Abstract 19078: Early Predictors of Survival to Heart Transplantation in Patients With Amyloid Light-chain Cardiomyopathy
Background: Amyloid cardiomyopathy progresses more rapidly and has different manifestations than more common cardiomyopathies. Nevertheless, survival of selected cardiac amyloid patients after orthotopic heart transplantation (OHT) followed by autologous stem cell transplant is similar to other restrictive cardiomyopathies. The importance of clinical presentation and pre-transplantation characteristics on outcome in patients with AL amyloid cardiomyopathy evaluated for heart transplantation is still not well defined.
Methods: The impact of echocardiographic, hemodynamic, and clinical parameters on outcome in 46 patients evaluated for OHT and enrolled in the International Consortium for Cardiac Amyloid Transplant (iCCAT) database was studied. Cox proportional hazards models of time to death were used. End point was death after transplant evaluation censored at transplant.
Results: The median age at the time of transplant evaluated was 57.6 (+/- 9.6) years, and the mean time from presentation to listing was 27 (+/- 26) days. 19 (41%) patients underwent OHT after an average wait time of 72 days. Univariate risk factors associated with death after initial evaluation were cardiac output (p=0.012), right ventricular stroke work index (p=0.033), left ventricular end diastolic dimension (p=0.024), left ventricular outflow tract velocity time integral (p=0.019), mean arterial pressures (p=0.005), NT pro-BNP (p=0.007), presence of pleural effusion (p=0.005), as well as elevated kappa and lambda serum free light chain concentrations (p=0.004). Whereas left ventricular ejection fraction did not correlate with death after evaluation, increased right ventricular wall thickness remained a strong mortality predictor in a multivariate model with light chain difference and cardiac output (HR 1.6 for every 1 mm increase in thickness, p=0.017).
Conclusions: Pleural involvement, high light chain burden, and right ventricular infiltration predicted death while awaiting a donor organ and may be markers of more systemic disease. Ultimately, this could affect transplant candidate selection and allocation strategies.
Author Disclosures: J. Steiner: None. V. Selby: None. K. Verkouw: None. J. Svetlichnaya: None. D. Defaria Yeh: None. T. De Marco: None. M. Semigran: None.
- © 2015 by American Heart Association, Inc.