GUIDELINES AND STANDARDS American Society of Echocardiography Clinical Recommendations for Multimodality Cardiovascular Imaging of Patients with Hypertrophic Cardiomyopathy Endorsed by the American Society of Nuclear Cardiology, Society for Cardiovascular Magnetic Resonance, and Society of Cardiovascular Computed Tomography Sherif F. Nagueh, MD, FASE, Chair,* S. Michelle Bierig, RDCS, FASE,* Matthew J. Budoff, MD,§ Milind Desai, MD,* Vasken Dilsizian, MD,† Benjamin Eidem, MD, FASE,* Steven A. Goldstein, MD,* Judy Hung, MD, FASE,* Martin S. Maron, MD,‡ Steve R. Ommen, MD,* and Anna Woo, MD,* Houston, Texas; St. Louis, Missouri; Los Angeles, California; Cleveland, Ohio; Baltimore, Maryland; Rochester, Minnesota; Washington, District of Columbia; Boston, Massachusetts; Toronto, Ontario, Canada (J Am Soc Echocardiogr 2011;24:473-98.) Keywords: Hypertrophic cardiomyopathy, Echocardiography, Nuclear imaging, Cardiovascular magnetic res- onance, Cardiac computed tomography TABLE OF CONTENTS From the Methodist DeBakey Heart and Vascular Center, Houston, Texas (S.F.N.); St. Anthony’s Medical Center, St. Louis, Missouri (S.M.B.); Los Angeles Biomedical Research Institute, Torrance, California (M.J.B.); Cleveland Clinic, Abbreviations 474 Cleveland, Ohio (M.D.); the University of Maryland School of Medicine, Organization of the Writing Group and Evidence Review 474 Baltimore, Maryland (V.D.); Mayo Clinic, Rochester, Minnesota (B.E., S.R.O.); 1. Introduction 474 Washington Hospital Center, Washington, District of Columbia (S.A.G.); 2. Echocardiography 474 Massachusetts General Hospital, Boston, Massachusetts (J.H.); Tufts Medical A. Cardiac Structure 474 Center, Boston, Massachusetts (M.S.M.); and Toronto General Hospital, B. Assessment of LV Systolic Function 475 University of Toronto, Toronto, Ontario, Canada (A.W.). C. Assessment of LV Diastolic Function 477 D. Dynamic Obstruction and Mitral Valve Abnormalities 477 The following authors reported no actual or potential conflicts of interest in relation E. Mitral Regurgitation in HCM 480 to this document: Sherif F. Nagueh, MD, FASE, S. Michelle Bierig, RDCS, FASE, F. Myocardial Ischemia, Fibrosis, and Metabolism 481 Matthew J. Budoff, MD, Milind Desai, MD, Vasken Dilsizian, MD, Benjamin Eidem, G. Guidance of Septal Reduction Procedures 481 MD, FASE, Steven A. Goldstein, MD, Judy Hung, MD, FASE, Steve R. Ommen, i. Surgical Myectomy 481 MD, and Anna Woo, MD. The following author reported a relationship with one ii. Alcohol Septal Ablation 481 or more commercial interests: Martin S. Maron, MD, serves as a consultant for iii. Permanent Pacing 483 PGx Health. H. Screening and Preclinical Diagnosis 483 Attention ASE Members: 3. Nuclear Imaging 484 The ASE has gone green! Visit www.aseuniversity.org to earn free continuing A. Cardiac Structure 484 medical education credit through an online activity related to this article. Cer- B. Radionuclide Angiography for LV Systolic Function 484 tificates are available for immediate access upon successful completion of C. Radionuclide Angiography for LV Diastolic Function 484 the activity. Nonmembers will need to join the ASE to access this great mem- D. Dynamic Obstruction and Mitral Valve Abnormalities 484 ber benefit! E. Mitral Regurgitation in HCM 484 F. Myocardial Ischemia, Fibrosis, and Metabolism 484 i. SPECT 484 Reprint requests: American Society of Echocardiography, 2100 Gateway Centre ii. Positron Emission Tomography (PET) 485 Boulevard, Suite 310, Morrisville, NC 27560 (E-mail: [email protected]). iii. Imaging Metabolism 486 Writing Group of the *American Society of Echocardiography (ASE) †American G. Guidance of Septal Reduction Procedures 486 Society of Nuclear Cardiology, ‡ Society for Cardiovascular Magnetic H. Screening and Preclinical Diagnosis 486 Resonance, and §Society of Cardiovascular Computed Tomography. 3. Cardiovascular Magnetic Resonance 486 0894-7317/$36.00 A. Cardiac Structure 486 B. Assessment of LV Systolic Function 487 Copyright 2011 by the American Society of Echocardiography. C. Assessment of LV Diastolic Function 487 doi:10.1016/j.echo.2011.03.006 473 474 Nagueh et al Journal of the American Society of Echocardiography May 2011 D. Dynamic Obstruction and The purpose of this document is to review the strengths and Abbreviations Mitral Valve Abnormali- applications of the current imaging modalities and provide ASE = American Society of ties 487 recommendation guidelines for using these techniques to optimize Echocardiography E. Mitral Regurgitation in the management of patients with HCM. The recommendations are HCM 488 CAD based on observational studies, sometimes obtained in a small = Coronary artery F. Myocardial Ischemia, Fibrosis, number of patients, and from the clinical experience of the writing disease and Metabolism 488 group members, given the scarcity of multimodality imaging compar- CMR i. Ischemia 488 = Cardiovascular ative effectiveness studies. Notwithstanding these recommendations, magnetic resonance ii. Fibrosis 488 iii. Imaging Metabolism 489 the writing group believes that the selection of a given imaging CT = Computed tomography G. Guidance of Septal Reduc- modality must be individualized. tion Procedures 489 EF = Ejection fraction H. Screening and Preclinical HCM = Hypertrophic Diagnosis 489 1. INTRODUCTION cardiomyopathy 4. Cardiac Computed Tomogra- phy 489 ICD HCM is the most common genetic cardiomyopathy. Across multiple = Implantable A. Cardiac Structure 489 geographies and ethnicities, the prevalence is approximately 0.2%.1 cardioverter-defibrillator B. Assessment of LV Systolic HCM is transmitted in an autosomal dominant inheritance pattern. LA = Left atrial Function 490 C. Assessment of LV Diastolic The natural history is benign in the majority of patients, with a near LGE = Late gadolinium Function 490 normal life span. However, adverse outcomes, including sudden car- enhancement D. Dynamic Obstruction and diac death, lifestyle-limiting symptoms secondary to dynamic left ven- Mitral Valve Abnormali- tricular (LV) outflow tract (LVOT) obstruction and/or diastolic filling LV = Left ventricular ties 490 abnormalities, atrial fibrillation, and LV systolic dysfunction, occur in LVOT = Left ventricular E. Mitral Regurgitation in some patients.1 outflow tract HCM 490 The clinical diagnosis of HCM is based on the demonstration of LV F. Myocardial Ischemia, Fibrosis, MCE hypertrophy in the absence of another disease process that can rea- = Myocardial contrast and Metabolism 490 sonably account for the magnitude of hypertrophy present.1 Many echocardiography G. Guidance of Septal Reduc- patients are diagnosed serendipitously when a cardiac murmur or RV = Right ventricular tion Procedures 490 H. Screening and Preclinical electrocardiographic abnormality prompts echocardiographic evalua- SAM = Systolic anterior Diagnosis 491 tion. Others present with dyspnea, chest pain, and/or presyncope. motion 5. Hypertrophic Cardiomyopathy Sudden cardiac death occurs in approximately 1% of patients with Imaging in the Pediatric Popula- HCM each year, and detecting patients at risk for sudden cardiac STE = Speckle-tracking tion 491 echocardiography death is one of the most challenging clinical dilemmas. At the current 6. Role of Imaging in the Differential time, a set of clinical risk factors1 and imaging results are considered in SPECT = Single photon- Diagnosis of Hypertrophic Car- the context of each patient’s specific circumstances to help each pa- emission computed diomyopathy 491 tient decide whether an implantable cardioverter-defibrillator (ICD) 7. Recommendations for Clinical tomography represents an appropriate choice for that patient.1 Applications 492 TEE = Transesophageal A. Cardiac Structure 492 The management of HCM is based on a thorough understanding echocardiography B. Assessment of LV Systolic and of the underlying anatomy and pathophysiology. In addition, careful assessment for concomitant structural heart disease is crucial to allow 3D = Three-dimensional Diastolic Function 493 C. Assessment of LVOT Ob- appropriate patient selection for advanced therapies. TTE = Transthoracic struction 493 Various imaging modalities can be used to assess cardiac structure echocardiography D. Evaluation of Patients Under- and function, the presence and severity of dynamic obstruction, the going Invasive Therapy 493 2D = Two-dimensional presence of mitral valve abnormalities, and the severity of mitral re- E. Diagnosis of CAD in Patients gurgitation, as well as myocardial ischemia, fibrosis, and metabolism. With HCM 494 In addition, imaging can be used to guide treatment, screening and F. Screening 494 preclinical diagnosis and to detect phenocopies. G. Role of Imaging in Identifying Patients at High Risk for Sudden Cardiac Death 494 ORGANIZATION OF THE WRITING GROUP AND EVIDENCE 2. ECHOCARDIOGRAPHY REVIEW A. Cardiac Structure The writing group was composed of acknowledged experts in hyper- LV volumes and the pattern of hypertrophy can be well defined by trophic cardiomyopathy (HCM) and its imaging representing the echocardiography (Figure 1, Video 1 [ view video clip online], ASE, the American Society of Nuclear Cardiology, the Society for Table 1). Ventricular volumes in HCM are usually normal or slightly Cardiovascular Magnetic Resonance, and the Society of reduced. Traditionally, the biplane Simpson’s method has been ap- Cardiovascular Computed Tomography. The document was re- plied to the measurement of LV volumes and ejection fraction viewed by the ASE Guidelines and Standards Committee and four of- (EF).2 Recently, real-time