Diagnosis and management of the cardiac amyloidoses

Circulation

Volumn 112, Issue 13, 2005, Pages 2047-2060

  Falk, Rodney H ^a,b  

^a BRIGHAM AND WOMEN S HOSPITAL   (United States)

^b HARVARD VANGUARD MEDICAL ASSOCIATES   (United States)

Author keywords

Amyloid; Cardiomyopathy; Echocardiography; Heart diseases; Heart failure

Indexed keywords

AMYLOID; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; DIURETIC AGENT; PREALBUMIN;

AMYLOIDOSIS; GENETIC DISORDER; HEART AMYLOIDOSIS; HEART CATHETERIZATION; HEART FAILURE; HUMAN; MYOCARDIAL DISEASE; PATHOGENESIS; PLASMA CELL DYSCRASIA; PRIORITY JOURNAL; REVIEW; SENILITY;

AMYLOID; AMYLOIDOSIS; CARDIOMYOPATHIES; HUMANS;

EID: 25444434458     PISSN: 00097322     EISSN: None     Source Type: Journal    
DOI: 10.1161/CIRCULATIONAHA.104.489187     Document Type: Review

References (123)

1. Merlini G, Bellotti V. Molecular mechanisms of amyloidosis. N Engl J Med. 2003;349:583-596.
2. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997;337:898-909.
3. Sipe JD, Cohen AS. History of the amyloid fibril. J Struct Biol. 2000; 130:88-98.
4. Selkoe DJ. Folding proteins in fatal ways. Nature. 2003;426:900-904.
5. Merlini G, Westermark P. The systemic amyloidoses: clearer understanding of the molecular mechanisms offers hope for more effective therapies. J Intern Med. 2004;255:159-178.
6. Dubrey SW, Cha K, Simms RW, Skinner M, Falk RH. Electrocardiography and Doppler echocardiography in secondary (AA) amyloidosis. Am J Cardiol 1996;77:313-315.
7. Westermark P, Benson MD, Buxbaum JM, Cohen AS, Frangione B, Ikeda S, Masters CL, Merlini G, Saraiva MJ, Sipe J. Amyloid protein fibril nomenclature-2002. Amyloid. 2002;9:197-200.
8. Smith TJ, Kyle RA, Lie JT. Clinical significance of histopathologic patterns of cardiac amyloidosis. Mayo Clin Proc. 1984;59:547-555.
9. Gertz MA, Lacy MQ, Dispenzieri A. Amyloidosis hematology. Oncol Clin North Am. 1999;13:1211-1220.
10. Dubrey SW, Cha K, Anderson J, Chamarthi B, Reisinger J, Skinner M, Falk RH, The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. QJM. 1998;91:141-157.
11. Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995;32:45-59.
12. Mueller PS, Edwards WD, Gertz MA. Symptomatic ischemic heart disease resulting from obstructive intramural coronary amyloidosis. Am J Med. 2000;109:181-188.
13. Miani D, Rocco M, Alberti E, Spedicato L, Fioretti PM. Amyloidosis of epicardial and intramural coronary arteries as an unusual cause of myocardial infarction and refractory angina pectoris. Ital Heart J. 2002;3:479-482.
14. Zabernigg A, Schranzhofer R, Kreczy A, Gattringer K. Continuously elevated cardiac troponin I in two patients with multiple myeloma and fatal cardiac amyloidosis. Ann Oncol. 2003;14:1791.
15. Dispenzieri A, Kyle RA, Gertz MA, Therneau TM, Miller WL, Chandrasekaran K, McConnell JP, Burritt MF, Jaffe AS. Survival in patients with primary systemic amyloidosis and raised serum cardiac troponins. Lancet. 2003;361:1787-1789.
16. Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, McConnell JP, Litzow MR, Gastineau DA, Tefferi A, Inwards DJ, Micallef IN, Ansell SM, Porrata LF, Elliott MA, Hogan WJ, Rajkumar SV, Fonseca R, Greipp PR. Witzig TE, Lust JA, Zeldenrust SR, Snow DS, Hayman SR, McGregor CG, Jaffe AS. 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. 2004;104:1881-1887.
17. Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, Greipp PR, Witzig TE, Lust JA, Rajkumar SV, Fonseca R, Zeldenrust SR, McGregor CG, Jaffe AS. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 2004;22:3751-3757.
18. Cantwell RV, Aviles RJ, Bjornsson J, Wright RS, Freeman WK, Oh JK, Hoyer JD, Markovic S, Jaffe AS. Cardiac amyloidosis presenting with elevations of cardiac troponin I and angina pectoris. Clin Cardiol. 2002;25:33-37.
19. Falk RH, Rubinow A, Cohen AS. Cardiac arrhythmias in systemic amyloidosis: correlation with echocardiographic abnormalities. J Am Coll Cardiol. 1984;3:107-113.
20. Chamarthi B, Dubrey SW, Cha K, Skinner M, Falk RH. Features and prognosis of exertional syncope in light-chain associated AL cardiac amyloidosis. Am J Cardiol. 1997;80:1242-1245.
21. Rubinow A, Cohen AS. Skin involvement in generalized amyloidosis: a study of clinically involved and uninvolved skin in 50 patients with primary and secondary amyloidosis. Ann Intern Med. 1978;88:781-785.
22. Daoud MS, Lust JA, Kyle RA, Pittelkow MR. Monoclonal gammopathies and associated skin disorders. J Am Acad Dermatol. 1999; 40:507-535: quiz 536-538.
23. Burroughs EI, Aronson AE, Duffy JR, Kyle RA. Speech disorders in systemic amyloidosis. Br J Disord Commun. 1991;26:201-206.
24. Park MA, Mueller PS, Kyle RA, Larson DR, Plevak MF, Gertz MA. Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients. Medicine. 2003;82:291-298.
25. Derrick EK, Price ML. Primary systemic amyloid with nail dystrophy. J Royal Soc Med. 1995;88:290P-291P.
26. Klein AL, Cohen GI. Doppler echocardiographic assessment of constrictive pericarditis, cardiac amyloidosis, and cardiac tamponade. Cleveland Clin J Med. 1992;59:278-290.
27. Tyberg TI, Goodyer AV, Hurst VWD, Alexander J, Langou RA. Left ventricular filling in differentiating restrictive amyloid cardiomyopathy and constrictive pericarditis. Am J Cardiol. 1981;47:791-796.
28. Murphy L, Falk RH. Left atrial kinetic energy in AL amyloidosis: can it detect early dysfunction? Am J Cardiol. 2000;86:244-246.
29. Modesto KM, Dispenzieri A, Cauduro AS, Lacy MQ, Khandheria BK, Pellika PA, Belohlavek M, Seward JB, Kyle RA, Tajik AJ, Gertz MA, Abraham TP. Left atrial myopathy in cardiac amyloidosis: implications of novel echocardiographic techniques. Eur Heart J. 2005;26:173-179.
30. Bernardi L, Passino C, Porta C, Anesi E, Palladini G, Merlini G. Widespread cardiovascular autonomic dysfunction in primary amyloidosis: does spontaneous hyperventilation have a compensatory role against postural hypotension? Heart. 2002;88:615-621.
31. Berk JL, Keane J, Seldin DC, Sanchorawala V, Koyama J, Dember LM, Falk RH. Persistent pleural effusions in primary systemic amyloidosis: etiology and prognosis. Chest. 2003;124:969-977.
32. Gillmore JD, Lovat LB, Hawkins PN. Amyloidosis and the liver. J Hepatol. 1999;30(suppl 1):17-33.
33. Chopra S, Rubinow A, Koff RS, Cohen AS. Hepatic amyloidosis: a histopathologic analysis of primary (AL) and secondary (AA) forms. Am J Pathol. 1984;115:186-193.
34. Murtagh B, Hammill SC, Gertz MA, Kyle RA, Tajik AJ, Grogan M. Electrocardiographic findings in primary systemic amyloidosis and biopsy-proven cardiac involvement. Am J Cardiol. 2005;95:535-537.
35. Siqueira-Filho AG, Cunha CL, Tajik AJ, Seward JB, Schattenberg TT, Giuliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation. 1981;63:188-196.
36. Klein AL, Hatle LK, Taliercio CP, Taylor CL, Kyle RA, Bailey KR, Seward JB, Tajik AJ. Serial Doppler echocardiographic follow-up of left ventricular diastolic function in cardiac amyloidosis. J Am Coll Cardiol. 1990;16:1135-1141.
