Recurrence of transthyretin amyloid cardiomyopathy following heart transplantation - a long term follow-up analysis
M Poledniczek, M Ungericht, P Schlegel, F Duca, G Poelzl, K Uyanik-Uenal, A Zuckermann, A Meyer, C Hengstenberg, N Frey, F Aus Dem SiepenAbstract
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.