magnetic resonance

Pozo, Eduardo; Castellano, Jose M; Deochand, Rajiv; Kanwar, Anubhav; Pazos, Pablo; García‐Lunar, Inés; Cham, Matthew D.; Jacobi, Adam; Narula, Jagat; Fuster, Valentín; Sanz, Javier

doi:10.1186/1532-429x-15-s1-p154

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“…Given a significant majority of our AL amylodosis heart failure patients were Mayo cardiac stage III, the RWT provides an important tool to further aid clinicians in determining prognosis, as patients with RWT above and below the median have significantly different mortality rates. Therefore, increased RWT in AL cardiac amyloidosis with evidence of symptoms of heart failure, in addition to being a marker to discriminate cardiac amyloid hearts from those without amyloid fibril deposition 23 ; RWT is useful in identifying “at risk” subjects and provides a simple non-invasive assessment of amyloid fibril burden on LV structure derived from routine echocardiographic measurements, thus conferring its advantage over more novel echocardiographic parameters such as left ventricular strain.…”

Section: Discussionmentioning

confidence: 99%

Predictors of Mortality in Light Chain Cardiac Amyloidosis with Heart Failure

Tahir

Doros

Kim

et al. 2019

Sci Rep

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Cardiac involvement in systemic amyloidosis (AL) occurs in ~50% of all AL patients. However once symptomatic heart failure develops, therapeutic options are limited thereby conferring a poor overall prognosis. The median survival is <6 months when AL patients are untreated for the underlying plasma cell dyscrasia. We thus sought to identify risk factors of increased mortality in treatment - naïve , AL cardiac amyloidosis with heart failure. Patients with biopsy-proven AL cardiac amyloid, who presented with heart failure and did not received prior AL treatment, were enrolled between 2004–2014, at the initial visit to the Amyloidosis Center at Boston University Medical Center. Routine laboratory tests, physical examination and echocardiography data were collected. There were 165 predominantly white (76.4%), and male (61%) patients, with a mean age of 61.6 ± 9.5 years. Median survival was 10.9 months (95% CI 6.2–14.7). By multivariate analysis increased relative wall thickness (RWT) [HR 6.70; 95% CI 2.45–18.30), older age (HR 1.04; 95% CI 1.01–1.06), higher New York Heart Association (NYHA) functional class (HR 1.50; 95% CI 1.02–2.2), log brain natriuretic peptide (BNP) levels (HR 1.45; 95% CI 1.15–1.81) and C-reactive protein (CRP) levels (HR 1.02; 95% CI 1.00–1.04) were significant predictors for increased mortality. In conclusion, in treatment-naïve, AL cardiac amyloidosis patients with heart failure symptoms who lack these high-risk features may have a better outcome. These findings might allow for better risk stratification although outcomes are still poor.

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Section: Discussionmentioning

confidence: 99%