Abstract
BackgroundCardiac 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 resultsWe 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).ConclusionOur 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.Graphic abstract
Highlights
Cardiac amyloidosis is an infiltrative process of the extracellular matrix that increases myocardial wall thickness in the absence of actual cardiomyocyte hypertrophy [1]
In those in whom the diagnosis was reached by non-cardiac biopsy, definition of cardiac involvement was based on echocardiography and/or cardiac magnetic resonance (CMR) plus elevations of biomarkers
Our study shows that clinical presentation and disease progression differed between AL and ATTRwt, CV death was the predominant mode of death in both subtypes
Summary
Cardiac amyloidosis is an infiltrative process of the extracellular matrix that increases myocardial wall thickness in the absence of actual cardiomyocyte hypertrophy [1]. Acquired monoclonal immunoglobulin light-chain amyloidosis (AL), the hereditary, transthyretin (TTR)-related form (ATTRm), and wild-type (non-mutant) TTR-related amyloidosis (ATTRwt) systemic “senile” amyloidosis account for more than 90% of all cardiac amyloidosis (CA) [2]. While frequency of cardiac amyloidosis (CA) in ATTRm is variable and depends on the specific mutation [12], ATTRwt almost exclusively affects the heart. CA is typically associated with heart failure and dictates the clinical course of the disease. Disease profiles and clinical courses differ between AL and ATTR [2, 13]. 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. 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 twothirds of fatal casualties in both groups
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