37. Child JS, Levisman JA, Abbasi AS, MacAlpin RN. Echocardiographic manifestations of infiltrative cardiomyopathy: a report of seven cases due to amyloid. Chest. 1976;70:726-731.
38. Carroll JD, Gaasch WH, McAdam KP. Amyloid cardiomyopathy: characterization by a distinctive voltage/mass relation. Am J Cardiol. 1982;49:9-13.
39. Sedlis SP, Saffitz JE, Schwob VS, Jaffe AS. Cardiac amyloidosis simulating hypertrophic cardiomyopathy. Am J Cardiol. 1984;53:969-970.
40. Hemmingson LO, Eriksson P. Cardiac amyloidosis mimicking hypertrophic cardiomyopathy. Acta Med Scand. 1986;219:421-423.
41. Klein AL, Hatle LK, Burstow DJ, Seward JB, Kyle RA, Bailey KR, Luscher TF, Gertz MA, Tajik AJ. Doppler characterization of left ventricular diastolic function in cardiac amyloidosis. J Am Coll Cardiol. 1989;13:1017-1026.
42. Abdalla I, Murray RD, Lee JC, Stewart WJ, Tajik AJ, Klein AL. Duration of pulmonary venous atrial reversal flow velocity and mitral inflow a wave: new measure of severity of cardiac amyloidosis. J Am Soc Echocardiogr. 1998;11:1125-1133.
43. Dubrey S, Pollak A, Skinner M, Falk RH. Atrial thrombi occurring during sinus rhythm in cardiac amyloidosis: evidence for atrial electromechanical dissociation. Br Heart J. 1995;74:541-544.
44. Plehn JF, Southworth J, Cornwell GG. Brief report: atrial systolic failure in primary amyloidosis. N Engl J Med. 1992;327:1570-1573.
45. Santarone M, Corrado G, Tagliagambe LM, Manzillo GF, Tadeo G, Spata M, Longhi M. Atrial thrombosis in cardiac amyloidosis: diagnostic contribution of transesophageal echocardiography. J Am Soc Echocardiogr. 1999;12:533-536.
46. Moyssakis I, Triposkiadis F, Pantazopoulos NJ, Kyriakidis M, Nihoyannopoulos P. Left atrial systolic function in primary and familial amyloidosis: assessment from left atrial volume change. Clin Cardiol. 2004;27:528-532.
47. Koyama J, Ray-Sequin PA, Davidoff R, Falk RH. Usefulness of pulsed tissue Doppler imaging for evaluating systolic and diastolic left ventricular function in patients with AL (primary) amyloidosis. Am J Cardiol. 2002;89:1067-1071.
48. Koyama J, Davidoff R, Falk RH. Longitudinal myocardial velocity gradient derived from pulsed Doppler tissue imaging in AL amyloidosis: a sensitive indicator of systolic and diastolic dysfunction. J Am Soc Echocardiogr. 2004;17:36-44.
49. Koyama J, Ray-Sequin PA, Falk RH. Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation. 2003;107:2446-2452.
50. Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, Sheppard MN, Poole-Wilson PA, Hawkins PN, Pennell DJ. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 2005;111:186-193.
51. Kwong RY, Falk RH. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation. 2005;111:122-124.
52. Swanton RH, Brooksby IA. Davies MJ, Coltart DJ, Jenkins BS, Webb-Peploe MM. Systolic and diastolic ventricular function in cardiac amyloidosis: studies in six cases diagnosed with endomyocardial biopsy. Am J Cardiol. 1977;39:658-664.
53. Kern MJ, Lorell BH, Grossman W. Cardiac amyloidosis masquerading as constrictive pericarditis. Cathet Cardiovasc Diagn. 1982;8:629-635.
54. Robbins MA, Pizzarello RA, Stechel RP, Chiaramida SA, Gulotta SJ. Resting and exercise hemodynamics in constrictive pericarditis and a case of cardiac amyloidosis mimicking constriction. Cathet Cardiovasc Diagn. 1983;9:463-471.
