Abstract
Cardiac involvement in patients with amyloidosis is common. It produces significant clinical symptoms in about 40% of patients with AL amyloidosis. A significant proportion of patients with familial amyloidosis have clinical involvement of the heart, and heart failure is almost always the presenting feature of senile systemic amyloidosis. The severity of the cardiac symptoms and the response to treatment vary depending upon the type of amyloid deposition. In this chapter, the different types of cardiac amyloidosis are reviewed and the diagnostic workup and appropriate therapies are addressed.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Dubrey SW, Cha K, Anderson J, Chamarthi B, Reisinger J, Skinner M, et al. The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. QJM. 1998 Feb;91(2):141–57.
Carroll JD, Gaasch WH, McAdam KP. Amyloid cardiomyopathy: characterization by a distinctive voltage/mass relation. Am J Cardiol. 1982;49(1):9–13.
Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, et al. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 2005 Jan 18;111(2):186–93.
Migrino RQ, Christenson R, Szabo A, Bright M, Truran S, Hari P. Prognostic implication of late gadolinium enhancement on cardiac MRI in light chain (AL) amyloidosis on long term follow up. BMC Med Phys. 2009 May 5;9(1):5.
Ruberg FL, Appelbaum E, Davidoff R, Ozonoff A, Kissinger KV, Harrigan C, et al. Diagnostic and prognostic utility of cardiovascular magnetic resonance imaging in light-chain cardiac amyloidosis. Am J Cardiol. 2009 Feb 15;103(4):544–9.
Maceira AM, Prasad SK, Hawkins PN, Roughton M, Pennell DJ. Cardiovascular magnetic resonance and prognosis in cardiac amyloidosis. J Cardiovasc Magn Reson. 2008;10(1):54.
Kribben A, Witzke O, Hillen U, Barkhausen J, Daul AE, Erbel R. Nephrogenic systemic fibrosis: pathogenesis, diagnosis, and therapy. J Am Coll Cardiol. 2009 May 5;53(18):1621–8.
Hazenberg BP, van Rijswijk MH, Piers DA, Lub-de Hooge MN, Vellenga E, Haagsma EB, et al. Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis. Am J Med. 2006 Apr;119(4):355.e15–24.
Glaudemans AW, Slart RH, Zeebregts CJ, Veltman NC, Tio RA, Hazenberg BP, et al. Nuclear imaging in cardiac amyloidosis. Eur J Nucl Med Mol Imaging. 2009 Jan 21;36:702–14.
Pellikka PA, Holmes DR Jr., Edwards WD, Nishimura RA, Tajik AJ, Kyle RA. Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement. Arch Intern Med. 1988;148(3):662–6.
Reisinger J, Dubrey SW, Lavalley M, Skinner M, Falk RH. Electrophysiologic abnormalities in AL (primary) amyloidosis with cardiac involvement. J Am Coll Cardiol. 1997;30(4):1046–51.
Dubrey S, Pollak A, Skinner M, Falk RH. Atrial thrombi occurring during sinus rhythm in cardiac amyloidosis: evidence for atrial electromechanical dissociation [see comments]. Br Heart J. 1995;74(5):541–4.
Feng D, Syed IS, Martinez M, Oh JK, Jaffe AS, Grogan M, et al. Intracardiac thrombosis and anticoagulation therapy in cardiac amyloidosis. Circulation. 2009 May 12;119(18):2490–7.
Feng D, Edwards WD, Oh JK, Chandrasekaran K, Grogan M, Martinez MW, et al. Intracardiac thrombosis and embolism in patients with cardiac amyloidosis. Circulation 2007 Nov 20;116(21):2420–6.
Zubkov AY, Rabinstein AA, Dispenzieri A, Wijdicks EF. Primary systemic amyloidosis with ischemic stroke as a presenting complication. Neurology 2007 Sep 11;69(11): 1136–41.
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(1):36–44.
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(9):1067–71.
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(19):2446–52.
