Cardiac amyloidosis surgery: Difference between revisions
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==Surgery== | ==Surgery== | ||
===Pacemaker=== | ===Pacemaker=== | ||
Patients with cardiac amyloidosis (CA) are prone to conduction system disease due to infiltration of the heart's electrical conduction system by the amyloid deposits. Progressive conduction system disease is common in TTR type CA often requiring pacemaker implantation. Electrophysiologic function appears to be abnormal most commonly in the His-Purkinje system. Prolonged infra-His conduction time is an independent predictor of [[sudden death]] in these patients. <ref name="pmid9316537">{{cite journal |author=Reisinger J, Dubrey SW, Lavalley M, Skinner M, Falk RH |title=Electrophysiologic abnormalities in AL (primary) amyloidosis with cardiac involvement |journal=[[Journal of the American College of Cardiology]] |volume=30 |issue=4 |pages=1046–51 |year=1997 |month=October |pmid=9316537 |doi= |url=}}</ref> | Patients with cardiac amyloidosis (CA) are prone to conduction system disease due to infiltration of the heart's electrical conduction system by the amyloid deposits. Progressive conduction system disease is common in TTR type CA and senile CA often requiring pacemaker implantation. Electrophysiologic function appears to be abnormal most commonly in the His-Purkinje system. Prolonged infra-His conduction time is an independent predictor of [[sudden death]] in these patients. <ref name="pmid9316537">{{cite journal |author=Reisinger J, Dubrey SW, Lavalley M, Skinner M, Falk RH |title=Electrophysiologic abnormalities in AL (primary) amyloidosis with cardiac involvement |journal=[[Journal of the American College of Cardiology]] |volume=30 |issue=4 |pages=1046–51 |year=1997 |month=October |pmid=9316537 |doi= |url=}}</ref> | ||
===Cardiac Transplantation=== | ===Cardiac Transplantation=== | ||
[[Heart transplantation]] is an option to be considered in the treatment of CA. In patients with | [[Heart transplantation]] is an option to be considered in the treatment of CA. Involvement of non-cardiac organs in AL type CA makes the patient not suitable for cardiac transplantation. Only 4% of the patients have isolated cardiac amyloidosis unlike senile CA. In those patients with isolated heart involvement, cardiac transplantation followed by initiation of autologous [[hematopoietic stem cell transplantation]] ([[HSCT]]) within 12 months, has been shown to be associated with better survival rates. However, selection of suitable candidates is a key step while considering cardiac transplantation. A suitable patient with AL type CA would be: | ||
* Young (<60 years age) | * Young (<60 years age) | ||
* Mayo stage III CA | * Mayo stage III CA | ||
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* Low-level bone marrow plasmacytosis | * Low-level bone marrow plasmacytosis | ||
* Should be eligible for HSCT after heart transplantation | * Should be eligible for HSCT after heart transplantation | ||
According to a study in the UK, 5 year survival rates after transplantation were found to be lower compared with the rates after transplantation for non-AL amyloidosis.<ref name="pmid15477107">{{cite journal |author=Dubrey SW, Burke MM, Hawkins PN, Banner NR |title=Cardiac transplantation for amyloid heart disease: the United Kingdom experience |journal=[[The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation]] |volume=23 |issue=10 |pages=1142–53 |year=2004 |month=October |pmid=15477107 |doi=10.1016/j.healun.2003.08.027 |url=}}</ref> | |||
===Liver Transplantation=== | |||
[[Transthyretin]] is produced exclusively in the liver. Transplantation of liver abolishes the production of mutated amyloid protein and thereby halts the deposition of amyloid in the heart.<ref name="pmid10827225">{{cite journal |author=Suhr OB, Herlenius G, Friman S, Ericzon BG |title=Liver transplantation for hereditary transthyretin amyloidosis |journal=[[Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society]] |volume=6 |issue=3 |pages=263–76 |year=2000 |month=May |pmid=10827225 |doi=10.1053/lv.2000.6145 |url=}}</ref> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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[[CME Category::Cardiology]] | |||
[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
[[Category:Rheumatology]] | [[Category:Rheumatology]] | ||
Latest revision as of 06:36, 15 March 2016
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Risk calculators and risk factors for Cardiac amyloidosis surgery |
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]
Overview
When heart function is very poor, a heart transplant may be considered for some patients, but not those with AL type amyloidosis since their disease compromises many organs. In one type of secondary amyloidosis, liver transplantation is also required.
Surgery
Pacemaker
Patients with cardiac amyloidosis (CA) are prone to conduction system disease due to infiltration of the heart's electrical conduction system by the amyloid deposits. Progressive conduction system disease is common in TTR type CA and senile CA often requiring pacemaker implantation. Electrophysiologic function appears to be abnormal most commonly in the His-Purkinje system. Prolonged infra-His conduction time is an independent predictor of sudden death in these patients. [1]
Cardiac Transplantation
Heart transplantation is an option to be considered in the treatment of CA. Involvement of non-cardiac organs in AL type CA makes the patient not suitable for cardiac transplantation. Only 4% of the patients have isolated cardiac amyloidosis unlike senile CA. In those patients with isolated heart involvement, cardiac transplantation followed by initiation of autologous hematopoietic stem cell transplantation (HSCT) within 12 months, has been shown to be associated with better survival rates. However, selection of suitable candidates is a key step while considering cardiac transplantation. A suitable patient with AL type CA would be:
- Young (<60 years age)
- Mayo stage III CA
- NYHA III/IV symptoms after adequate diuresis
- Good renal and/or liver functioning
- No significant autonomic neuropathy
- Low-level bone marrow plasmacytosis
- Should be eligible for HSCT after heart transplantation
According to a study in the UK, 5 year survival rates after transplantation were found to be lower compared with the rates after transplantation for non-AL amyloidosis.[2]
Liver Transplantation
Transthyretin is produced exclusively in the liver. Transplantation of liver abolishes the production of mutated amyloid protein and thereby halts the deposition of amyloid in the heart.[3]
References
- ↑ 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) - ↑ Dubrey SW, Burke MM, Hawkins PN, Banner NR (2004). "Cardiac transplantation for amyloid heart disease: the United Kingdom experience". The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation. 23 (10): 1142–53. doi:10.1016/j.healun.2003.08.027. PMID 15477107. Unknown parameter
|month=ignored (help) - ↑ Suhr OB, Herlenius G, Friman S, Ericzon BG (2000). "Liver transplantation for hereditary transthyretin amyloidosis". Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society. 6 (3): 263–76. doi:10.1053/lv.2000.6145. PMID 10827225. Unknown parameter
|month=ignored (help)