55. Lorell BH, Grossman W. Profiles in constrictive pericarditis, restrictive cardiomyopathy and cardiac tamponade. In: Bairn DS, Grossman W, eds. Grossman's Cardiac Catheterization, Angiography and Intervention. 6th ed. Philadelphia, Pa: Lippincott, Williams & Wilkins; 2000:829-850.
56. Hurrell DG, Nishimura RA, Higano ST, Appleton CP, Danielson GK, Holmes DR Jr, Tajik AJ. Value of dynamic respiratory changes in left and right ventricular pressures for the diagnosis of constrictive pericarditis. Circulation. 1996;93:2007-2013.
57. Pomerance A, Slavin G, McWatt J. Experience with the sodium sulphate-Alcian blue stain for amyloid in cardiac pathology. J Clin Pathol. 1976;29:22-26.
58. Libbey CA, Skinner M, Cohen AS. Use of abdominal fat tissue aspirate in the diagnosis of systemic amyloidosis. Arch Intern Med. 1983;143:1549-1552.
59. Ansari-Lari MA, Ali SZ. Fine-needle aspiration of abdominal fat pad for amyloid detection: a clinically useful test? Diagn Cytopathol. 2004;30: 178-181.
60. Ardehali H, Qasim A, Cappola T, Howard D, Hruban R, Hare JM, Baughman KL, Kasper EK. Endomyocardial biopsy plays a role in diagnosing patients with unexplained cardiomyopathy. Am Heart J. 2004;147:919-923.
61. Gertz MA, Grogan M, Kyle RA, Tajik AJ. Endomyocardial biopsy-proven light chain amyloidosis (AL) without echocardiographic features of infiltrative cardiomyopathy. Am J Cardiol. 1997;80:93-95.
62. Abraham RS, Katzmann JA, Clark RJ, Bradwell AR, Kyle RA, Gertz MA. Quantitative analysis of serum free light chains: a new marker for the diagnostic evaluation of primary systemic amyloidosis. Am J Clin Pathol. 2003;119:274-278.
63. Katzmann JA, Clark RJ, Roshini S, Abraham RS, Bryant S, Lymp JF, Bradwell AR, Kyle RA. Serum reference intervals and diagnostic ranges for free and free immunoglobulin light chains: relative sensitivity for detection of monoclonal light chains. Clin Chem. 2002;48:1437-1444.
64. Katzmann JA, Abraham RS, Dispenzieri A, Lust JA, Kyle RA. Diagnostic performance of quantitative kappa and lambda free light chain assays in clinical practice. Clin Chem. 2005;51:878-881.
65. Swan N, Skinner M, O'Hara CJ. Bone marrow core biopsy specimens in AL (primary) amyloidosis: a morphologic and immunohistochemical study of 100 cases. Am J Clin Pathol. 2003;120:610-616.
66. Kyle RA, Therneau TM, Rajkumar SV, Larson DR, Plevak MF, Melton LJ. Long-term follow-up of 241 patients with monoclonal gammopathy of undetermined significance: the original Mayo Clinic series 25 years later. Mayo Clin Proc. 2004;79:859-866.
67. Rajkumar SV, Kyle RA, Themeau TM, Clark RJ, Bradwell AR, Melton LJ, Larson DR, Plevak MF, Katzmann JA. Presence of monoclonal free light chains in the serum predicts risk of progression in monoclonal gammopathy of undetermined significance. Br J Haematol. 2004;127:308-310.
68. Anesi E, Palladini G, Perfetti V, Arbustini E, Obici L, Merlini G. Therapeutic advances demand accurate typing of amyloid deposits. Am J Med. 2001;111:243-244.
69. Arbustini E, Verga L, Concardi M, Palladini G, Obici L, Merlini G. Electron and immuno-electron microscopy of abdominal fat identifies and characterizes amyloid fibrils in suspected cardiac amyloidosis. Amyloid. 2002;9:108-114.
70. O'Hara CJ, Falk RH. The diagnosis and typing of cardiac amyloidosis. Amyloid. 2003;10:127-129.
71. Lachmann HJ, Booth DR, Booth SE, Bybee A, Gilbertson JA, Gillmore JD, Pepys MB, Hawkins PN. Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. N Engl J Med. 2002;346:1786-1791.