Ogiwara F, Koyama J, Ikeda S, Kinoshita O, Falk RH. Comparison of the strain Doppler echocardiographic features of familial amyloid polyneuropathy (FAP) and light-chain amyloidosis. Am J Cardiol. 2005 Feb 15;95(4):538–40.
Sharma PP, Payvar S, Litovsky SH. Histomorphometric analysis of intramyocardial vessels in primary and senile amyloidosis: epicardium versus endocardium. Cardiovasc Pathol. 2008 Mar–Apr;17(2):65–71.
Palladini G, Lavatelli F, Russo P, Perlini S, Perfetti V, Bosoni T, et al. Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL. Blood 2006 May 15;107(10):3854–8.
Brenner DA, Jain M, Pimentel DR, Wang B, Connors LH, Skinner M, et al. Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress. Circ Res. 2004;94(8):1008–10.
Liao RL, Jain M, Teller P, Connors LH, Ngoy S, Skinner M, et al. Infusion of light chains from patients with cardiac amyloidosis causes diastolic dysfunction in isolated mouse hearts. Circulation 2001;104(14):1594–7.
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(1):74–82.
Collins AB, Smith RN, Stone JR. Classification of amyloid deposits in diagnostic cardiac specimens by immunofluorescence. Cardiovasc Pathol. 2008 Jul 10;18:205–16.
Benson MD, Breall J, Cummings OW, Liepnieks JJ. Biochemical characterisation of amyloid by endomyocardial biopsy. Amyloid 2009 Mar;16(1):9–14.
Kyle RA, Gertz MA, Greipp PR, Witzig TE, Lust JA, Lacy MQ, et al. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med. 1997 Apr 24;336(17):1202–7.
Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995 Jan;32(1):45–59.
Palladini G, Campana C, Klersy C, Balduini A, Vadacca G, Perfetti V, et al. Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis. Circulation 2003;107(19):2440–5.
Nordlinger M, Magnani B, Skinner M, Falk RH. Is elevated plasma B-natriuretic peptide in amyloidosis simply a function of the presence of heart failure?. Am J Cardiol. 2005;96(7):982–4.
Takemura G, Takatsu Y, Doyama K, Itoh H, Saito Y, Koshiji M, et al. Expression of atrial and brain natriuretic peptides and their genes in hearts of patients with cardiac amyloidosis. J Am Coll Cardiol. 1998;31(4):754–65.
Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 2004;22(18):3751–7.
Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, et al. 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(6):1881–7.
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(4):535–7.
Velazquez-Cecena JL, Lubell DL, Nagajothi N, Al-Masri H, Siddiqui M, Khosla S. Syncope from dynamic left ventricular outflow tract obstruction simulating hypertrophic cardiomyopathy in a patient with primary AL-type amyloid heart disease. Tex Heart Inst J. 2009;36(1):50–4.
Dinwoodey DL, Skinner M, Maron MS, Davidoff R, Ruberg FL. Light-chain amyloidosis with echocardiographic features of hypertrophic cardiomyopathy. Am J Cardiol. 2008 Mar 1;101(5):674–6.
Pollak A, Falk RH. Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis. Chest 1993;104(2):618–20.
Al Suwaidi J, Velianou JL, Gertz MA, Cannon RO, Higano ST, Holmes DR, et al. Systemic amyloidosis presenting with angina pectoris. Ann Intern Med. 1999;131(11): 838–41.
Hongo M, Yamamoto H, Kohda T, Takeda M, Kinoshita O, Uchikawa S, et al. Comparison of electrocardiographic findings in patients with AL (primary) amyloidosis and in familial amyloid polyneuropathy and anginal pain and their relation to histopathologic findings. Am J Cardiol. 2000;85(7):849–53.
Narang R, Chopra P, Wasir HS. Cardiac amyloidosis presenting as ischemic heart disease. A case report and review of literature. Cardiology 1993;82(4):294–300.
