The Journal of Heart and Lung Transplantation
International Society for Heart and Lung Transplantation.

Outcomes in patients with cardiac amyloidosis undergoing heart transplantation: the eurotransplant experience

Published:January 11, 2023DOI:


      When advanced heart failure occurs in cardiac amyloidosis, prognosis is poor. In this setting heart transplantation (HTX) is a treatment option for selected patients. We here present the results of post-transplantation outcomes in cardiac amyloidosis within the Eurotransplant area, investigating possible predictors of survival.


      Of 115 patients undergoing HTX due to cardiac amyloidosis in the Eurotransplant region between November 1987 and May 2020, detailed assessment prior to transplantation was available in 85 patients. The present study was conducted in a retrospective approach. Primary endpoint was mortality after HTX. Baseline variables were entered in a Cox proportional hazards model with the primary endpoint as a dependent variable.


      Median overall survival following HTX was 6.3 years in the overall collective and the subgroup. Univariate Cox proportional hazards model revealed a significant relationship between overall survival and the transplantation period (2008 to 2020 vs 1987 to 2007; median survival 9.7 years vs 1.8 years, hazard ratio 0.45, p = 0.01). Further predictors were albumin concentration (hazard ratio 0.92, p < 0.001), and systolic blood pressure (hazard ratio 0.96, p < 0.001). The transplant period as well as albumin concentration remained significant independent predictors in the AL sub cohort in a multivariate Cox proportional hazards model.


      HTX is a viable treatment option for patients at an advanced stage of cardiac amyloidosis as overall survival after transplantation has improved in the modern age. Patients at a very advanced stage of the disease, indicated by low serum albumin and blood pressure, show worse outcomes following HTX. Optimal timing and careful patient selection may therefore be particularly important to further improve post-HTX survival in amyloidosis patients.


      AL (Immunoglobulin lightchain), ATTR (Transthyretin), HTX (Heart transplantation), HU (High urgency), ISHLT (The International Society for Heart and Lung Transplantation), NYHA (New York Heart Association), ASCT (Autologous stem cell transplantation), NTproBNP (N-terminal prohormone of brain natriuretic peptide), hsTnT (High-sensitivity troponin T), SvO2 (Mixed venous oxygen saturation), PCWP (Pulmonary capillary wedge pressure), PAP (Pulmonary artery pressure), CI (Cardiac index), CVP (Central venous pressure), PVR (Pulmonary vascular resistance), LVEF (Left ventricular ejection fraction), LVEDD (Left ventricular enddiastolic diameter), LVESD (Left ventricular endsystolic diameter)
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        • Merlini G
        • Bellotti V
        Molecular mechanisms of amyloidosis.
        N Engl J Med. 2003; 349: 583-596
        • Bellotti V
        • Nuvolone M
        • Giorgetti S
        • et al.
        The workings of the amyloid diseases.
        Ann Med. 2007; 39: 200-207
        • Gertz MA
        • Dispenzieri A
        • Sher T
        Pathophysiology and treatment of cardiac amyloidosis.
        Nat Rev Cardiol. 2015; 12: 91-102
        • Siddiqi OK
        • Ruberg FL
        Cardiac amyloidosis: an update on pathophysiology, diagnosis, and treatment.
        Trends Cardiovasc Med. 2018; 28: 10-21
        • Rubin J
        • Maurer MS
        Cardiac amyloidosis: overlooked, underappreciated, and treatable.
        Annu Rev Med. 2020; 71: 203-219
        • Gertz MA
        Immunoglobulin light chain amyloidosis: 2018 Update on diagnosis, prognosis, and treatment.
        Am J Hematol. 2018; 93: 1169-1180
        • Maurer MS
        • Schwartz JH
        • Gundapaneni B
        • et al.
        Tafamidis treatment for patients with transthyretin amyloid cardiomyopathy.
        N Engl J Med. 2018; 379: 1007-1016
        • Adams D
        • Gonzalez-Duarte A
        • O’Riordan WD
        • et al.
        Patisiran, an RNAi therapeutic, for hereditary transthyretin amyloidosis.
        N Engl J Med. 2018; 379: 11-21
        • Selvanayagam JB
        • Hawkins PN
        • Paul B
        • Myerson SG
        • Neubauer S
        Evaluation and management of the cardiac amyloidosis.
        J Am Coll Cardiol. 2007; 50: 2101-2110
        • Volz MJ
        • Pleger ST
        • Weber A
        • et al.
        Initial experience with percutaneous mitral valve repair in patients with cardiac amyloidosis.
        Eur J Clin Invest. 2021; 51e13473
        • Swiecicki PL
        • Edwards BS
        • Kushwaha SS
        • Dispenzieri A
        • Park SJ
        • Gertz MA
        Left ventricular device implantation for advanced cardiac amyloidosis.
        J Heart Lung Transplant. 2013; 32: 563-568
        • Lin G
        • Dispenzieri A
        • Kyle R
        • Grogan M
        • Brady PA
        Implantable cardioverter defibrillators in patients with cardiac amyloidosis.
        J Cardiovasc Electrophysiol. 2013; 24: 793-798
        • Sousa M
        • Monohan G
        • Rajagopalan N
        • Grigorian A
        • Guglin M
        Heart transplantation in cardiac amyloidosis.
        Heart Fail Rev. 2017; 22: 317-327
        • Kristen Av.
        • Kreusser MM
        • Blum P
        • et al.
        Improved outcomes after heart transplantation for cardiac amyloidosis in the modern era.
        J Heart Lung Transplant. 2018; 37: 611-618
        • Davis MK
        • Lee PHU
        • Witteles RM
        Changing outcomes after heart transplantation in patients with amyloid cardiomyopathy.
        J Heart Lung Transplant. 2015; 34: 658-666
        • Gray Gilstrap L
        • Niehaus E
        • Malhotra R
        • et al.
        Predictors of survival to orthotopic heart transplant in patients with light chain amyloidosis.
        J Heart Lung Transplant. 2014; 33: 149-156
        • Vaidya GN
        • Patel JK
        • Kittleson M
        • et al.
        Intermediate-term outcomes of heart transplantation for cardiac amyloidosis in the current era.
        Clin Transplant. 2021; 35e14308
      1. CD Barrett, KM Alexander, H Zhao, et al., Outcomes in patients with cardiac amyloidosis undergoing heart transplantation, JACC Heart Fail, 8, 461–468, 2020.

