What is the clinical evidence from this study?

Study design: Cohort. Population: Cardiac amyloidosis (AL and ATTRwt) (n=114). Intervention: AL amyloidosis vs. ATTRwt amyloidosis. Primary outcome: All-cause mortality (HR 2.28, 95% CI 1.27-4.10, p=0.006).

May 27, 2019Open Access

When and how do patients with cardiac amyloidosis die?

Key Result

Patients with AL cardiac amyloidosis had a significantly higher risk of all-cause mortality compared to those with ATTRwt (HR 2.28), though cardiovascular events were the predominant mode of death in both groups.

Study Design

Type

Cohort (n=114)

Multicenter

Yes

Structured PICO

Population

114 patients with confirmed AL or wild-type ATTR cardiac amyloidosis, followed for a median of 21.1 months to evaluate disease progression and mode of death.

Outcome

All-cause mortality and mode of death (cardiovascular vs. non-cardiovascular)hard clinical

Despite significant differences in clinical presentation and overall survival between AL and ATTRwt cardiac amyloidosis, cardiovascular events account for more than two-thirds of deaths in both subtypes.

Main Result

Hazard Ratio: 2.28 (95% CI 1.27–4.1)

Absolute Event Rate: 65% vs 44%

p-value: p=0.006

Limitations

Retrospective study design
Exact cause of death could not be retrieved in a substantial number of patients
Mode of death in ten patients was classified based on death records or information from relatives without autopsy
No information available on non-deadly strokes, heart failure admissions, and pacemaker dependency

Abstract

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. 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). 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.

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