Mesquita T, Chorao M, Soares I, Mello e Silva A, Abecasis P. Primary amyloidosis as a cause of microvascular angina and intermittent claudication. Rev Port Cardiol. 2005 Dec;24(12):1521–31.
Churchill CH, Abril A, Krishna M, Callman ML, Ginsburg WW. Jaw claudication in primary amyloidosis: unusual presentation of a rare disease. J Rheumatol. 2003;30(10):2283–6.
Schneider BF, Normansell D, Ayers CR, Hess CE. Intermittent claudication as the presenting symptom in primary amyloidosis. Acta Haematologica 1993;90(2):106–7.
Yokota T, Okabayashi H, Ishihara T, Kawano H, Wakabayashi F, Miyamoto AT, et al. Immunohistochemical and pathological characteristics of dystrophic amyloid in surgically excised cardiac valves. Pathol Int. 1994;44(3):182–5.
Kristen AV, Dengler TJ, Hegenbart U, Schonland SO, Goldschmidt H, Sack FU, et al. Prophylactic implantation of cardioverter-defibrillator in patients with severe cardiac amyloidosis and high risk for sudden cardiac death. Heart Rhythm 2008 Feb;5(2): 235–40.
Dhoble A, Khasnis A, Olomu A, Thakur R. Cardiac amyloidosis treated with an implantable cardioverter defibrillator and subcutaneous array lead system: report of a case and literature review. Clin Cardiol. 2009 May 19;32:E63–5.
Comenzo RL, Vosburgh E, Falk RH, Sanchorawala V, Reisinger J, Dubrey S, et al. Dose-intensive melphalan with blood stem-cell support for the treatment of AL (amyloid light-chain) amyloidosis: survival and responses in 25 patients. Blood 1998 May 15;91(10):3662–70.
Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, et al. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med. 2004 Jan 20;140(2):85–93.
Jaccard A, Moreau P, Leblond V, Leleu X, Benboubker L, Hermine O, et al. High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N Engl J Med. 2007 Sep 13;357(11):1083–93.
Palladini G, Russo P, Lavatelli F, Nuvolone M, Albertini R, Bosoni T, et al. Treatment of patients with advanced cardiac AL amyloidosis with oral melphalan, dexamethasone, and thalidomide. Ann Hematol. 2009 Apr;88(4):347–50.
Hosenpud JD, DeMarco T, Frazier OH, Griffith BP, Uretsky BF, Menkis AH, et al. 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 Suppl 5:III338–43.
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(4): 346–50.
Dubrey S, Simms RW, Skinner M, Falk RH. Recurrence of primary (AL) amyloidosis in a transplanted heart with four-year survival. Am J Cardiol. 1995;76(10):739–41.
Dubrey S, Burke M, Hawkins P, Banner N. Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant. 2004;23(10): 1142–53.
Kpodonu J, Massad MG, Caines A, Geha AS. Outcome of heart transplantation in patients with amyloid cardiomyopathy. J Heart Lung Transplant. 2005 Nov;24(11): 1763–5.
Roig E, Almenar L, Gonzalez-Vilchez F, Rabago G, Delgado J, Gomez-Bueno M, et al. Outcomes of heart transplantation for cardiac amyloidosis: subanalysis of the Spanish registry for heart transplantation. Am J Transplant. 2009 May 6;9:1414–9.
Gillmore JD, Goodman HJ, Lachmann HJ, Offer M, Wechalekar AD, Joshi J, et al. Sequential heart and autologous stem cell transplantation for systemic AL amyloidosis. Blood. 2006 Feb 1;107(3):1227–9.
Maurer MS, Raina A, Hesdorffer C, Bijou R, Colombo P, Deng M, et al. Cardiac transplantation using extended-donor criteria organs for systemic amyloidosis complicated by heart failure. Transplantation 2007 Mar 15;83(5):539–45.
Chen JM, Russo MJ, Hammond KM, Mancini DM, Kherani AR, Fal JM, et al. Alternate waiting list strategies for heart transplantation maximize donor organ utilization. Ann Thorac Surg. 2005 Jul;80(1):224–8.
