Skip to main content
Top
Published in: Heart and Vessels 9/2022

Open Access 11-03-2022 | Echocardiography | Original Article

Disease progression in cardiac morphology and function in heart failure: ATTR cardiac amyloidosis versus hypertensive left ventricular hypertrophy

Authors: M. Y. Henein, B. Pilebro, Per Lindqvist

Published in: Heart and Vessels | Issue 9/2022

Login to get access

Abstract

Background

Transthyretin cardiac amyloidosis (ATTR-CA) is today more frequently recognized but the rate of progression of cardiac dysfunction is not well established. The aim of this study is to investigate the nature of cardiac structure and function changes, over time, in a retrospective cohort of ATTR-CA patients.

Methods

Fifty-one patients with ATTR-CA (mean age 78 ± 7 years, 30 females) were compared with 20 patients with heart failure but no amyloidosis (HFnCA) (mean age 76 ± 7 years, 5 females), all with septal thickness >  = 14 mm. All patients underwent DPD scintigraphy and an echocardiogram (Echo 2) which was compared with a previous echocardiographic examination (Echo 1), performed at least 3 years before.

Results

Over the follow-up period, the interventricular septal thickness (IVST) and relative wall thickness (RWT) in ATTR-CA increased from 16 (4) to 18 (5) mm and from 0.51 (0.17) to 0.62 (0.21) respectively, p < 0.001 for both, by a mean increase of 0.4 mm/year and 0.03 mm/year, (p = 0.009 and p = 0.002 compared with HFnCA), respectively. RWT > 0.45 (AUC = 0.77) and RELAPS > 2.0 (AUC 0.86) both predicted positive DPD diagnosis for ATTR-CA.

