Key result
Heart transplantation for ATTR-CM carries ~10% late recurrence of cardiac amyloidosis.
Why the study?
Heart transplantation remains an established treatment option of last resort in selected patients with transthyretin amyloid cardiomyopathy, and long-term data on disease recurrence following transplantation were lacking.
Cohort (n=34)
Yes
This first long-term analysis demonstrates that recurrence of transthyretin amyloid cardiomyopathy in the cardiac allograft following heart transplantation is rare (10.3%) and occurs very late (>10 years post-transplant).
Rare late recurrence should not yet change post-HTX monitoring; leaves open need for prospective validation of surveillance strategies.
Background Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive disease of the myocardium. Despite advances in disease-modifying therapeutics, heart transplantation (HTX) remains an established treatment option of last resort in selected patients. Methods In this retrospective multi-centre analysis, ATTR-CM patients who have undergone HTX were included. Follow-up examinations included either endomyocardial biopsy, or non-invasive diagnosis / exclusion of cardiac amyloidosis by means of bone scintigraphy or cardiac magnetic resonance imaging. Results 34 ATTR-CM patients (59.0 ± 6.8 years, 70.6% male) underwent HTX between December 2006 and June 2024. Patients presented with advanced disease, with 31 (92.2%) patients in New York Heart Association (NYHA) functional class III, 16 (47.1%) in the revised National Amyloidosis Centre (NAC) stage ≥ 3, and a median N-terminal prohormone of b-type natriuretic peptide of 7,095 pg/mL (IQR: 4,453 – 11,353). From listing for HTX until transplantation, a median of 101 days (IQR: 51 – 388) passed, and three month survival was 85.3% (n = 29). HTX patients were followed for a median of 46.4 months (IQR: 21.9 – 129). Within the observation period, 10 (34.5%) patients deceased. Recurrence of cardiac TTR amyloidosis was diagnosed in 3 (10.3%) of patients between 136 and 183 months after HTX by endomyocardial biopsy. In the remaining 26 patients, recurrence of ATTR-CM was excluded non-invasively. Conclusions This is the first long-term analysis describing recurrence of cardiac TTR amyloidosis following HTX. In our cohort, recurrence was rare and only detected after >10 years after HTX.
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Poledniczek et al. (2026) conducted a cohort in Transthyretin amyloid cardiomyopathy (ATTR-CM) (n=34). Heart transplantation (HTX) was evaluated on Recurrence of cardiac TTR amyloidosis following heart transplantation. Heart transplantation in ATTR-CM patients was associated with a 10.3% recurrence rate of cardiac TTR amyloidosis (3/29 survivors), detected between 136 and 183 months post-transplant.
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