Cardiac amyloidosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]; Raviteja Guddeti, M.B.B.S. [3]; Cafer Zorkun, M.D., Ph.D. [4]; Lakshmi Gopalakrishnan, M.B.B.S. [5]
Overview
The presence or absence of cardiac involvement with amyloid is the most important prognostic factor.[1]
Natural History
If untreated cardiac amyloidosis (CA) is associate with high complication rate and eventually death. Cardiac involvement in AL amyloidosis is associated with a higher mortality rate compared with that in senile and familial forms of amyloidosis. Also, heart failure in AL type CA is difficult to treat adequately compared with that associated with TTR type.
Complications
The following are the complications of CA:
- Congestive heart failure
- Sudden cardiac death
- Myocardial infarction
- Pericardial effusion
- Valvular defects, aortic stenosis being the most common followed by mitral regurgitation
- Atrial fibrillation
- Tachyarrhythmias
- Ventricular arrhythmias
- Sick sinus syndrome (occasionally)
- Heart blocks and other conduction defects. Higher degree blocks are unusual in AL type CA. But in the TTR type progressive conduction system disease is common and requires pacemaker implantation. Most commonly electrophysiologic function appears to be abnormal in the His-Purkinje system. Prolonged infra-His conduction time is an independent predictor of sudden death in these patients. [2]
- Syncope or presyncope
- Increased sensitivity to digoxin with the potential for digoxin toxicity and related arrhythmias. The amyloid protein tends to bind the digoxin and increase its local concentration in the conduction system for instance.
- Ascites (fluid accumulation in the abdomen)
- Cardiac tamponade[3]
- Stroke and claudication due to intracardiac thrombus causing thromboembolism.
Prognosis
Cardiac amyloidosis is a chronic and progressive condition. A cardiologist may estimate the prognosis according to the thickness of the left ventricle and to the degree of restriction in the heart (diastolic dysfunction).
References
- ↑ Gertz MA, Lacy MQ, Dispenzieri A (1999). "Amyloidosis: recognition, confirmation, prognosis, and therapy". Mayo Clinic Proceedings. Mayo Clinic. 74 (5): 490–4. doi:10.4065/74.5.490. PMID 10319082. Retrieved 2012-02-13. Unknown parameter
|month=ignored (help) - ↑ Reisinger J, Dubrey SW, Lavalley M, Skinner M, Falk RH (1997). "Electrophysiologic abnormalities in AL (primary) amyloidosis with cardiac involvement". Journal of the American College of Cardiology. 30 (4): 1046–51. PMID 9316537. Unknown parameter
|month=ignored (help) - ↑ Navarro JF, Rivera M, Ortuño J (1992). "Cardiac tamponade as presentation of systemic amyloidosis". International Journal of Cardiology. 36 (1): 107–8. PMID 1428240. Unknown parameter
|month=ignored (help)