Lacy MQ, Dispenzieri A, Hayman SR, Kumar S, Kyle RA, Rajkumar SV, et al. Autologous stem cell transplant after heart transplant for light chain (Al) amyloid cardiomyopathy. J Heart Lung Transplant. 2008 Aug;27(8):823–9.
Mignot A, Varnous S, Redonnet M, Jaccard A, Epailly E, Vermes E, et al. Heart transplantation in systemic (AL) amyloidosis: a retrospective study of eight French patients. Arch Cardiovasc Dis. 2008 Sep;101(9):523–32.
Sack FU, Kristen A, Goldschmidt H, Schnabel PA, Dengler T, Koch A, et al. 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 Feb;33(2):257–62.
Mignot A, Bridoux F, Thierry A, Varnous S, Pujo M, Delcourt A, et al. Successful heart transplantation following melphalan plus dexamethasone therapy in systemic AL amyloidosis. Haematologica 2008 Mar;93(3):e32–5.
Audard V, Matignon M, Weiss L, Remy P, Pardon A, Haioun C, et al. Successful long-term outcome of the first combined heart and kidney transplant in a patient with systemic Al amyloidosis. Am J Transplant. 2009 Jan;9(1):236–40.
Hess EP, White RD. Out-of-hospital cardiac arrest in patients with cardiac amyloidosis: presenting rhythms, management and outcomes in four patients. Resuscitation 2004;60(1):105–11.
Connors LH, Lim A, Prokaeva T, Roskens VA, Costello CE. Tabulation of human transthyretin (TTR) variants, 2003. Amyloid 2003 Sep;10(3):160–84.
Westermark P, Sletten K, Johansson B, Cornwell GG III.. Fibril in senile systemic amyloidosis is derived from normal transthyretin. Proc Natl Acad Sci USA 1990 Apr;87(7):2843–5.
Yazaki M, Tokuda T, Nakamura A, Higashikata T, Koyama J, Higuchi K, et al. Cardiac amyloid in patients with familial amyloid polyneuropathy consists of abundant wild-type transthyretin. Biochem Biophys Res Commun. 2000 Aug 11;274(3):702–6.
Jacobson DR, Pastore RD, Yaghoubian R, Kane I, Gallo G, Buck FS, et al. Variant-sequence transthyretin (isoleucine 122) in late-onset cardiac amyloidosis in black Americans. New Engl J Med. 1997 Feb 13;336(7):466–73.
Gillmore JD, Booth DR, Pepys MB, Hawkins PN. Hereditary cardiac amyloidosis associated with the transthyretin Ile122 mutation in a white man. Heart (Br Card Soc). 1999 Sep;82(3):e2.
Eriksson M, Schonland S, Yumlu S, Hegenbart U, von Hutten H, Gioeva Z, et al. Hereditary apolipoprotein AI-associated amyloidosis in surgical pathology specimens: identification of three novel mutations in the APOA1 gene. J Mol Diagn. 2009 May;11(3): 257–62.
Westermark P, Mucchiano G, Marthin T, Johnson KH, Sletten K. Apolipoprotein A1-derived amyloid in human aortic atherosclerotic plaques. Am J Pathol. 1995 Nov;147(5):1186–92.
Gillmore JD, Stangou AJ, Tennent GA, Booth DR, O’Grady J, Rela M, et al. Clinical and biochemical outcome of hepatorenal transplantation for hereditary systemic amyloidosis associated with apolipoprotein AI Gly26Arg. Transplantation 2001;71(7): 986–92.
Gillmore JD, Lachmann HJ, Rowczenio D, Gilbertson JA, Zeng CH, Liu ZH, et al. Diagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen A alpha-chain amyloidosis. J Am Soc Nephrol. 2009 Feb;20(2):444–51.
