Cardiac amyloidosis medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]; Aarti Narayan, M.B.B.S [3]; Cafer Zorkun, M.D., Ph.D. [4]; Lakshmi Gopalakrishnan, M.B.B.S. [5]
Overview
Major cardiac manifestations of systemic amyloidosis include heart failure and fatal arrhythmias. Therefore treatment of cardiac amyloidosis includes treatment of heart failure and arrhythmias and treatment of the underlying disease. Treatment of heart failure associated with cardiac amyloidosis differs from therapy usually attempted in patients with systolic or diastolic dysfunction.
Medical Therapy
Acute Pharmacotherapy
- Diuretics and angiotensin-converting inhibitors are the standard therapy regimen to treat congestive heart failure secondary to cardiac amyloidosis. However, presence of hypotension may limit the use of diuretics
- Digoxin and calcium channel blockers bind to the amyloid fibrils and thereby have been reported to increase the incidence of congestive heart failure and produce arrhythmias. Hence, these drugs may be avoided in patients with both AL and TTR cardiac amyloidosis.[1][2][3]
- In patients with symptomatic bradycardia, pacemakers may be indicated.[4][5]
Pacemaker
A pacemaker may be needed if the conduction system is involved.
Supportive Measures
Physical activity may continue as long as the patient can tolerate it.
Diet restrictions vary with the extent of cardiomyopathy and heart failure. These may include salt and/or fluid restrictions.
References
- ↑ Gertz MA, Skinner M, Connors LH, Falk RH, Cohen AS, Kyle RA (1985). "Selective binding of nifedipine to amyloid fibrils". The American Journal of Cardiology. 55 (13 Pt 1): 1646. PMID 4003315. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Gertz MA, Falk RH, Skinner M, Cohen AS, Kyle RA (1985). "Worsening of congestive heart failure in amyloid heart disease treated by calcium channel-blocking agents". The American Journal of Cardiology. 55 (13 Pt 1): 1645. PMID 4003314. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Rubinow A, Skinner M, Cohen AS (1981). "Digoxin sensitivity in amyloid cardiomyopathy". Circulation. 63 (6): 1285–8. PMID 7014028. Retrieved 2012-02-13. Unknown parameter
|month=ignored (help) - ↑ Mathew V, Olson LJ, Gertz MA, Hayes DL (1997). "Symptomatic conduction system disease in cardiac amyloidosis". The American Journal of Cardiology. 80 (11): 1491–2. PMID 9399732. Retrieved 2012-02-13. Unknown parameter
|month=ignored (help) - ↑ Mathew V, Chaliki H, Nishimura RA (1997). "Atrioventricular sequential pacing in cardiac amyloidosis: an acute Doppler echocardiographic and catheterization hemodynamic study". Clinical Cardiology. 20 (8): 723–5. PMID 9259166. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help)