        • Chen Q
        • Moriguchi J
        • Levine R
        • et al.
        Outcomes of heart transplantation in cardiac amyloidosis patients: a single center experience.
        Transplant Proc. 2021; 53: 329-334
      2. Smits J. Eurotransplant Manual©, Chapter 6, p. 18, 2021.

        • Mehra MR
        • Canter CE
        • Hannan MM
        • et al.
        The 2016 International Society for Heart Lung Transplantation listing criteria for heart transplantation: a 10-year update.
        J Heart Lung Transplant. 2016; 35: 1-23
      3. Declaration of Helsinki World Medical Association declaration of Helsinki.
        Bull World Health Organ. 2013; 79 (JAVA.)
      4. ISHLT Board. International Society for Heart and Lung Transplantation Statement on Transplant Ethics. 2014.

        • Kittleson MM
        • Maurer MS
        • Ambardekar A v.
        • et al.
        Cardiac amyloidosis: evolving diagnosis and management: a scientific statement from the American Heart Association.
        Circulation. 2020; 142: e7-e22
        • Kreusser MM
        • Volz MJ
        • Knop B
        • et al.
        A novel risk score to predict survival in advanced heart failure due to cardiac amyloidosis.
        Clin Res Cardiol. 2020; 109: 700-713
        • Dispenzieri A
        • Gertz MA
        • Kyle RA
        • et al.
        Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis.
        J Clin Oncol. 2004; 22: 3751-3757
        • Grogan M
        • Scott CG
        • Kyle RA
        • et al.
        Natural history of wild-type transthyretin cardiac amyloidosis and risk stratification using a novel staging system.
        J Am Coll Cardiol. 2016; 68: 1014-1020
        • Gillmore JD
        • Damy T
        • Fontana M
        • et al.
        A new staging system for cardiac transthyretin amyloidosis.
        Eur Heart J. 2018; 39: 2799-2806
        • Gertz MA
        • Comenzo R
        • Falk RH
        • et al.
        Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis.
        Am J Hematol. 2005, 79, 319-328;
      5. ISHLT. Focus theme: trends in recipient characteristics and impact on outcomes. 2021.

      6. The International Society of Heart and Lung Transplantation. ISHLT transplant registry quarterly reports for heart in Europe. 2018.

        • Kimmich CR
        • Terzer T
        • Benner A
        • et al.
        Daratumumab for systemic AL amyloidosis: prognostic factors and adverse outcome with nephrotic-range albuminuria.
        Blood. 2020; 135: 1517-1530
        • Wechalekar AD
        • Schonland SO
        • Kastritis E
        • et al.
        A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis.
        Blood. 2013; 121: 3420-3427
        • Zhang Z
        • Pereira SL
        • Luo M
        • Matheson EM
        Evaluation of blood biomarkers associated with risk of malnutrition in older adults: a systematic review and meta-analysis.
        Nutrients. 2017; 9: 829
        • Franco OH
        • Peeters A
        • Bonneux L
        • de Laet C
        Blood pressure in adulthood and life expectancy with cardiovascular disease in men and women: life course analysis.
        Hypertension. 2005; 46: 280-286