Abstract

Study Objectives:

To assess prevalence, severity, and prognostic value of sleep-disordered breathing (SDB), in the three main cardiac amyloidosis (CA) types, i.e., light-chain (AL), transthyretin-related familial (m-TTR), or senile (WT-TTR).

Methods:

Patients consecutively referred for CA diagnosis work-up underwent cardiac assessment and nocturnal polygraphy. SDB was defined as apnea-hypopnea index (AHI) ≥ 5/h. Multivariate analysis was used to identify predictors of a major adverse cardiac event (MACE) defined as death, heart transplantation and acute heart failure.

Results:

Seventy CA patients were included (31 AL, 22 m-TTR, 17 WT-TTR). The mean ± standard deviation age and left ventricular ejection fraction were 71 ± 12 years and 49% ± 13% and median (interquartile range) N terminal pro brain natriuretic peptide (NT-proBNP) was 3,932 (1,607; 7,028) pg/mL. The prevalence of SDB was 90% without difference between amyloidosis types. SDB was central in 27% and obstructive in 73%. AL had less frequent severe SDB compared to m-TTR and WT-TTR (P = 0.015) but longer time with peripheral capillary oxygen saturation (SpO2)<90% (P = 0.037). After a median follow-up of 7.5 (2.8; 14.9) months, 49% patients experienced MACE. Time with nocturnal SpO2<90% was the only independent predictor of MACE. The best-identified threshold was 30 min. Values>30 min were associated with bad prognosis (Log-rank χ2: 8.01, P value = 0.005). Using binomial logistic regression, determinants of time with nocturnal SpO2<90% were New York Heart Association class (P = 0.011), and log-NT-proBNP (P = 0.04) but not AHI.

Conclusions:

In CA population, prevalence of SDB is high (90%) and dominated by the obstructive pattern. Bad prognosis in this population was driven by nocturnal desaturation, reflecting heart failure severity and respiratory involvement.

Significance

Sleep-disordered breathing is frequent and associated with poor prognosis in patients with heart failure. Cardiac amyloidosis is a severe condition, its entire understanding including co-morbidities that have additional prognostic impact, is of crucial importance. This is the first study to focus on sleep consequences of cardiac amyloidosis, highlighting that SDB is highly prevalent in these patients. The attention of physicians should be drawn to the need of SDB screening in this population. Moreover, this study provides a first potential therapeutic target to consider in this particular population that is nocturnal desaturation. Further studies are needed to establish the prognostic value in each type of cardiac amyloidosis, as to evaluate the effects of SDB treatment on outcomes.

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