Abstract 19304: Clinical Profile and Outcomes of Patients Undergoing Combined Heart and Liver Transplantation With Transthyretin Amyloidosis: A Report From the International Consortium on Cardiac Amyloid Transplantation (iCCAT)
Background: Orthotopic heart transplant (OHT) is an accepted treatment option for transthyretin amyloidosis (ATTR) patients with end-stage heart failure. The incremental survival benefit of adjunctive liver transplant remains poorly defined.
Methods: We aim to describe the clinical profiles/prognoses of patients undergoing OHT/liver transplant compared with OHT alone. From January 1998 to May 2015, 42 of 48 ATTR patients being evaluated for OHT at 10 institutions underwent transplant and were included in the final analysis. Data were entered into an encrypted online database, Research Electronic Data Capture. The primary endpoint of post-OHT all-cause mortality was compared between 3 cohorts defined by transplant and mutation status using Kaplan-Meier survival analysis: OHT alone-wild type (WT) (n=12; Cohort A); OHT alone-ATTR mutation (n=21; Cohort B); and combined OHT/liver transplant (n=9; Cohort C).
Results: Of those undergoing OHT, 12 (28.6%) were WT, 15 (32.5%) had a V122I mutation, 3 (7.1%) had a T60A mutation, 8 had other mutations, and 4 had unknown mutations. For the subset of 9 patients who underwent combined OHT/liver transplant (4 concurrently, 5 sequentially), the mutations were as follows: V122I (n=3), T60A (n=3), S23A (n=2), and Tyr77 (n=1). Baseline characteristics did not differ significantly across the 3 cohorts for age, sex, LV ejection fraction, and other demographic, clinical, and hemodynamic characteristics. Peripheral neuropathy was present in 2 (17%) of Cohort A, 7 (33%) of Cohort B, and 4 (44%) of Cohort C (p=0.08). Post-OHT all-cause mortality was similar across cohorts (log-rank P=0.218; Figure).
Conclusions: This is the largest analysis of ATTR cardiac amyloidosis patients receiving heart and liver transplantation. ATTR patients receiving OHT/liver transplant versus OHT alone have similar median-term survival. The added survival benefit of liver transplantation to OHT in ATTR mutant patients should be investigated further.
- transthyretin amyloidosis
- peripheral neuropathy
- orthotopic heart transplant
- orthotopic liver transplant
Author Disclosures: K.C. Verkouw: None. M. Vaduganathan: None. J.R. Stone: None. M. Maurer: None. T. De Marco: None. V. Selby: None. J. Estep: None. J. Patel: None. M. Hanna: None. D. Seldin: None. M. Zucker: None. D. Baran: Research Grant; Modest; Maquet. Honoraria; Modest; Maquet. M.J. Semigran: None.
- © 2015 by American Heart Association, Inc.