Mourad G, Delabre JP, Garrigue V. Cardiac amyloidosis with the E526V mutation of the fibrinogen A alpha-chain. N Engl J Med. 2008 Dec 25;359(26):2847–8.
Kiuru S, Matikainen E, Kupari M, Haltia M, Palo J. Autonomic nervous system and cardiac involvement in familial amyloidosis, Finnish type (FAF). J Neurol Sci. 1994 Oct;126(1):40–8.
Chastan N, Baert-Desurmont S, Saugier-Veber P, Derumeaux G, Cabot A, Frebourg T, et al. Cardiac conduction alterations in a French family with amyloidosis of the Finnish type with the p.Asp187Tyr mutation in the GSN gene. Muscle Nerve 2006 Jan;33(1):113–9.
Zeldenrust S, Gertz M, Uemichi T, Bjornsson J, Wiesner R, Schwab T, et al. Orthotopic liver transplantation for hereditary fibrinogen amyloidosis. Transplantation 2003;75(4):560–1.
Holmgren G, Ericzon BG, Groth CG, Steen L, Suhr O, Andersen O, et al. Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis. Lancet 1993 May 1;341(8853):1113–6.
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 Jul 15;64(1):74–80.
Stangou AJ, Hawkins PN, Heaton ND, Rela M, Monaghan M, Nihoyannopoulos P, et al. Progressive cardiac amyloidosis following liver transplantation for familial amyloid polyneuropathy: implications for amyloid fibrillogenesis. Transplantation 1998 Jul 27;66(2):229–33.
Pomfret EA, Lewis WD, Jenkins RL, Bergethon P, Dubrey SW, Reisinger J, et al. Effect of orthotopic liver transplantation on the progression of familial amyloidotic polyneuropathy. Transplantation 1998 Apr 15;65(7):918–25.
Yazaki M, Mitsuhashi S, Tokuda T, Kametani F, Takei YI, Koyama J, et al. Progressive wild-type transthyretin deposition after liver transplantation preferentially occurs onto myocardium in FAP patients. Am J Transplant. 2007 Jan;7(1):235–42.
Liepnieks JJ, Benson MD. Progression of cardiac amyloid deposition in hereditary transthyretin amyloidosis patients after liver transplantation. Amyloid 2007 Dec;14(4):277–82.
Ihse E, Stangou AJ, Heaton ND, O’Grady J, Ybo A, Hellman U, et al. Proportion between wild-type and mutant protein in truncated compared to full-length ATTR: an analysis on transplanted transthyretin T60A amyloidosis patients. Biochem Biophys Res Commun. 2009 Feb 20;379(4):846–50.
Arpesella G, Chiappini B, Marinelli G, Mikus PM, Dozza F, Pierangeli A, et al. Combined heart and liver transplantation for familial amyloidotic polyneuropathy. J Thorac Cardiovasc Surg. 2003 May;125(5):1165–6.
Grazi GL, Cescon M, Salvi F, Ercolani G, Ravaioli M, Arpesella G, et al. Combined heart and liver transplantation for familial amyloidotic neuropathy: considerations from the hepatic point of view. Liver Transplant. 2003 Sep;9(9):986–92.
Zeldenrust S, Gertz M, Uemichi T, Bjornsson J, Wiesner R, Schwab T, et al. Orthotopic liver transplantation for hereditary fibrinogen amyloidosis. Transplantation 2003;75:560–1.
Dubrey SW, Burke MM, Hawkins PN, Banner NR. Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant. 2004 Oct;23(10):1142–53.
Gillmore JD, Stangou AJ, Tennent GA, Booth DR, O’Grady J, Rela M, et al. Clinical and biochemical outcome of hepatorenal transplantation for hereditary systemic amyloidosis associated with apolipoprotein AI Gly26Arg. Transplantation 2001 Apr 15;71(7): 986–92.
Inomata Y, Zeledon ME, Asonuma K, Okajima H, Takeichi T, Ishiko T, et al. Whole-liver graft without the retrohepatic inferior vena cava for sequential (domino) living donor liver transplantation. Am J Transplant. 2007 Jun;7(6):1629–32.
