[Primary cardiac amyloidosis -- condition which can be diagnosed by a cardiologist].
Primary amyloidosis is a systemic disorder caused by the clonal production and tissue deposition of immunoglobulin light chain proteins. The disease symptoms are typical of multisystem failure. Common presenting features include nephrotic syndrome, hepatomegaly, sensomotor peripheral neuropathy and, in the case of cardiac involvement, congestive heart failure. This last sign appears very seldom as alone, without any others. Cardiac involvement generally denotes a poor prognosis, regardless of the method of treatment. The median survival rate from onset of congestive heart failure is 6 months. Only the patients with earliest diagnosis made and advanced treatment (chemotherapy, autologous stem-cell transplantation, heart transplantation) introduced have the chance of the lengthening of life. The authors present a case of 52-year-old man with a primary amyloidosis, who suffered from severe, not responding to treatment, congestive heart failure. Because of lack of the other organ involvement symptoms, the correct diagnosis was made very late. The authors place emphasis on a simple diagnostic tool such as the correlation between the low voltage in the limb ECG leads and the echocardiographic sings of left ventricular hypertrophy. The combination of specific ECG, echocardiographic findings and positive extracardiac tissue biopsy may be sufficient to reach correct diagnosis. These examinations are easy accessible in non-specialist hospitals.
Zielińska
Marzenna
M
Klinika Kardiologii, I Katedra Kardiologii i Kardiochirurgii, Uniwersytet Medyczny, ul. Sterlinga 1/3, 91-425 Łódź. mzielinska@wshe.lodz.pl
Koniarek
Włodzimierz
W
Kaczmarek
Krzysztof
K
Maciejewski
Marek
M
Wagrowska-Danilewicz
Małgorzata
M
Goch
Jan H
JH
D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis*; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D004452:Echocardiography; D004562:Electrocardiography; D017809:Fatal Outcome; D006333:Heart Failure / Q000209:etiology; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D008875:Middle Aged