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] Untreated cardiac amyloidosis is associated with a very poor prognosis and a high mortality rate. The most common cardiac complications include heart failure, sudden cardiac death due to electromechanical dissociation and pericardial effusion.
Natural History
Untreated cardiac amyloidosis is associated with high rate of cardiac complications and eventually death. Cardiac involvement in AL amyloidosis is associated with a higher mortality rate compared with that in senile amyloidosis and familial forms of amyloidosis. Also, heart failure in AL type amyloidosis of the heart is more difficult to treat as compared with that associated with TTR type amyloid.
Complications
The following are the complications of cardiac amyloidosis:
- Congestive heart failure[2][3]
- Sudden cardiac death (SCD)[4] is largely due to electromechanical dissociation unlike other disease states where sudden cardiac death is predominantly due to other causes such as ventricular arrhythmia.
- Myocardial infarction[5]
- Pericardial effusion[6]
- Cardiac tamponade[7] It should be noted, however, that the classic echocardiographic signs of cardiac tamponade, such as right atrial indentation and right ventricular compression, are not seen in cardiac tamponade associated with cardiac amyoidosis due to the stiffness of the atrial and ventricular walls. Therefore a high degree of suspicion should be maintained in patients with heart failure and a mild to moderate pericardial effusion. [6]
- Valvular heart diseases, with aortic stenosis being the most common followed by mitral regurgitation
- Atrial fibrillation[8]
- Tachyarrhythmias[9]
- Ventricular arrhythmias[10]
- 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. Frequently, the 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. [11]
- Syncope or presyncope[5]
- 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)
- Stroke and claudication due to intracardiac thrombus causing thromboembolism.[12][13] Risk factors include:
- Atrial fibrillation
- Atrial dysfunction due to infiltration of the atria by amyloid
- Left ventricular diastolic dysfunction
- Increased right ventricular wall thickness
- Tachycardia
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). Cardiac involvement is the most important predictor of survival in patients with AL amyloidosis.
Mayo Staging System
Studies have shown that cardiac torponin T and N-terminal proBNP are powerful prognostic indicators in AL amyloidosis. Based on the combined levels of both the indicators a staging system has been developed for risk stratification of patients with AL amyloidosis. Two prognostic models for risk stratification were designed using threshold values for torponins and NT-proBNP. The threshold values used for the markers were: NT-proBNP <332 ng/L, cTnT <0.035 μ<g/L, and cTnI <0.1 <μg/L.[14][15]
| Stage | NT-proBNP | cTnT/cTnI | Median Survival (months) |
| Stage I | Low | Low | 26.4 - 27.2 |
| Stage II | Low or Elevated | Elevated or Low | 10.5 - 11.1 |
| Stage III | Elevated | Elevated | 3.5 - 4.1 |
In 2009 another staging system was proposed incorporating serum free light chain levels into the Mayo staging system. The threshold values used were: NT-proBNP <332 ng/L, cTnT <0.035 μ<g/L, and serum FLC <500mg/L. As the stage increased it was observed that survival rates reduced drastically.
| Stage | NT-proBNP | cTnT | Serum FLC |
| Stage I | Normal | Normal | Low |
| Stage II | High | Normal | Low |
| Stage III | High | Normal | High |
| Stage IV | High | High | High |
The presence of nodular deposits, thick perimyocytic layers of amyloid, and small myocyte diameters in an endomyocardial biopsy are associated with the worst prognosis. [16]
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) - ↑ Shah S, Dungu J, Dubrey SW (2013). "Senile cardiac amyloidosis: an underappreciated cause of heart failure". BMJ Case Reports. 2013. doi:10.1136/bcr-2012-007635. PMID 23391947.
- ↑ Swiecicki PL, Edwards BS, Kushwaha SS, Dispenzieri A, Park SJ, Gertz MA (2013). "Left ventricular device implantation for advanced cardiac amyloidosis". The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation. 32 (5): 563–8. doi:10.1016/j.healun.2013.01.987. PMID 23474361. Unknown parameter
|month=ignored (help) - ↑ Edler C, Saeger W, Orth U, Braun C, Wulff B, Sperhake J (2012). "[Hereditary cardiac amyloidosis with transthyretin mutations. A cause of sudden death ]". Herz (in German). 37 (4): 456–60. doi:10.1007/s00059-011-3566-9. PMID 22301727. Unknown parameter
|month=ignored (help) - ↑ 5.0 5.1 Mugnai G, Cicoira M, Rossi A, Vassanelli C (2011). "Syncope in cardiac amyloidosis and chronic ischemic heart disease: A case report". Experimental and Clinical Cardiology. 16 (2): 51–3. PMC 3126684. PMID 21747665.
- ↑ 6.0 6.1 Brodarick S, Paine R, Higa E, Carmichael KA (1982). "Pericardial tamponade, a new complication of amyloid heart disease". The American Journal of Medicine. 73 (1): 133–5. PMID 7091168. 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) - ↑ Yunis NA, Petrasko MS, Cannistra LB (2000). "An Elderly Man With Progressive Dyspnea on Exertion and Atrial Fibrillation as Manifestations of Senile Cardiac Amyloidosis". The American Journal of Geriatric Cardiology. 9 (2): 69–72. PMID 11416540. Unknown parameter
|month=ignored (help) - ↑ Cheng Z, Zhu K, Tian Z, Zhao D, Cui Q, Fang Q (2013). "The findings of electrocardiography in patients with cardiac amyloidosis". Annals of Noninvasive Electrocardiology : the Official Journal of the International Society for Holter and Noninvasive Electrocardiology, Inc. 18 (2): 157–62. doi:10.1111/anec.12018. PMID 23530486. Unknown parameter
|month=ignored (help) - ↑ Lin G, Dispenzieri A, Brady PA (2010). "Successful termination of a ventricular arrhythmia by implantable cardioverter defibrillator therapy in a patient with cardiac amyloidosis: insight into mechanisms of sudden death". European Heart Journal. 31 (12): 1538. doi:10.1093/eurheartj/ehp592. PMID 20061577. 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) - ↑ Feng D, Syed IS, Martinez M; et al. (2009). "Intracardiac thrombosis and anticoagulation therapy in cardiac amyloidosis". Circulation. 119 (18): 2490–7. doi:10.1161/CIRCULATIONAHA.108.785014. PMID 19414641. Unknown parameter
|month=ignored (help) - ↑ Feng D, Edwards WD, Oh JK; et al. (2007). "Intracardiac thrombosis and embolism in patients with cardiac amyloidosis". Circulation. 116 (21): 2420–6. doi:10.1161/CIRCULATIONAHA.107.697763. PMID 17984380. Unknown parameter
|month=ignored (help) - ↑ Dispenzieri A, Gertz MA, Kyle RA; et al. (2004). "Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis". Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology. 22 (18): 3751–7. doi:10.1200/JCO.2004.03.029. PMID 15365071. Unknown parameter
|month=ignored (help) - ↑ Dispenzieri A, Gertz MA, Kyle RA; et al. (2004). "Prognostication of survival using cardiac troponins and N-terminal pro-brain natriuretic peptide in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation". Blood. 104 (6): 1881–7. doi:10.1182/blood-2004-01-0390. PMID 15044258. Unknown parameter
|month=ignored (help) - ↑ Arbustini E, Merlini G, Gavazzi A; et al. (1995). "Cardiac immunocyte-derived (AL) amyloidosis: an endomyocardial biopsy study in 11 patients". American Heart Journal. 130 (3 Pt 1): 528–36. PMID 7661071. Unknown parameter
|month=ignored (help)