Singer R, Mehrabi A, Schemmer P, Kashfi A, Hegenbart U, Goldschmidt H, et al. Indications for liver transplantation in patients with amyloidosis: a single-center experience with 11 cases. Transplantation 2005 Sep 27;80 Suppl 1:S156–9.
Goto T, Yamashita T, Ueda M, Ohshima S, Yoneyama K, Nakamura M, et al. Iatrogenic amyloid neuropathy in a Japanese patient after sequential liver transplantation. Am J Transplant. 2006 Oct;6(10):2512–5.
Stangou AJ, Heaton ND, Hawkins PN. Transmission of systemic transthyretin amyloidosis by means of domino liver transplantation. N Engl J Med. 2005 Jun 2;352(22):2356.
Cardoso I, Merlini G, Saraiva MJ. 4'-iodo-4'-deoxydoxorubicin and tetracyclines disrupt transthyretin amyloid fibrils in vitro producing noncytotoxic species: screening for TTR fibril disrupters. FASEB J. 2003 May;17(8):803–9.
Palha JA, Ballinari D, Amboldi N, Cardoso I, Fernandes R, Bellotti V, et al.. 4'-Iodo-4'-deoxydoxorubicin disrupts the fibrillar structure of transthyretin amyloid. Am J Pathol. 2000 Jun;156(6):1919–25.
Peterson SA, Klabunde T, Lashuel HA, Purkey H, Sacchettini JC, Kelly JW. Inhibiting transthyretin conformational changes that lead to amyloid fibril formation. Proc Natl Acad Sci USA 1998 Oct 27;95(22):12956–60.
Saraiva MJ. Transthyretin mutations in hyperthyroxinemia and amyloid diseases. Human Mutation 2001 Jun;17(6):493–503.
Oza VB, Smith C, Raman P, Koepf EK, Lashuel HA, Petrassi HM, et al. Synthesis, structure, and activity of diclofenac analogues as transthyretin amyloid fibril formation inhibitors. J Med Chem. 2002 Jan 17;45(2):321–32.
Almeida MR, Gales L, Damas AM, Cardoso I, Saraiva MJ. Small transthyretin (TTR) ligands as possible therapeutic agents in TTR amyloidoses. Curr Drug targets. CNS Neurol Disord. 2005 Oct;4(5):587–96.
Gillmore JD, Hawkins PN. Drug Insight: emerging therapies for amyloidosis. Nat Clin Pract Nephrol. 2006 May;2(5):263–70.
Coelho T, Coelho T, Maia L, da Silva AM, et al. A landmark clinical trial of a novel small molecule transthyretin (TTR) stabilizer, Fx-1006A, in patients with TTR amyloid polyneuropathy: a phase II/III, randomized, double-blind, placebo-controlled study. Neurology 2009;72 Suppl. 3:A205.
Coelho T, Waddington-Cruz M, Plante-Bordeneuve V, et al. Correlation of clinical outcomes and disease burden in patients with transthyretin (TTR) amyloid polyneuropathy: study Fx-005, a landmark clinical trail of Fx-1006A, a novel small molecule TTR stabilizer. J Neurol. 2008;255 Suppl 1:78.
Sekijima Y, Dendle MA, Kelly JW. Orally administered diflunisal stabilizes transthyretin against dissociation required for amyloidogenesis. Amyloid 2006 Dec;13(4):236–49.
Tojo K, Sekijima Y, Kelly JW, Ikeda SI. Diflunisal stabilizes familial amyloid polyneuropathy-associated transthyretin variant tetramers in serum against dissociation required for amyloidogenesis. Neurosci Res. 2006 Oct 5;56:441–9.
Mairal T, Nieto J, Pinto M, Almeida MR, Gales L, Ballesteros A, et al. Iodine atoms: a new molecular feature for the design of potent transthyretin fibrillogenesis inhibitors. PLoS ONE 2009;4(1):e4124.
