Abstract 18160: Predictors of Survival to Orthotopic Heart Transplant in Patients with Immunoglobulin Light Chain Amyloid Cardiomyopathy
Introduction: Heart failure from immunoglobulin AL amyloidosis is rapidly fatal. Serial orthotopic heart transplant (OHT) followed by high dose melphalan / autologous stem cell transplant (ASCT) improves outcomes in selected patients. Debates still focus on the appropriate selection criteria for this approach.
Methods: We performed a retrospective analysis of all patients with AL amyloidosis evaluated at Massachusetts General Hospital (2000 - 2011). Demographic and clinical data were obtained at the time of evaluation for OHT and ASCT. Kaplan-Meier and Cox regression analyses were performed to identify predictors of survival to OHT.
Results: Of 41 patients (age 56 ±9 years), 18 (44%) underwent OHT, of which 14 subsequently underwent ASCT. One patient died before ASCT of sepsis, and 3 are awaiting ASCT. Three-year survival is 75%. Of the 14 patients, 3 (21%) died from sepsis (n=1), progressive amyloidosis (n=1), or cardiac arrest (n=1). Of the 23 untransplanted patients, 21 (91%) died with a median time to death from initial evaluation of 55 days. Thirteen were listed for OHT but 12 died before transplant from cardiac arrest (n=5), progressive systemic amyloidosis (n=4), sepsis (n=2), and heart failure (n=1). Ten were not listed for OHT due to contra-indicating factors (severe systemic amyloidosis (n=3), cardiac arrest (n=2), coagulopathy (n=1), sepsis (n=1), heart failure (n=1), stroke (n=1), severe depression (n=1)). Of those listed for OHT (n=31), higher weight and BMI were associated with a lower likelihood of survival to transplant (189 ± 46 vs. 162 ± 20 lbs, P=0.03; Cox hazard ratio (HR) of death before OHT of 1.22 for every 10-lb increase in weight, 95% CI 1.03-1.44). For patients undergoing OHT, wait time from evaluation correlated with BMI (r=0.48, P=0.04). Of the hemodynamic variables studied, greater systemic arterial pulse pressure predicted survival to OHT (HR for death before transplant 0.43 for every 10 mmHg increase, 95% CI 0.19-0.98, P=0.045).
Conclusion: Long-term survival is excellent in cardiac amyloid patients receiving serial OHT and ASCT. Mortality remains high while patients await cardiac donors. Predictors of survival to OHT include increased systemic pulse pressure and lower weight and BMI due to shorter wait time for suitable organs.
- © 2011 by American Heart Association, Inc.