Evaluation of Contemporary Echocardiographic and Histomorphology Parameters in Predicting Mortality in Patients With Endomyocardial Biopsy-Proven Cardiac AL Amyloidosis

Authors

Efstratios Koutroumpakis
Adam Niku
Christopher K Black
Abdelrahman Ali
Humaira Sadaf
Juhee Song
Nicolas Palaskas
Cezar Iliescu
Jean-Bernard Durand
Syed Wamique Yusuf
Hans C Lee
L Maximilian Buja
Anita Deswal
Jose Banchs

Publication Date

1-1-2022

Journal

Frontiers in Cardiovascular Medicine

Abstract

INTRODUCTION: This study examined the role of echocardiographic and cardiac histomorphology parameters in predicting mortality in patients with cardiac AL amyloidosis.

METHODS: Patients with endomyocardial biopsy-proven cardiac AL amyloidosis treated at MD Anderson Cancer Center between 6/2011 and 6/2020 were identified. Stored echocardiographic images and endomyocardial biopsy samples were processed for myocardial strain analysis and a detailed histomorphology characterization.

RESULTS: Of 43 patients; 44% were women and 63% white. Median age was 65 years; 51% underwent stem cell transplantation (SCT). Thirty patients (70%) died during follow up (median follow up: 4.1 years). Lower LA strain (

CONCLUSION: Lower LA strain independently predicted mortality in our cohort, and its performance in the routine assessment of AL amyloidosis may be beneficial. Furthermore, SCT for cardiac AL amyloidosis was associated with improved OS. These findings need to be confirmed by larger studies in the era of contemporary systemic therapies.

Keywords

cardiac AL amyloidosis, echocardiography, histomorphology, pathology, mortality, left atrial strain (LA strain), stem cell transplant (SCT)

Comments

Supplementary Materials

PMID: 3676230