Prevalence, Severity, and Prognostic Value of Sleep Apnea Syndromes in Cardiac Amyloidosis.
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).



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.



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 χ(2): 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.



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.
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Diane
D
Bodez
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Aziz
A
Guellich
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Clinical Investigation Centre, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Mounira
M
Kharoubi
Physiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Ala
A
Covali-Noroc
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Claire-Marie
CM
Tissot
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Soulef
S
Guendouz
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Luc
L
Hittinger
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Jean-Luc
JL
Dubois-Randé
INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Physiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Jean-Pascal
JP
Lefaucheur
Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Neurology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Violaine
V
Planté-Bordeneuve
DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Physiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Serge
S
Adnot
DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Physiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Laurent
L
Boyer
Cardiology Department, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Mondor Amyloidosis Network, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|INSERM U955, UPEC, IMRB, GRC Amyloid Research Institute, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|DHU ATVB, AP-HP Henri Mondor Teaching Hospital, Créteil, France.|Clinical Investigation Centre, AP-HP Henri Mondor Teaching Hospital, Créteil, France.
Thibaud
T
Damy
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D009202:Cardiomyopathies / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology / Q000401:mortality; D006801:Humans; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D012891:Sleep Apnea Syndromes / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology
cardiac amyloidosis; heart failure; prognosis; sleep-disordered breathing