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Circulation. 2022;145:e895–e1032. DOI: 10.1161/CIR.0000000000001063 May 3, 2022 e913 CLINICAL STATEMENTS AND GUIDELINES Recommendation-Specific Supportive Text 1. and 2. Inherited dilated, restrictive, and hypertrophic cardiomyopathies have been identified, although 1 gene variant may cause different phenotypes in the same family. The most common pathogenic variants identified are truncations in the large structural protein titin, which have been implicated in DCM3–5 and also in peripartum or alcoholic cardiomyopathies; however, variants that do not cause disease are also common. Pathogenic variants in lamin A/C can be associated with conduction block and atrial arrhythmias as well as ventricular arrhythmias, which may progress more rapidly than symptoms of HF. Although previously linked with the phenotype of arrhythmogenic RV cardiomyopathy, desmosomal protein variants are now recognized to affect the left ventricle also with or without the right ventricle, and the term arrhythmogenic cardiomyopathy is now preferred for the phenotype of arrhythmias combined with DCM. Filamin-C mutations have been associated with skeletal myopathies and with isolated cardiomyopathy with ventricular arrhythmias. The identification of pathogenic variants associated with increased risk of sudden death may trigger consideration of primary prevention implantable cardioverter-defibrillators (ICDs) even in patients who have LVEF >0.35 or <3 months of guidelinerecommended therapies.6 Evidence of desmosomal cardiac disease carries the additional implication of advice to avoid strenuous exercise, which may accelerate ventricular remodeling.7 Genetic confirmation of symptomatic Fabry’s cardiomyopathy is an indication for replacement therapy with the enzyme agalsidase beta, and migalastat was recently approved for this uncommon cardiomyopathy. 4.4 Evaluation With Cardiac Imaging Recommendations for Evaluation With Cardiac Imaging Referenced studies that support the recommendations are summarized in the Online Data Supplements. COR LOE Recommendations 1 C-LD 1. In patients with suspected or new-onset HF, or those presenting with acute decompensated HF, a chest x-ray should be performed to assess heart size and pulmonary congestion and to detect alternative cardiac, pulmonary, and other diseases that may cause or contribute to the patient’s symptoms.1,2 1 C-LD 2. In patients with suspected or newly diagnosed HF, transthoracic echocardiography (TTE) should be performed during initial evaluation to assess cardiac structure and function.3 1 C-LD 3. In patients with HF who have had a significant clinical change, or who have received GDMT and are being considered for invasive procedures or device therapy, repeat measurement of EF, degree of structural remodeling, and valvular function are useful to inform therapeutic interventions.4–7 1 C-LD 4. In patients for whom echocardiography is inadequate, alternative imaging (eg, cardiac magnetic resonance [CMR], cardiac computed tomography [CT], radionuclide imaging) is recommended for assessment of LVEF.8–15 2a B-NR 5. In patients with HF or cardiomyopathy, CMR can be useful for diagnosis or management.16–23 2a B-NR 6. In patients with HF, an evaluation for possible ischemic heart disease can be useful to identify the cause and guide management.24–27 2b B-NR 7. In patients with HF and coronary artery disease (CAD) who are candidates for coronary revascularization, noninvasive stress imaging (stress echocardiography, single-photon emission CT [SPECT], CMR, or positron emission tomography [PET]) may be considered for detection of myocardial ischemia to help guide coronary revascularization.28–32 3: No Benefit C-EO 8. In patients with HF in the absence of: 1) clinical status change, 2) treatment interventions that might have had a significant effect on cardiac function, or 3) candidacy for invasive procedures or device therapy, routine repeat assessment of LV function is not indicated. Synopsis Cardiac imaging has a key role in the initial evaluation of individuals with suspected HF and, when indicated, in the serial assessment of patients with HF. After a complete history and physical examination, a comprehensive TTE is the most useful initial diagnostic test given the vast amount of diagnostic and prognostic information provided. The determination of LVEF is a fundamental step to classify HF and to guide evidence-based pharmacological and device-based therapy. In certain situations, the echocardiogram is unable to accurately assess cardiac structure and/or function or more information is needed to determine the cause of the cardiac dysfunction. Other imaging modalities, such as CMR, SPECT or radionuclide ventriculography, PET, or cardiac CT or invasive coronary angiography, can provide additional and complementary information to cardiac ultrasound.11 In general, cardiac imaging tests, including repeat tests, are performed only when the results have a meaningful impact on clinical care. Recommendations for Evaluation With Cardiac Imaging (Continued) COR LOE Recommendations Downloaded from http://ahajournals.org by on October 7, 2026