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Heart Transplantation for Homozygous Familial Transthyretin (TTR) V122I Cardiac Amyloidosis
Article first published online: 3 MAR 2008
DOI: 10.1111/j.1600-6143.2008.02162.x
©2008 The Authors Journal compilation © 2008 The American Society of Transplantation and the American Society of Transplant Surgeons
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How to Cite
Hamour, I. M., Lachmann, H. J., Goodman, H. J. B., Petrou, M., Burke, M. M., Hawkins, P. N. and Banner, N. R. (2008), Heart Transplantation for Homozygous Familial Transthyretin (TTR) V122I Cardiac Amyloidosis. American Journal of Transplantation, 8: 1056–1059. doi: 10.1111/j.1600-6143.2008.02162.x
Publication History
- Issue published online: 14 APR 2008
- Article first published online: 3 MAR 2008
- Received 03 December 2007, revised and accepted for publication 07 January 2008
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Keywords:
- Amyloidosis;
- heart transplantation;
- transthyretin;
- TTR V122I
The patient with heart failure due to cardiac amyloidosis caused by the familial transthyretin V122I variant received a successful heart transplant with no evidence of recurrence at 3 years.
Heart failure is the usual cause of death in patients with amyloid cardiomyopathy. The commonest form of hereditary cardiac amyloidosis is associated with the Val122Ile variant of transthyretin (TTR), which is carried by 3–4% of the African American population. Here, we report the outcome of the first cardiac transplantation in a patient with TTR V122I. A 59-year-old Caribbean man presented with biventricular failure. Other than previous bilateral carpel tunnel syndrome, he had been well and had no evidence of extracardiac amyloidosis. An endomyocardial biopsy demonstrated amyloid of TTR type. Sequencing of TTR gene indicated homozygosity for V122I. He underwent cardiac transplantation and 3 years later, remains well with no evidence of allograft or systemic amyloid deposition.