A Case Report of Restrictive Cardiomyopathy Due To Secondary Cardiac Amyloidosis

Document Type : Case report

Authors

1 Associate Professor .Fellowship of echocardiography Cardiology Department,Ghaem Hospital , Mashhad university of Medical Sciences (MUMS)Mashad. IRAN

2 Resident of cardiology, Ghaem Hospital, (MUMS), Mashhad. IRAN

3 AP&CP Pathologist. Moayed Laboratory. Mashhad .IRAN

Abstract

Introduction
Restrictive cardiomyopathy defined by increased ventricular wall stiffness and impaired LV filling and heart failure symptoms. This type of cardiomyopathy occurs with lower frequency relative to the dilated and hypertrophic cardiomyopathies. IRCM in 50% is idiopathic and in 50% is secondary to specific clinical disorders especially amyloidosis. Cardiac amyloidosis usually occurs during primary amyloidosis (AL type amyloidosis). Primary amyloidosis is often seen in people with multiple myelomacancer or in the in course of immunocytic dyscrasia. Disease process results from tissue deposition of proteins that have a unique secondary structure. Cardiac amyloidosis is an invariably progressive infiltrative cardiomyopathy that carries a grave prognosis we have presented a case of Cardiac amyloidosis which is a rare clinical disorder.
[
Case report
The Present case was a 46 years old man who admitted for progressive dyspnea and congestive heart failure. Echocardiography revealed increased ventricular wall thickness with small size ventricles, enlarged atria, and a thickened intertribal septum and advanced diastolic dysfunction. Electrocardiography revealed low QRS voltage and normal sinus rhythm. Gingival Biopsy examined by congo Red staining and immunohistochemistry identified specific amyloid proteins. Pathology report confirmed amyloidosis.
Conclusion
In patient complaining of progressive congestive heart failure and suspected to have restrictive cardiomyopathy, cardiac amyloidosis should be born in mind.

Keywords

References

1-Liao R, Jain M, Teller P. Isolation of light chains from patients with cardiac amyloidosis causes diastolic dysfunction
in isolated mouse hearts. Circulation 2001; 104:1594.
2- Nadkar MY, Pandit AP, Bamburde SH, Raminder Singh. Cardiac Amyloidosis .Ann Inter Med 2008; 9:321.
3-Wald DS, Gray HH. Restrictive cardiomyopathy in systemic amyloidosis. Q J Med 2003; 96:380.
4-Gertz MA, Rajkumar SV. Primary systemic amyloidosis. Curr Treat Options Oncol 2002; 3:261.
5-Libby P .Braunwalds heart disease:a textbook of cardiovascular medicine. 8th ed.
6-Reisninger J, Dubrey SW, Lavalley M, Skinner M, Falk RH. Electrophysiologic abnormalities in AL (primary)
amyloidosis with cardiac involvement. J AM Coll Cardiol 1997; 30:1046.
7-Trikas A, Rallidis L, Hawkins P, Oakley CM, Nihoyannopoulos P. Comparison of usefulness between exercise
capacity and echocardiographic indices of left ventricular function in cardiac amyloidosis. Am J Cardiol 1999; 84:1049.
8- Cacoub P, Axler O, De Zuttere D, Hausfater P, Amoura Z, Walter S, et al. Amyloidosis and cardiac involvement.
Am Med Interne (P aris) 2000; 151:611. 
medical journal of mashhad university of medical sciences
Volume 53, Issue 2 - Serial Number 2
May and June 2009
Pages 123-126

Files

Share

How to cite

Statistics