How to diagnose cardiac amyloidosis early: impact of ECG, tissue Doppler echocardiography, and myocardial biopsy.
OBJECTIVE
To detect cardiac amyloidosis (CA) earlier, it is inevitable to improve diagnostic strategies.


RESULTS
The impact of ECG, echocardiography including tissue Doppler imaging (TDI) and strain, and myocardial biopsies was evaluated in 30 patients (63% (n = 19) men, mean age 66 +/- 8 years, NYHA 3.0 +/- 0.5, 73% with prior myocardial decompensation), in whom we proved CA. Amyloid was confirmed by apple-green birefringence under polarised light, and the causing protein by immunohistochemical examinations. Genetic analyses excluded familial CA. All patients (AL-lambda (n = 22), AL-kappa (n = 3), senile amyloidosis (n = 5)) had echocardiographic signs of restrictive cardiomyopathy (RCM), typical TDI and strain parameters (E'septal; E' lateral < 8 cm/s; E/E' > 8; S' < or =9 cm/s; global longitudinal strain (GLS) -7.9 +/- 3.8%). Pericardial effusions were present in 63% of patients. ECGs were suspicious in many patients: 19 (63%) had low-voltage, 23 (77%) reduced R waves in V(1)-V(4), and 57% both. Abnormalities, retrospectively had been present for 0.5-4 years. Twenty (67%) patients died 232 +/- 268 [2-1020] days after CA was diagnosed, but 502 +/- 333 [30-1440] days after the first symptom.


CONCLUSIONS
Accurate ECG evaluations, careful echocardiographic search for RCM, reduced strain/strain rates, and general indications to myocardial biopsies with correct analyses are needed to diagnose CA.
Piper
Cornelia
C
Department of Cardiology, Heart and Diabetes Center North Rhine-Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany. cpiper@hdz-nrw.de
Butz
Thomas
T
Farr
Martin
M
Faber
Lothar
L
Oldenburg
Olaf
O
Horstkotte
Dieter
D
D000368:Aged; D000369:Aged, 80 and over; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology; D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology; D015331:Cohort Studies; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013997:Time Factors
D000682:Amyloid