Abstract
Amyloidosis is a rare systemic disease that is notoriously difficult to diagnose early. Cardiac involvement is associated with a poor prognosis. We present the case of a 72-year-old gentleman who underwent elective coronary artery bypass grafting and mitral valve repair. While initially making a good post-operative recovery, a sudden deterioration on post-operative day 3 led to an unexpected mortality. Post-mortem examination demonstrated previously undiagnosed severe systemic amyloidosis, which particularly affected his heart, due to underlying multiple myeloma. It is important to emphasise that due to the absence of the typical findings on routine pre-operative investigations suggestive of cardiac amyloidosis in this patient, cardiac surgeons should be aware that symptoms inconsistent with angiography findings should prompt consideration of amyloidosis. Diagnostic tools including cardiac MRI and ultimately endomyocardial biopsy could then facilitate earlier recognition of this enigmatic disease process.
Similar content being viewed by others
References
Sack FU, Kristen A, Goldschmidt H, Schnabel PA, Dengler T, Koch A, Karck M. Treatment options for severe cardiac amyloidosis: heart transplantation combined with chemotherapy and stem cell transplantation for patients with AL-amyloidosis and heart and liver transplantation for patients with ATTR-amyloidosis. Eur J Cardiothorac Surg. 2008;33(2):257–62.
Kristen AV, Sack FU, Schonland SO, Hegenbart U, Helmke BM, Koch A, Schnabel PA, Röcken C, Hardt S, Remppis A, Goldschmidt H, Karck M, Ho AD, Katus HA, Dengler TJ. Staged heart transplantation and chemotherapy as a treatment option in patients with severe cardiac light-chain amyloidosis. Eur J Heart Fail. 2009;11(10):1014–20.
Neben-Wittich MA, Wittich CM, Mueller PS, Larson DR, Gertz MA, Edwards WD. Obstructive intramural coronary amyloidosis and myocardial ischaemia are common in primary amyloidosis. Am J Med. 2005;118(11):1287.
Piper C, Butz T, Farr M, Faber L, Oldenburg O, Horstkotte D. How to diagnose cardiac amyloidosis early: impact of ECG, tissue Doppler echocardiography, and myocardial biopsy. Amyloid. 2010;17(1):1–9.
Halwani O, Delgado DH. Cardiac amyloidosis: an approach to diagnosis and management. Expert Rev Cardiovasc Ther. 2010;8(7):1007–13.
Bonow RO, Carabello RA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2008;118:e523–661.
Braun G, Sechtem U, Mahrholdt H. Cardiac amyloidosis––cardiovascular magnetic resonance imaging as a valuable diagnostic tool. Dtsch Med Wochenschr. 2009;134(15):734–7.
Whitaker DC, Tungekar MF, Dussek JE. Angina with a normal coronary angiogram caused by amyloidosis. Heart. 2004;90(9):e54.
Massoudy P, Szabo AK, Dirsch O, Wienecke H, van de Wal HJ, Jakob HG. Amyloid of heart and lungs in a patient with low output syndrome after coronary artery bypass grafting. Herz. 2003;28(5):453–6.
Zacek P, Medilek K, Lonsky V, Laco J, Nova M, Dominik J. Cardiac amyloidosis in the cardiosurgical operating room––a rare but fatal trap. Thorac Cardiovasc Surg. 2007;55(2):65–7.
Schwartz JG, Ghidoni JJ. Unsuspected amyloidosis and cardiac arrest following mitral valve replacement. Int J Cardiol. 1985;9(4):485–7.
Kotani N, Hashimoto H, Muraoka M, Kabara S, Okawa H, Matsuki A. Fatal perioperative myocardial infarction in four patients with cardiac amyloidosis. Anesthesiology. 2000;92(3):873–5.
Acknowledgments
We would like to thank Professor Peter Hamilton for his assistance in the production of the histopathology images.
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Fitzmaurice, G.J., Wishart, V. & Graham, A.N.J. An unexpected mortality following cardiac surgery: a post-mortem diagnosis of cardiac amyloidosis. Gen Thorac Cardiovasc Surg 61, 417–421 (2013). https://doi.org/10.1007/s11748-012-0164-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11748-012-0164-6