72. Gertz MA, Falk RH, Skinner M, Cohen AS, Kyle RA. Worsening of congestive heart failure in amyloid heart disease treated by calcium channel-blocking agents. Am J Cardiol. 1985;55:1645.
73. Pollak A, Falk RH. Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis. Chest. 1993;104:618-620.
74. Rubinow A, Skinner M, Cohen AS. Digoxin sensitivity in amyloid cardiomyopathy. Circulation. 1981;63:1285-1288.
75. De Lorenzi E, Giorgetti S, Grossi S, Merlini G, Caccialanza G, Bellotti V. Pharmaceutical strategies against amyloidosis: old and new drugs in targeting a "protein misfolding disease." Curr Med Chem. 2004;11:1065-1084.
76. Sanchorawala V, Wright DG, Seldin DC, Falk RH, Finn KT, Dember LM, Berk JL, Quillen K, Anderson JJ, Comenzo RL, Skinner M. High-dose intravenous melphalan and autologous stem cell transplantation as initial therapy or following two cycles of oral chemotherapy for the treatment of AL amyloidosis: results of a prospective randomized trial. Bone Marrow Transplant. 2004;33:381-388.
77. Gertz MA, Lacy MQ, Dispenzieri A. Therapy for immunoglobulin light chain amyloidosis: the new and the old. Blood Reviews. 2004;18:17-37.
78. Palladini G, Perfetti V, Obici L, Caccialanza R, Semino A, Adami F, Cavallero G, Rustichelli R, Virga G, Merlini G. Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation. Blood. 2004;103:2936-2938.
79. Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, Anderson JJ, O'Hara C, Finn KT, Libbey CA, Wiesman J, Quillen K, Swan N, Wright DG. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med. 2004;140:85-93.
80. Seldin DC, Choufani EB, Dember LM, Wiesman JF, Berk JL, Falk RH, O'Hara C, Fennessey S, Finn KT, Wright DG, Skinner M, Sanchorawala V. Tolerability and efficacy of thalidomide for the treatment of patients with light chain-associated (AL) amyloidosis. Clin Lymphoma. 2003;3:241-246.
81. Sanchorawala V, Wright DG, Seldin DC, Falk RH, Berk JL, Dember LM, Finn KT, Skinner M. Low-dose continuous oral melphalan for the treatment of primary systemic (AL) amyloidosis. Br J Haematol. 2002; 117:886-889.
82. Falk RH, Reisinger J, Dubrey SW, Mendes LA, Sanchorawala V, Ekery D, Comenzo R, Vosburgh E, Skinner M. The effect of cardiac involvement on the outcome of intravenous melphalan therapy and autologous stem cell rescue for AL amyloidosis. In: Kyle RA. Gertz MA. eds. Amyloid and the Amyloidoses: VIIIIth International Symposium on Amyloidosis. Rochester. Minn: Parthenon; 1998:181-183.
83. Dubrey S, Mendes L, Skinner M, Falk RH. Resolution of heart failure in patients with AL amyloidosis. Ann Intern Med. 1996;125:481-484.
84. Liao R, Jain M, Teller P. Connors LH, Ngoy S, Skinner M, Falk RH, Apstein CS. Infusion of light chains from patients with cardiac amyloidosis causes diastolic dysfunction in isolated mouse hearts. Circulation. 2001;104:1594-1597.
85. Brenner DA, Jain M, Pimentel DR, Wang B, Connors LH, Skinner M, Apstein CS, Liao R. Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress. Circ Res. 2004;94:1008-1010.
86. Lachmann HJ, Gallimore R, Gillmore JD, Carr-Smith HD, Bradwell AR, Pepys MB, Hawkins PN. Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapy. Br J Haematol. 2003;122:78-84.
87. Skinner M, Anderson J, Simms R, Falk R, Wang M, Libbey C, Jones LA, Cohen AS. Treatment of 100 patients with primary amyloidosis: a randomized trial of melphalan, prednisone, and colchicine versus colchicine only. Am J Med. 1996;100:290-298.
