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Transthyretin Cardiac Amyloidosis and Aortic Stenosis: Connection and Therapeutic Implications

[ Vol. 16 , Issue. 3 ]

Author(s):

Jorge Penalver*, Maxwell Ambrosino, Hee D. Jeon, Akanksha Agrawal, Napatt Kanjanahattakij, Marie Pitteloud, Jessica Stempel and Aman Amanullah   Pages 221 - 230 ( 10 )

Abstract:


Background: There is a growing interest in the observed significant incidence of transthyretin cardiac amyloidosis in elderly patients with aortic stenosis. Approximately, 16% of patients with severe aortic stenosis undergoing aortic valve replacement have transthyretin cardiac amyloidosis. Outcomes after aortic valve replacement appear to be worst in patients with concomitant transthyretin cardiac amyloidosis.

Methods: Publications in PubMed, Cochrane Library, and Embase databases were systematically searched from January 2012 to September 2018 using the keywords transthyretin, amyloidosis, and aortic stenosis. All studies published in English that reported the prevalence, association and outcomes of transthyretin cardiac amyloidosis in patients with aortic stenosis undergoing were included.

Results/Conclusion: The relationship between aortic stenosis and transthyretin cardiac amyloidosis is not well understood. A few studies have proven successful surgical management when both conditions coexist. This systematic review suggests that transthyretin cardiac amyloidosis is common in elderly patients with aortic stenosis and tend to have high mortality rates after AVR. The significant incidence of the two diseases occurring simultaneously warrants further investigation to improve management strategies in the future.

Keywords:

Aortic stenosis, transthyretin, cardiac amyloidosis, aortic valve replacement, left ventricular myocardium, hereditary amyloidosis.

Affiliation:

Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, Department of Medicine, Cardiovascular Disease, Einstein Medical Center, PA 19141

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