title: [Echocardiographic assessment of diagnosis and prognosis of biopsy-proven amyloid cardiomyopathy].
abstract: Amyloid cardiomyopathy is myocardial infiltrative disorder which mostly has been seen as the consequence of systemic amiloidosis. The diffuse global myocardial infiltration of nonfunctional amyloid displaces the contractile myocites giving rice to relaxation abnormality and diastolic dysfunction of restrictive or congestive type of both ventricles, but more frequently with right-sided congestion, while systolic left ventricular function deteriorates late in disease process. We report a patient with amyloid cardiomyopathy and nephrotic syndrome underlying primary amiloidosis. Our aim is to point out at echocardiographic assessment of diagnosis and prognosis of amyloid cardiomyopathy, which is proven by postmortal endomyocardial biopsy. The hallmark of echocardiographic diagnostics are the findings of the thickened ventricular and septal walls, small ventricular cavities, dilated atria with thickened interatrial septum and atrioventricular valves, and granular-sparkling and hyperrefractile myocardium. Doppler assessment diagnostically gives us the insight in restrictive physiology of both ventricles, and the inverse relation of the left ventricular thickness and voltage on the ECG is high specific. Echocardiographic evaluation of mean left ventricular thickness in amyloid cardiomyopathy is very important prognostic parameter. so that if it is > or =15 mm, median survival is 0.4 years, whereas in our patient with median thickness of 2.76 cm the survival was only three months. The advanced diastolic dysfunction of the left ventricle with an increased transmitral E/A ratio and deceleration time of < or =150 ms is strong predictor of cardiac death. In this case of restrictive transmitral flow E/A was 1.7 and DT 100 ms and they were ominous prognostic signs of survival.
authors.affiliation: Zavod Za Kardiovaskularne Bolesti, Klinika Za Unutarnje Bolesti Klinicka Bolnica Dubrava, Zagreb, Hrvatska. ISOKOL@KBD.HR
authors.forename: Ivan
authors.initials: I
authors.lastname: Sokol
authors.forename: Josip
authors.initials: J
authors.lastname: Vincelj
authors.forename: Muhamed
authors.initials: M
authors.lastname: Sarić
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging*; D001706:Biopsy; D009202:Cardiomyopathies / Q000150:complications / Q000000981:diagnostic imaging*; D015150:Echocardiography, Doppler; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D009404:Nephrotic Syndrome / Q000150:complications; D011379:Prognosis