Vnitr Lek 2008, 54(10):1010-1013

The isolated form of cardiac amyloidosis in the form of beginning infiltrative cardiomyopathy without restrictive physiology

P. Kuchynka¹, T. Paleček^1,*, S. Šimek¹, J. C. Lubanda¹, M. Elleder², I. Špička³, P. Jansa¹, A. Linhart¹

¹ II. interní klinika - klinika kardiologie a angiologie 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Aleš Linhart, DrSc.

² Ústav dědičných a metabolických poruch 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Milan Elleder, DrSc.

³ I. interní klinika - klinika hematologie 1. lékařské fakulty UK a VFN Praha, přednosta doc. MUDr. Marek Trněný, DrSc.

The authors describe an interesting case of isolated cardiac manifestation of AL-amyloidosis manifesting as an incipient infiltrative cardiomyopathy with heart failure symptoms due to moderate left ventricular diastolic dysfunction. Restrictive cardiomyopathy with severe diastolic dysfunction is considered as the characteristic manifestation of fully developed cardiac amyloidosis. However, the organ deposition of amyloid is progressive and left ventricular filling worsens continuously, starting with less advanced forms of diastolic dysfunction; the restrictive physiology is characteristic only for advanced phases of the disease. Therefore, the possibility of the incipient infiltrative cardiomyopathy due to the amyloidosis should be considered in patients with heart failure symptoms and echocardiographic findings of unexplained left ventricular hypertrophy with only mild or moderate diastolic dysfunction.

Keywords: heart failure; echocardiography; cardiomyopathy; amyloidosis

Received: April 27, 2008; Accepted: June 3, 2008; Published: October 1, 2008  Show citation