Miller SR, Sekijima Y, Kelly JW. Native state stabilization by NSAIDs inhibits transthyretin amyloidogenesis from the most common familial disease variants. Lab Invest. 2004 May;84(5):545–52.
Miroy GJ, Lai Z, Lashuel HA, Peterson SA, Strang C, Kelly JW. Inhibiting transthyretin amyloid fibril formation via protein stabilization. Proc Natl Acad Sci USA 1996 Dec 24;93(26):15051–6.
Bergstrom J, Gustavsson A, Hellman U, Sletten K, Murphy CL, Weiss DT, et al. Amyloid deposits in transthyretin-derived amyloidosis: cleaved transthyretin is associated with distinct amyloid morphology. J Pathol. 2005 Jun;206(2):224–32.
Kyle RA, Spittell PC, Gertz MA, Li CY, Edwards WD, Olson LJ, et al. The premortem recognition of systemic senile amyloidosis with cardiac involvement. Am J Med. 1996 Oct;101(4):395–400.
Ng B, Connors LH, Davidoff R, Skinner M, Falk RH. Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis. Arch Intern Med. 2005 Jun 27;165(12):1425–9.
Fuchs U, Zittermann A, Suhr O, Holmgren G, Tenderich G, Minami K, et al. Heart transplantation in a 68-year-old patient with senile systemic amyloidosis. Am J Transplant. 2005 May;5(5):1159–62.
Dubrey SW, Cha K, Simms RW, Skinner M, Falk RH. Electrocardiography and Doppler echocardiography in secondary (AA) amyloidosis. Am J Cardiol. 1996 Feb 1;77(4): 313–5.
Kaye GC, Butler MG, d’Ardenne AJ, Edmondson SJ, Camm AJ, Slavin G. Isolated atrial amyloid contains atrial natriuretic peptide: a report of six cases. Br Heart J. 1986 Oct;56(4):317–20.
Kawamura S, Takahashi M, Ishihara T, Uchino F. Incidence and distribution of isolated atrial amyloid: histologic and immunohistochemical studies of 100 aging hearts. Pathol Int. 1995 May;45(5):335–42.
Steiner I. The prevalence of isolated atrial amyloid. J Pathol. 1987 Dec;153(4):395–8.
Ariyarajah V, Steiner I, Hajkova P, Khadem A, Kvasnicka J, Apiyasawat S, et al. The association of atrial tachyarrhythmias with isolated atrial amyloid disease: preliminary observations in autopsied heart specimens. Cardiology 2009;113(2):132–7.
Goette A, Rocken C. Atrial amyloidosis and atrial fibrillation: a gender-dependent “arrhythmogenic substrate”? Eur Heart J. 2004 Jul;25(14):1185–6.
Leone O, Boriani G, Chiappini B, Pacini D, Cenacchi G, Martin Suarez S, et al. Amyloid deposition as a cause of atrial remodelling in persistent valvular atrial fibrillation. Eur Heart J. 2004 Jul;25(14):1237–41.
Steiner I, Hajkova P. Patterns of isolated atrial amyloid: a study of 100 hearts on autopsy. Cardiovasc Pathol. 2006 Sep–Oct;15(5):287–90.
Rocken C, Peters B, Juenemann G, Saeger W, Klein HU, Huth C, et al. Atrial amyloidosis: an arrhythmogenic substrate for persistent atrial fibrillation. Circulation 2002 Oct 15;106(16):2091–7.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2010 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Falk, R.H., Dubrey, S.W. (2010). Amyloid Heart Disease. In: Gertz, M., Rajkumar, S. (eds) Amyloidosis. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-631-3_8
Download citation
DOI: https://doi.org/10.1007/978-1-60761-631-3_8
Published:
Publisher Name: Humana Press, Totowa, NJ
Print ISBN: 978-1-60761-630-6
Online ISBN: 978-1-60761-631-3
eBook Packages: MedicineMedicine (R0)