Abstract

Introduction: Autologous stem cell transplantation (ASCT) is an effective therapy for prolonging survival in patients with systemic AL amyloidosis (ALA). Cardiac dysfunction due to amyloid involvement is a strong determinant of mortality. Whether ASCT can improve cardiac function is not well understood. Hypothesis: To assess changes in conventional echocardiographic parameters and global longitudinal strain (GLS) at baseline and 1 year post-ASCT and to determine predictors of 1 year mortality post-ASCT. Methods: Our study included fifty-one patients with newly diagnosed, biopsy-proven systemic AL amyloidosis undergoing ASCT. Cardiac biomarkers and echocardiographic measurements were obtained in all patients at baseline and at 1 year post-ASCT in 1 year survivors. Speckle-tracking global longitudinal strain (GLS) was retrospectively analyzed using vendor neutral software (TomTec Imaging Systems GmbH). Results: The mean age was 56.4 +/- 7.9 and 47.1% were female. Cardiac involvement was present in 68.6% based on consensus criteria. The mean baseline EF was 64.9 +/- 8.3. Complete hematologic response at 1 year was 55.8%. Mortality was 15.7% at 1 year. Long-term mortality was 27.5% (mean follow-up 4.0 +/- 2.6 years). Predictors of 1 year mortality were baseline GLS, troponin, BNP, septal thickness and E/A ratio. Age, gender and creatinine were not predictive. Among survivors at 1 year post-ASCT, there was no significant change in the biomarker or echocardiographic indices compared to baseline, regardless of hematologic response. Conclusions: Markers of cardiac dysfunction at baseline are predictive of 1 year mortality in patients with systemic ALA. Among those who survived to 1 year post-ASCT, there was no change in cardiac function assessed by echocardiography or biomarkers compared to baseline.

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