The isolated form of cardiac amyloidosis in the form of beginning infiltrative cardiomyopathy without restrictive physiology
Authors:
P. Kuchynka 1; T. Paleček 1; S. Šimek 1; J. C. Lubanda 1; M. Elleder 2; I. Špička 3; P. Jansa 1; A. Linhart 1
Authors‘ workplace:
II. interní klinika – klinika kardiologie a angiologie 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Aleš Linhart, DrSc.
1; Ústav dědičných a metabolických poruch 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Milan Elleder, DrSc.
2; I. interní klinika – klinika hematologie 1. lékařské fakulty UK a VFN Praha, přednosta doc. MUDr. Marek Trněný, DrSc.
3
Published in:
Vnitř Lék 2008; 54(10): 1010-1013
Category:
Case Report
Overview
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.
Key words:
heart failure – echocardiography – cardiomyopathy – amyloidosis
Sources
1. Pepys MB. Amyloidosis. Annu Rev Med 2006; 57: 223–241.
2. Adam Z, Sčudla V. Clinical maniofestation of AL-amyloidosis and some other types of amyloidosis. Vnitř Lék 2001; 47: 36–45.
3. Shah BK, Inoue Y, Mehra MR. Amyloidosis and the heart. Arch Intern Med 2006; 166: 1805–1813.
4. Dubrey SW, Cha K, Anderson J et al. The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. Q J Med 1998; 91: 141–157.
5. Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 patients. Semin Hematol 1995; 32: 45–49.
6. Palka P, Lange A, Donnelly E et al. Dop-pler tissue echocardiographic features of cardiac amyloidosis. J Am Soc Echocardiogr 2002; 15: 1353–1359.
7. Brychta T, Pařenica J, Zatočil T et al. Restrictive cardiomyopathy as a manifestation of primary amyloidosis. Vnitř Lék 2004; 50: 66–71.
8. Murtagh B, Hammill SC, Gertz MA et al. Electrocardiographic findings in primary systemic amyloidosis and biopsy proven cardiac involvement. Am J Cardiol 2005; 95: 535–537.
9. Klein AL, Hatle LK, Burstow DJ et al. Doppler characterization of left ventricular diastolic function in cardiac amyloidosis. J Am Coll Cardiol 1989; 13: 1017–1026.
10. Linhart A, Paleček T, Aschermann M. Echokardiografie pro praxi. Praha: Audioscan 2002: 117–118.
11. Pellikka PA, Holmes DR, Edwards WD et al. Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement. Arch Intern Med 1988; 148: 662–666.
12. Oh JK, Hatle LK, Tajik AJ et al. Diastolic heart failure can be diagnose by comprehensive two-dimensional and Doppler echocardiography. J Am Coll Cardiol 2006; 47: 500–506.
13. Elliott P, Anderson B, Arbustini E et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008; 29: 270–276.
14. Comenzo RL, Gertz MA. Autologous stem cell transplantation for primary systemic amyloidosis. Blood 2002; 99: 4276–4282.
15. Jaccard A, Moreau P, Leblond V et al. High‑dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N Engl J Med 2007; 357: 1083–1093.
Labels
Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
2008 Issue 10
Most read in this issue
- Dual antiplatelet treatment
- Hemopurification in sepsis: current view
- Hemophilia
- Cardiac Arrhythmias in Obstructive Sleep Apnea