Conclusion

In ATTR-CA patients, the overtime-increase in RWT and IVST was worse than that seen in patients with heart failure but no cardiac amyloidosis. Also, RWT and relative apical sparing predicted diagnosis of ATTR-CA, thus could strengthen the use of follow-up echocardiographic findings as red flag for the diagnosis of ATTR-CA.
Literature
1.
go back to reference Maleszewski JJ (2015) Cardiac amyloidosis: pathology, nomenclature, and typing. Cardiovasc Pathol 24(6):343–350CrossRef Maleszewski JJ (2015) Cardiac amyloidosis: pathology, nomenclature, and typing. Cardiovasc Pathol 24(6):343–350CrossRef
2.
go back to reference Falk RH (2005) Diagnosis and management of the cardiac amyloidoses. Circulation 112(13):2047–2060CrossRef Falk RH (2005) Diagnosis and management of the cardiac amyloidoses. Circulation 112(13):2047–2060CrossRef
3.
go back to reference Falk RH, Dubrey SW (2010) Amyloid heart disease. Prog Cardiovasc Dis 52(4):347–361CrossRef Falk RH, Dubrey SW (2010) Amyloid heart disease. Prog Cardiovasc Dis 52(4):347–361CrossRef
4.
go back to reference Gonzalez-Lopez E, Gallego-Delgado M, Guzzo-Merello G, de Haro-Del Moral FJ, Cobo-Marcos M, Robles C, Bornstein B, Salas C, Lara-Pezzi E, Alonso-Pulpon L, Garcia-Pavia P (2015) Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J 36(38):2585–2594CrossRef Gonzalez-Lopez E, Gallego-Delgado M, Guzzo-Merello G, de Haro-Del Moral FJ, Cobo-Marcos M, Robles C, Bornstein B, Salas C, Lara-Pezzi E, Alonso-Pulpon L, Garcia-Pavia P (2015) Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J 36(38):2585–2594CrossRef
5.
go back to reference Tanskanen M, Peuralinna T, Polvikoski T, Notkola IL, Sulkava R, Hardy J, Singleton A, Kiuru-Enari S, Paetau A, Tienari PJ, Myllykangas L (2008) Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy study. Ann Med 40(3):232–239CrossRef Tanskanen M, Peuralinna T, Polvikoski T, Notkola IL, Sulkava R, Hardy J, Singleton A, Kiuru-Enari S, Paetau A, Tienari PJ, Myllykangas L (2008) Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy study. Ann Med 40(3):232–239CrossRef
6.
go back to reference Mohammed SF, Mirzoyev SA, Edwards WD, Dogan A, Grogan DR, Dunlay SM, Roger VL, Gertz MA, Dispenzieri A, Zeldenrust SR, Redfield MM (2014) Left ventricular amyloid deposition in patients with heart failure and preserved ejection fraction. JACC Heart Fail 2(2):113–122CrossRef Mohammed SF, Mirzoyev SA, Edwards WD, Dogan A, Grogan DR, Dunlay SM, Roger VL, Gertz MA, Dispenzieri A, Zeldenrust SR, Redfield MM (2014) Left ventricular amyloid deposition in patients with heart failure and preserved ejection fraction. JACC Heart Fail 2(2):113–122CrossRef
7.
go back to reference Hafeez AS, Bavry AA (2020) Diagnosis of transthyretin amyloid cardiomyopathy. Cardiol Ther 9(1):85–95CrossRef Hafeez AS, Bavry AA (2020) Diagnosis of transthyretin amyloid cardiomyopathy. Cardiol Ther 9(1):85–95CrossRef
8.
go back to reference Gillmore JD, Maurer MS, Falk RH, Merlini G, Damy T, Dispenzieri A, Wechalekar AD, Berk JL, Quarta CC, Grogan M, Lachmann HJ, Bokhari S, Castano A, Dorbala S, Johnson GB, Glaudemans AW, Rezk T, Fontana M, Palladini G, Milani P, Guidalotti PL, Flatman K, Lane T, Vonberg FW, Whelan CJ, Moon JC, Ruberg FL, Miller EJ, Hutt DF, Hazenberg BP, Rapezzi C, Hawkins PN (2016) Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation 133(24):2404–2412CrossRef Gillmore JD, Maurer MS, Falk RH, Merlini G, Damy T, Dispenzieri A, Wechalekar AD, Berk JL, Quarta CC, Grogan M, Lachmann HJ, Bokhari S, Castano A, Dorbala S, Johnson GB, Glaudemans AW, Rezk T, Fontana M, Palladini G, Milani P, Guidalotti PL, Flatman K, Lane T, Vonberg FW, Whelan CJ, Moon JC, Ruberg FL, Miller EJ, Hutt DF, Hazenberg BP, Rapezzi C, Hawkins PN (2016) Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation 133(24):2404–2412CrossRef
9.