88. Wardley AM, Jayson GC, Goldsmith DJ, Yenning MC, Ackrill P, Scarffe JH. The treatment of nephrotic syndrome caused by primary (light chain) amyloid with vincristine, doxorubicin and dexamethasone. Br J Cancer. 1998;78:774-776.
89. Hosenpud JD, Uretsky BF, Griffith BP, O'Connell JB, Olivari MT, Valantine HA. Successful intermediate-term outcome for patients with cardiac amyloidosis undergoing heart transplantation: results of a multicenter survey. J Heart Transplant. 1990;9:346-350.
90. Dubrey SW, Burke MM, Hawkins PN, Banner NR. Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant. 2004;23:1142-1153.
91. Hosenpud JD, DeMarco T, Frazier OH, Griffith BP, Uretsky BF, Menkis AH, O'Connell JB, Olivari MT, Valantine HA. Progression of systemic disease and reduced long-term survival in patients with cardiac amyloidosis undergoing heart transplantation: follow-up results of a multicenter survey. Circulation. 1991;84:III-338-II-43.
92. Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis. 2003;42:1154-1163.
93. Gallo G, Goni F, Boctor F, Vidal R, Kumar A, Stevens FJ, Frangione B. Ghiso J. Light chain cardiomyopathy: structural analysis of the light chain tissue deposits. Am J Pathol. 1996;148:1397-1406.
94. Buxbaum JN, Genega EN, Lazowski P, Kumar A, Tunick PA, Kronzon I, Gallo GA. Infiltrative nonamyloidotic monoclonal immunoglobulin light chain cardiomyopathy: an underappreciated manifestation of plasma cell dyscrasias. Cardiology. 2000;93:220-228.
95. Nakamura M, Satoh M, Kowada S, Satoh H, Tashiro A, Sato F, Masuda, Hiramori K. Reversible restrictive cardiomyopathy due to light-chain deposition disease. Mayo Clin Proc. 2002;77:193-196.
96. Dubrey SW, Cha K, Skinner M, LaValley M, Falk RH. Familial and primary (AL) cardiac amyloidosis: echocardiographically similar diseases with distinctly different clinical outcomes. Heart. 1997;78:74-82.
97. Ogiwara F, Koyama J, Ikeda S, Kinoshita O, Folk RH. Comparison of the strain Doppler echocardiographic features of familial amyloid polyneuropathy (FAP) and light-chain amyloidosis. Am J Cardiol. 2005;95:538-540.
98. Falk RH. The neglected entity of familial cardiac amyloidosis in African Americans. Ethnicity Dis. 2002;12:141-143.
99. Jacobson DR, Pastore RD, Yaghoubian R, Kane I, Gallo G, Buck FS, Buxbaum JN. Variant-sequence transthyretin (isoleucine 122) in late-onset cardiac amyloidosis in black Americans. N Engl J Med. 1997;336:466-473.
100. Jacobson DR, Pastore R, Pool S, Malendowicz S, Kane I, Shivji A, Embury SH, Ballas SK, Buxbaum JN. Revised transthyretin Ile 122 allele frequency in African-Americans. Hum Genet. 1996;98:236-238.
101. Berg A, Falk RH, Connors LH, Theberge R, Skare J, Murakami A, Skinner M. Transthyretin Ile-122 in a series of black patients with amyloidosis. In: Kyle RA, Gertz MA, eds. Amyloid and the Amyloidoses: VIIIIth International Symposium on Amyloidosis. Rochester, Minn: Parthenon Publishing Group; 1998.
102. Hamilton JA. Benson MD. Transthyretin: a review from a structural perspective. Cell Mol Life Sci. 2001;58:1491-1521.
103. Suhr OB, Herlenius G, Friman S, Ericzon BG. Liver transplantation for hereditary transthyretin amyloidosis. Liver Transplant. 2000;6:263-276.
104. Jonsen E, Suhr OB, Tashima K, Athlin E. Early liver transplantation is essential for familial amyloidotic polyneuropathy patients' quality of life. Amyloid. 2001;8:52-57.
105. Monteiro E, Perdigoto R, Furtado AL. Liver transplantation for familial amyloid polyneuropathy. Hepato-Gastroenterol. 1998;45:1375-1380.
