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. 2020 Jan;109(1):78-88.
doi: 10.1007/s00392-019-01490-2. Epub 2019 May 27.

When and how do patients with cardiac amyloidosis die?

F Escher  1   2 , M Senoner  1 , J Doerler  1 , M M Zaruba  1 , M Messner  1 , C Mussner-Seeber  1 , M Ebert  1 , C Ensinger  3 , A Mair  4 , A Kroiss  5 , H Ulmer  6 , S Schneiderbauer-Porod  7 , C Ebner  6 , G Poelzl  8
Affiliations

When and how do patients with cardiac amyloidosis die?

F Escher et al. Clin Res Cardiol. 2020 Jan.

Abstract

Background: Cardiac amyloidosis (CA) is an underappreciated cause of morbidity and mortality. Light-chain (AL) and transthyretin (ATTR) amyloidosis have different disease trajectories. No data are available on subtype-specific modes of death (MOD) in patients with CA.

Methods and results: We retrospectively investigated 66 with AL and 48 with wild-type ATTR amyloidosis (ATTRwt) from 2000 to 2018. ATTRwt differed from AL by age (74.6 ± 5.4 years vs. 63 ± 10.8 years), posterior wall thickness (16.8 ± 3.3 mm vs. 14.3 ± 2.2 mm), left ventricular mass index (180.7 ± 63.2 g/m2 vs. 133.5 ± 42.2 g/m2), and the proportions of male gender (91.7% vs. 59.1%), atrial enlargement (92% vs. 68.2%) and atrial fibrillation (50% vs. 12.1%). In AL NYHA Functional Class and proteinuria (72.7% vs. 39.6%) were greater; mean arterial pressure (84.4 ± 13.5 mmHg vs. 90.0 ± 11.3 mmHg) was lower. Unadjusted 5-year mortality rate was 65% in AL-CA vs. 44% in the ATTRwt group. Individuals with AL-CA were 2.28 times ([95%CI 1.27-4.10]; p = 0.006) more likely to die than were individuals with ATTRwt-CA. Information on MOD was available in 56 (94.9%) of 59 deceased patients. MOD was cardiovascular in 40 (66.8%) and non-cardiovascular in 16 (27.1%) patients. Cardiovascular [28 (68.3%) vs. 13 (80%)] death events were distributed equally between AL and ATTRwt (p = 0.51).

Conclusion: Our data indicate no differences in MOD between patients with AL and ATTRwt cardiac amyloidosis despite significant differences in clinical presentation and disease progression. Cardiovascular events account for more than two-thirds of fatal casualties in both groups.

Keywords: Cardiac amyloidosis; Light chain (AL) amyloidosis; Mode of death; Prognosis; Transthyretin (ATTR) amyloidosis.

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Conflict of interest statement

Poelzl G has received speaker honoraria from Pfizer and AKCEA Therapeutics.

Figures

Fig. 1
Fig. 1
Assembly of the cohort, participant flow, and mode of death in deceased patients. CA cardiac amyloidosis, HTx heart transplantation, LVAD left ventricular assist device, MOD mode of death
Fig. 2
Fig. 2
Correlation between cardiac AL and TTR amyloidosis and mortality Cumulative 5-year event rates estimated by univariate Cox proportional hazard regression analysis in 117 patients with cardiac amyloidosis according to subtype are presented. Numbers of patients at risk and event rates are shown below the graphs
Fig. 3
Fig. 3
a, b Mode of death stratified by amyloid subtype. All deaths are divided into cardiovascular (CV), non-CV, and unknown deaths; cardiovascular deaths are further subdivided into sudden cardiac death (SCD), cardiogenic shock, acute heart failure, cerebral haemorrhage, and multi-organ failure (MOF), while non-CV deaths are subdivided into sepsis/pneumonia, uraemia, GI bleeding, anaemia, accident, and cachexia
See this image and copyright information in PMC

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