Circadian rhythm of blood pressure reflects the severity of cardiac impairment in familial amyloid polyneuropathy.
BACKGROUND
Cardiac amyloidosis due to familial amyloid polyneuropathy (FAP) includes restrictive cardiomyopathy, thickened cardiac walls, conduction disorders and cardiac denervation. Impaired blood pressure variability has been documented in FAP related to the Val30Met mutation.


OBJECTIVE
To document blood pressure variability in FAP patients with various mutation types and its relationship to the severity of cardiac involvement.


METHODS
Blood pressure variability was analysed in 49 consecutive FAP patients and was compared with a matched control population. Cardiac evaluation included echocardiography, right heart catheterization, electrophysiological study, Holter electrocardiogram and metaiodobenzylguanidine (MIBG) scintigraphy.


RESULTS
A non-dipping pattern was found in 80% of FAP patients and in 35% of control patients (P<0.0001); this was due to a significantly lower diurnal blood pressure in FAP patients (FAP group, 113 ± 21 mmHg; control group, 124 ± 8 mmHg; P<0.0001), whereas nocturnal blood pressures were similar. Among FAP patients, a non-dipping pattern was significantly associated with haemodynamic involvement, cardiac thickening or conduction disorders. These associations did not depend on the average blood pressure levels. Impaired blood pressure variability was more frequent and more pronounced in patients with multiple criteria for severe cardiac amyloidosis.


CONCLUSIONS
Low blood pressure variability is common in cardiac amyloidosis due to FAP. A non-dipping pattern was more frequently observed in FAP patients with haemodynamic impairment, cardiac thickening or conduction disorders. It is suggested that impairment of circadian rhythm of blood pressure reflects the severity of cardiac amyloidosis due to FAP.
Cardiology Department, CHU Antoine-Béclère, Université Paris Sud, AP-HP, Clamart, France. vincent.algalarrondo@abc.aphp.fr
Vincent
V
Algalarrondo
Ludivine
L
Eliahou
Isabelle
I
Thierry
Abdeslam
A
Bouzeman
Madalina
M
Dasoveanu
Claude
C
Sebag
Ghassan
G
Moubarak
Dominique
D
Le Guludec
Didier
D
Samuel
David
D
Adams
Sylvie
S
Dinanian
Michel S
MS
Slama
D019797:3-Iodobenzylguanidine; D000328:Adult; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D001794:Blood Pressure; D006328:Cardiac Catheterization; D009202:Cardiomyopathies / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D002940:Circadian Rhythm; D003430:Cross-Sectional Studies; D004452:Echocardiography; D015716:Electrocardiography, Ambulatory; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D005602:France; D006801:Humans; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011228:Prealbumin / Q000235:genetics; D011237:Predictive Value of Tests; D019275:Radiopharmaceuticals; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors
D011228:Prealbumin; D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine