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Delayed Hyper-Enhancement Magnetic Resonance Imaging Provides Incremental Diagnostic and Prognostic Utility in Suspected Cardiac Amyloidosis

Bethany A. Austin, MD^_*, W.H. Wilson Tang, MD^_*, E. Rene Rodriguez, MD, Carmela Tan, MD, Scott D. Flamm, MD^_*,, David O. Taylor, MD^_*, Randall C. Starling, MD, MPH^_*, Milind Y. Desai, MD^_*,,_*

^_* Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio
Department of Pathology, Cleveland Clinic, Cleveland, Ohio
Imaging Institute, Cleveland Clinic, Cleveland, Ohio

^_* Reprint requests and correspondence: Dr. Milind Y. Desai, Director, Cardiac CT and MR, Department of Cardiovascular Medicine, J1-5, Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, Ohio 44195 (Email: desaim2{at}ccf.org).

Objectives: We sought to assess the diagnostic accuracy and incremental prognostic value of delayed hyper-enhancement cardiac magnetic resonance (DHE-CMR) compared with electrocardiographic and transthoracic echocardiographic (TTE) parameters in such patients.

Background: Utility of DHE-CMR in the diagnosis of patients with suspected cardiac amyloidosis (CA) has recently been demonstrated, but its incremental prognostic utility is unclear.

Methods: Forty-seven consecutive patients (mean age 63 years, 70% men, 55% New York Heart Association functional class >II) with suspected CA who underwent electrocardiography (ECG), TTE, DHE-CMR, and biopsy (38 endomyocardial, 9 extracardiac) were studied. Low voltage on ECG was defined as S-wave in lead V₁ + R-wave in lead V₅ or V₆ <15 mm. TTE parameters, including deceleration time, E/E' ratio, and diastolic grade were recorded. CMR was considered positive with diffuse DHE of the subendocardium extending to adjacent myocardium. All-cause mortality was ascertained.

Results: In the study population, 59% had low voltage on ECG, 30% had abnormal deceleration time 150 ms, 38% had E/E' ratio >15, and 47% had advanced (pseudonormal or restrictive) diastology. The diagnostic accuracy of DHE-CMR in patients undergoing endomyocardial biopsy was as follows: sensitivity 88%, specificity 90%, positive predictive value 88%, and negative predictive value 90%. On multivariable logistic regression testing of the diagnostic ability of various noninvasive imaging parameters, only DHE-CMR was significant (Wald chi-square statistic 9.6, p < 0.01). At 1-year post-biopsy, there were 9 (19%) deaths. On Cox proportional hazards analysis, only positive DHE-CMR was a predictor of 1-year mortality (Wald chi-square statistic 4.91, p = 0.03).

Conclusions: A characteristic DHE-CMR pattern is more accurate for diagnosis and is a stronger predictor of 1-year mortality in patients with suspected CA as compared with other noninvasive parameters.

Key Words: amyloidosis • cardiac magnetic resonance • echocardiography • biopsy • mortality

Abbreviations and Acronyms   CA = cardiac amyloidosis   CMR = cardiac magnetic resonance   DHE-CMR = delayed hyper-enhancement cardiac magnetic resonance   ECG = electrocardiogram   EMB = endomyocardial biopsy   LV = left ventricle   LVEF = left ventricular ejection fraction   MPI = myocardial performance index   NYHA = New York Heart Association   SSDI = Social Security Death Index   TTE = transthoracic echocardiography

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