106. Dubrey SW, Davidoff R, Skinner M, Bergethon P, Lewis D, Falk RH. Progression of ventricular wall thickening after liver transplantation for familial amyloidosis. Transplantation. 1997;64:74-80.
107. Stangou AJ, Hawkins PN, Heaton ND, Rela M, Monaghan M, Nihoyannopoulos P, O'Grady J, Pepys MB, Williams R. Progressive cardiac amyloidosis following liver transplantation for familial amyloid polyneuropathy: implications for amyloid fibrillogenesis. Transplantation. 1998;66:229-233.
108. Ruygrok PN, Gane EJ, McCall JL, Chen XZ, Haydock DA, Munn SR. Combined heart and liver transplantation for familial amyloidosis. Intern Med J. 2001;31:66-67.
109. Kelly JW. Attacking amyloid. N Engl J Med. 2005;352:722-723.
110. Miller SR, Sekijima Y, Kelly JW. Native state stabilization by NSAIDs inhibits transthyretin amyloidogenesis from the most common familial disease variants. Lab Invest. 2004;84:545-552.
111. Lachmann HJ, Hawkins PN. Novel pharmacological strategies in amyloidosis. Nephron Clin Pract. 2003;94:c85-c88.
112. Obici L, Bellotti V, Mangione P, Stoppini M, Arbustini E, Verga L, Zorzoli I, Anesi E, Zanotti G, Campana C, Vigano M, Merlini G. The new apolipoprotein A-I variant leu(174)→Ser causes hereditary cardiac amyloidosis, and the amyloid fibrils are constituted by the 93-residue N-terminal polypeptide. Am J Pathol. 1999;155:695-702.
113. Westermark P, Sletten K, Johansson B, Cornwell GG. Fibril in senile systemic amyloidosis is derived from normal transthyretin. Proc Natl Acad Sci USA. 1990;87:2843-2845.
114. Ng B, Connors LH, Davidoff R, Skinner M, Falk RH. Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated (AL) amyloidosis. Arch Intern Med. 2005;165:1425-1429.
115. Cornwell GG, Murdoch WL, Kyle RA, Westermark P, Pitkanen P. Frequency and distribution of senile cardiovascular amyloid: a clinicopathologic correlation. Am J Med. 1983;75:618-623.
116. Cornwell GGd, Westermark P. Senile amyloidosis: a protean manifestation of the aging process. J Clin Pathol. 1980;33:1146-1152.
117. Anagnostopoulos A, Evangelopoulou A, Sotou D, Gika D, Mitsibounas D, Dimopouios MA. Incidence and evolution of monoclonal gammopathy of undetermined significance (MGUS) in Greece. Ann Hematol. 2002;81:357-361.
118. Kyle RA, Spittell PC, Gertz MA, Li CY, Edwards WD, Olson LJ, Thibodeau SN. The premortem recognition of systemic senile amyloidosis with cardiac involvement. Am J Med. 1996;101:395-400.
119. Wright JR, Calkins E. Amyloid in the aged heart: frequency and clinical significance. J Am Geriatr Socy. 1975;23:97-103.
120. Pucci A, Wharton J, Arbustini E, Grasso M, Diegoli M, Needleman P. Vigano M, Polak JM. Atrial amyloid deposits in the failing human heart display both atrial and brain natriuretic peptide-like immunoreactivity. J Pathol. 1991;165:235-241.
121. Goette A, Rocken C. Atrial amyloidosis and atrial fibrillation: a gender-dependent "arrhythmogenic substrate"? Eur Mean J. 2004;25:1185-1186.
122. Leone O, Boriani G, Chiappini B, Pacini D, Cenacchi G, Martin Suarez S, Rapezzi C, Bacchi Reggiani ML, Marinelli G. Amyloid deposition as a cause of atrial remodelling in persistent valvular atrial fibrillation. Eur Heart J. 2004;25:1237-1241.
123. Rocken C, Peters B, Juenemann G, Saeger W, Klein HU, Huth C, Roessner A, Goette A. Atrial amyloidosis: an arrhythmogenic substrate for persistent atrial fibrillation. Circulation. 2002;106:2091-2097.