go back to reference Garcia-Pavia P, Rapezzi C, Adler Y, Arad M, Basso C, Brucato A, Burazor I, Caforio ALP, Damy T, Eriksson U, Fontana M, Gillmore JD, Gonzalez-Lopez E, Grogan M, Heymans S, Imazio M, Kindermann I, Kristen AV, Maurer MS, Merlini G, Pantazis A, Pankuweit S, Rigopoulos AG, Linhart A (2021) Diagnosis and treatment of cardiac amyloidosis. A position statement of the European society of cardiology working group on myocardial and pericardial diseases. Eur J of Heart Fail. 42(16):1554–1568CrossRef Garcia-Pavia P, Rapezzi C, Adler Y, Arad M, Basso C, Brucato A, Burazor I, Caforio ALP, Damy T, Eriksson U, Fontana M, Gillmore JD, Gonzalez-Lopez E, Grogan M, Heymans S, Imazio M, Kindermann I, Kristen AV, Maurer MS, Merlini G, Pantazis A, Pankuweit S, Rigopoulos AG, Linhart A (2021) Diagnosis and treatment of cardiac amyloidosis. A position statement of the European society of cardiology working group on myocardial and pericardial diseases. Eur J of Heart Fail. 42(16):1554–1568CrossRef
10.
go back to reference Arvidsson S, Henein MY, Wikstrom G, Suhr OB, Lindqvist P (2018) Right ventricular involvement in transthyretin amyloidosis. Amyloid 25(3):160–166CrossRef Arvidsson S, Henein MY, Wikstrom G, Suhr OB, Lindqvist P (2018) Right ventricular involvement in transthyretin amyloidosis. Amyloid 25(3):160–166CrossRef
11.
go back to reference Aljaroudi WA, Desai MY, Tang WH, Phelan D, Cerqueira MD, Jaber WA (2014) Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: state of the art review and focus on emerging nuclear techniques. J Nucl Cardiol 21(2):271–283CrossRef Aljaroudi WA, Desai MY, Tang WH, Phelan D, Cerqueira MD, Jaber WA (2014) Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: state of the art review and focus on emerging nuclear techniques. J Nucl Cardiol 21(2):271–283CrossRef
12.
go back to reference Jurcuţ R, Onciul S, Adam R, Stan C, Coriu D, Rapezzi C, Popescu BA (2020) Multimodality imaging in cardiac amyloidosis: a primer for cardiologists. Eur Heart J Cardiovasc Imaging 21(8):833–844CrossRef Jurcuţ R, Onciul S, Adam R, Stan C, Coriu D, Rapezzi C, Popescu BA (2020) Multimodality imaging in cardiac amyloidosis: a primer for cardiologists. Eur Heart J Cardiovasc Imaging 21(8):833–844CrossRef
13.
go back to reference Dlay JK, Duncan GW, Khoo TK, Williams-Gray CH, Breen DP, Barker RA, Burn DJ, Lawson RA, Yarnall AJ (2020) Progression of neuropsychiatric symptoms over time in an incident Parkinson’s disease Cohort (ICICLE-PD). Brain Sci 10(2):78CrossRef Dlay JK, Duncan GW, Khoo TK, Williams-Gray CH, Breen DP, Barker RA, Burn DJ, Lawson RA, Yarnall AJ (2020) Progression of neuropsychiatric symptoms over time in an incident Parkinson’s disease Cohort (ICICLE-PD). Brain Sci 10(2):78CrossRef
14.
go back to reference Itzhaki Ben Zadok O, Eisen A, Shapira Y, Monakier D, Iakobishvili Z, Schwartzenberg S, Abelow A, Ofek H, Kazum S, Ben-Avraham B, Hamdan A, Bental T, Sagie A, Kornowski R, Vaturi M (2020) Natural history and disease progression of early cardiac amyloidosis evaluated by echocardiography. Am J Cardiol 133:126–133 Itzhaki Ben Zadok O, Eisen A, Shapira Y, Monakier D, Iakobishvili Z, Schwartzenberg S, Abelow A, Ofek H, Kazum S, Ben-Avraham B, Hamdan A, Bental T, Sagie A, Kornowski R, Vaturi M (2020) Natural history and disease progression of early cardiac amyloidosis evaluated by echocardiography. Am J Cardiol 133:126–133
15.
go back to reference Lindmark K, Pilebro B, Sundstrom T, Lindqvist P (2020) Prevalence of wild type transtyrethin cardiac amyloidosis in a heart failure clinic. ESC Heart Fail 8(1):745–749CrossRef Lindmark K, Pilebro B, Sundstrom T, Lindqvist P (2020) Prevalence of wild type transtyrethin cardiac amyloidosis in a heart failure clinic. ESC Heart Fail 8(1):745–749CrossRef
16.
