Detection of Left Ventricular Systolic Dysfunction in Cardiac Amyloidosis with Strain Rate Echocardiography Diego Bellavia, MD, Theodore P. Abraham, MD, Patricia A. Pellikka, MD, Ghormallah B. Al-Zahrani, MBBS, Angela Dispenzieri, MD, Jae K. Oh, MD, Kent R. Bailey, PhD, Christina M. Wood, MS, Salvatore Novo, MD, Chinami Miyazaki, MD, and Fletcher A. Miller, Jr., MD, Rochester, Minnesota; and Palermo, Italy Background: We examined the potential role of Doppler myocardial imaging including tissue ve- locity imaging, strain imaging, and strain rate imaging for detection of left ventricular systolic dysfunction in cardiac amyloidosis (CA) and de- termined the minimum dataset required to make the diagnosis. Methods and Results: Doppler myocardial imaging was performed in 103 patients with amyloidosis (AL). Peak longitudinal systolic tissue velocity, sys- tolic strain rate (sSR), and systolic strain (sS) were determined for 16 left ventricular segments. Radial and circumferential sSR and sS were also measured. Patients with increased left ventricular wall thick- ness were classified with advanced-CA, and the re- mainder of the patients were classified with AL- normal-wall-thickness. The global means of peak systolic tissue velocity (3.6  1.0 vs. 3.9  0.9, P  .007), sSR (0.8  0.3 vs. 1.0  0.2, P < .001), and sS (9.9  3.7 vs. 15.6  3.3, P < .001) were significantly lower in advanced-CA compared with AL-normal-wall-thickness. The mean of either sSR or sS from 6 middle or all 16 segments similarly differ- entiated patients with advanced-CA from AL-normal- wall-thickness. Conclusions: Longitudinal sS most accurately de- tects longitudinal systolic dysfunction in AL and best differentiates patients with advanced-CA with increased ventricular thickness from patients with AL-normal-wall-thickness. Interrogation of six middle segments was sufficient in identifying patients with advanced-CA. Further studies are warranted to define the incremental prognostic value of these new parameters in predicting out- comes for patients with AL. (J Am Soc Echocardiogr 2007;20:1194-1202.) Primary amyloidosis (AL) is characterized by extracel- lular deposition of pathologic insoluble fibrillar protein in organs and tissues. 1,2 Congestive heart failure is seen in approximately 25% of patients with AL, and its development is associated with an average survival of less than 6 months. 3 In addition, cardiac involvement is the main contraindication to peripheral blood autol- ogous stem cell transplantation (PBSCT), which is effective in reducing symptoms and in increasing the survival to 4 years in 85% of patients. 4 To date, transthoracic echocardiography has played a major role in the evaluation of cardiac amyloidosis (CA) and is considered the procedure of choice for noninvasive diagnosis. 5 Classic two- dimensional (2D) echocardiography features associ- ated with CA include concentrically thickened left and (often) right ventricular walls, normal to small left ventricular (LV) cavity, ejection fraction ranging from low normal to moderately reduced, granular myocardial texture, dilated atria, and pericardial and pleural effusions. 6 Doppler-derived echocardio- graphic indices have been used to demonstrate high left-sided filling pressures, 7,8 Recent studies have shown the potential usefulness of Doppler myocar- dial imaging (DMI) 9-14 for the detection of LV dys- function associated with CA. 15 The purpose of this study was twofold: (1) to clarify the role for the various DMI modalities in determining the difference in longitudinal, radial, and circumferential LV dysfunction between pa- tients with advanced-CA from those patients with systemic AL-normal-wall-thickness; and (2) to estab- lish which of the DMI modalities is best for detec- tion of left systolic dysfunction in patients with primary systemic AL and the minimum number of segments necessary for this diagnosis. From the Cardiovascular Division, Mayo Clinic and Foundation, Rochester, Minneosota; and Department of Cardiovascular Dis- eases, University of Palermo, Palermo, Italy. This study was supported in part by a grant from the American Heart Association Heartland Affiliate (Grant No. 0620073Z). Reprint requests: Fletcher A. Miller, Jr, MD, Cardiovascular Division, Mayo Clinic and Foundation, Rochester, MN 55905 (E-mail: miller.fletcher@mayo.edu). 0894-7317/$32.00 Copyright 2007 by the American Society of Echocardiography. doi:10.1016/j.echo.2007.02.025 1194