go back to reference Quinones MA, Otto CM, Stoddard M, Waggoner A, Zoghbi WA (2002) Doppler quantification task force of the n, standards committee of the american society of E. Recommendations for quantification of Doppler echocardiography: a report from the Doppler Quantification Task Force of the Nomenclature and Standards Committee of the American Society of Echocardiography. J Am Soc Echocardiogr 15(2):167–184 Quinones MA, Otto CM, Stoddard M, Waggoner A, Zoghbi WA (2002) Doppler quantification task force of the n, standards committee of the american society of E. Recommendations for quantification of Doppler echocardiography: a report from the Doppler Quantification Task Force of the Nomenclature and Standards Committee of the American Society of Echocardiography. J Am Soc Echocardiogr 15(2):167–184
17.
go back to reference Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA, Picard MH, Roman MJ, Seward J, Shanewise JS, Solomon SD, Spencer KT, Sutton MS, Stewart WJ (2005) Chamber quantification Writing G, American Society of Echocardiography's G, Standards C, European Association of E. Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 18(12):1440–1463. Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA, Picard MH, Roman MJ, Seward J, Shanewise JS, Solomon SD, Spencer KT, Sutton MS, Stewart WJ (2005) Chamber quantification Writing G, American Society of Echocardiography's G, Standards C, European Association of E. Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 18(12):1440–1463.
18.
go back to reference Mitter SS, Shah SJ, Thomas JD (2017) A test in context: E/A and E/e’ to assess diastolic dysfunction and LV filling pressure. J Am Coll Cardiol 69(11):1451–1464CrossRef Mitter SS, Shah SJ, Thomas JD (2017) A test in context: E/A and E/e’ to assess diastolic dysfunction and LV filling pressure. J Am Coll Cardiol 69(11):1451–1464CrossRef
19.
go back to reference Galderisi M, Cosyns B, Edvardsen T, Cardim N, Delgado V, Di Salvo G, Donal E, Sade LE, Ernande L, Garbi M, Grapsa J, Hagendorff A, Kamp O, Magne J, Santoro C, Stefanidis A, Lancellotti P, Popescu B, Habib G (2017) Standardization of adult transthoracic echocardiography reporting in agreement with recent chamber quantification, diastolic function, and heart valve disease recommendations: an expert consensus document of the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 18(12):1301–1310CrossRef Galderisi M, Cosyns B, Edvardsen T, Cardim N, Delgado V, Di Salvo G, Donal E, Sade LE, Ernande L, Garbi M, Grapsa J, Hagendorff A, Kamp O, Magne J, Santoro C, Stefanidis A, Lancellotti P, Popescu B, Habib G (2017) Standardization of adult transthoracic echocardiography reporting in agreement with recent chamber quantification, diastolic function, and heart valve disease recommendations: an expert consensus document of the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 18(12):1301–1310CrossRef
20.
go back to reference Perugini E, Guidalotti PL, Salvi F, Cooke RM, Pettinato C, Riva L, Leone O, Farsad M, Ciliberti P, Bacchi-Reggiani L, Fallani F, Branzi A, Rapezzi C (2005) Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J Am Coll Cardiol 46(6):1076–1084CrossRef Perugini E, Guidalotti PL, Salvi F, Cooke RM, Pettinato C, Riva L, Leone O, Farsad M, Ciliberti P, Bacchi-Reggiani L, Fallani F, Branzi A, Rapezzi C (2005) Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J Am Coll Cardiol 46(6):1076–1084CrossRef
21.
go back to reference Lekavich CL, Barksdale DJ, Neelon V, Wu JR (2015) Heart failure preserved ejection fraction (HFpEF): an integrated and strategic review. Heart Fail Rev 20(6):643–653CrossRef Lekavich CL, Barksdale DJ, Neelon V, Wu JR (2015) Heart failure preserved ejection fraction (HFpEF): an integrated and strategic review. Heart Fail Rev 20(6):643–653CrossRef
22.
go back to reference Klaassen SH, van Veldhuisen DJ, Nienhuis H, van den Berg MP, Hazenberg BP, van der Meer P (2020) Cardiac transthyretin-derived amyloidosis: an emerging target in heart failure with preserved ejection fraction? Card Fail Rev 6:21CrossRef Klaassen SH, van Veldhuisen DJ, Nienhuis H, van den Berg MP, Hazenberg BP, van der Meer P (2020) Cardiac transthyretin-derived amyloidosis: an emerging target in heart failure with preserved ejection fraction? Card Fail Rev 6:21CrossRef
23.
go back to reference Oghina S, Bougouin W, Bezard M, Kharoubi M, Komajda M, Cohen-Solal A, Mebazaa A, Damy T, Bodez D (2021) The impact of patients with cardiac amyloidosis in HFPEF trials. JACC Heart failure 9(3):169–178CrossRef Oghina S, Bougouin W, Bezard M, Kharoubi M, Komajda M, Cohen-Solal A, Mebazaa A, Damy T, Bodez D (2021) The impact of patients with cardiac amyloidosis in HFPEF trials. JACC Heart failure 9(3):169–178CrossRef
24.
go back to reference Gustavsson S, Granasen G, Gronlund C, Wiklund U, Morner S, Henein M, Suhr OB, Lindqvist P (2015) Can echocardiography and ECG discriminate hereditary transthyretin V30M amyloidosis from hypertrophic cardiomyopathy? Amyloid 22(3):163–170CrossRef Gustavsson S, Granasen G, Gronlund C, Wiklund U, Morner S, Henein M, Suhr OB, Lindqvist P (2015) Can echocardiography and ECG discriminate hereditary transthyretin V30M amyloidosis from hypertrophic cardiomyopathy? Amyloid 22(3):163–170CrossRef
25.
go back to reference Kyriakou P, Mouselimis D, Tsarouchas A, Rigopoulos A, Bakogiannis C, Noutsias M, Vassilikos V (2018) Diagnosis of cardiac amyloidosis: a systematic review on the role of imaging and biomarkers. BMC Cardiovasc Disord 18(1):221CrossRef Kyriakou P, Mouselimis D, Tsarouchas A, Rigopoulos A, Bakogiannis C, Noutsias M, Vassilikos V (2018) Diagnosis of cardiac amyloidosis: a systematic review on the role of imaging and biomarkers. BMC Cardiovasc Disord 18(1):221CrossRef
26.
go back to reference Quarta CC, Solomon SD, Uraizee I, Kruger J, Longhi S, Ferlito M, Gagliardi C, Milandri A, Rapezzi C, Falk RH (2014) Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis. Circulation 129(18):1840–1849CrossRef Quarta CC, Solomon SD, Uraizee I, Kruger J, Longhi S, Ferlito M, Gagliardi C, Milandri A, Rapezzi C, Falk RH (2014) Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis. Circulation 129(18):1840–1849CrossRef
27.
go back to reference Kristen AV, Perz JB, Schonland SO, Hansen A, Hegenbart U, Sack FU, Goldschmidt H, Katus HA, Dengler TJ (2007) Rapid progression of left ventricular wall thickness predicts mortality in cardiac light-chain amyloidosis. J Heart Lung Transplant 26(12):1313–1319CrossRef Kristen AV, Perz JB, Schonland SO, Hansen A, Hegenbart U, Sack FU, Goldschmidt H, Katus HA, Dengler TJ (2007) Rapid progression of left ventricular wall thickness predicts mortality in cardiac light-chain amyloidosis. J Heart Lung Transplant 26(12):1313–1319CrossRef
28.
go back to reference Solomon SD, Adams D, Kristen A, Grogan M, Gonzalez-Duarte A, Maurer MS, Merlini G, Damy T, Slama MS, Brannagan TH 3rd, Dispenzieri A, Berk JL, Shah AM, Garg P, Vaishnaw A, Karsten V, Chen J, Gollob J, Vest J, Suhr O (2019) Effects of Patisiran, an RNA interference therapeutic, on cardiac parameters in patients with hereditary transthyretin-mediated amyloidosis. Circulation 139(4):431–443CrossRef Solomon SD, Adams D, Kristen A, Grogan M, Gonzalez-Duarte A, Maurer MS, Merlini G, Damy T, Slama MS, Brannagan TH 3rd, Dispenzieri A, Berk JL, Shah AM, Garg P, Vaishnaw A, Karsten V, Chen J, Gollob J, Vest J, Suhr O (2019) Effects of Patisiran, an RNA interference therapeutic, on cardiac parameters in patients with hereditary transthyretin-mediated amyloidosis. Circulation 139(4):431–443CrossRef
Metadata
Title
Disease progression in cardiac morphology and function in heart failure: ATTR cardiac amyloidosis versus hypertensive left ventricular hypertrophy
Authors
M. Y. Henein
B. Pilebro
Per Lindqvist
Publication date
11-03-2022
Publisher
Springer Japan
Published in
Heart and Vessels / Issue 9/2022
Print ISSN: 0910-8327
Electronic ISSN: 1615-2573
DOI
https://doi.org/10.1007/s00380-022-02048-5

Other articles of this Issue 9/2022

Heart and Vessels 9/2022 Go to the issue