nWisconsin Heart Hospital, Milwaukee, Wisconsin; Nuclear cardiology expert. oJohns Hopkins Medical Institute, Baltimore, Maryland. pSpecialty Chair, UT Southwestern Medical Center, Dallas, Texas. Reprint requests to: publications@acr.org Nonischemic Myocardial Disease treated with chelation therapy; cardiac sarcoidosis is treated with high-dose corticosteroids; cardiac amyloidosis is treated with chemotherapy for light-chain amyloidosis (AL type) and novel therapies for transthyretin type; Fabry disease is treated with enzyme replacement therapy; and severe HCM or endomyocardial fibrosis is treated with surgery [2]. An endomyocardial biopsy may be required for definitive diagnosis in some cases; however, it is an invasive procedure and the yield may be low because of the patchy nature of disease processes. In unexplained cardiomyopathy, the final diagnosis based on biopsy differed from initial diagnosis in 31% of patients, and endomyocardial biopsy made the final diagnosis in 75% of these cases [9]. Imaging is also helpful for quantification of the disease process, risk stratification, prognosis, and monitoring response to therapy. All elements are essential: 1) timing, 2) reconstructions/reformats, and 3) 3-D renderings. Standard CTs with contrast also include timing issues and reconstructions/reformats. Only in CTA, however, is 3-D rendering a required element. This corresponds to the definitions that the CMS has applied to the Current Procedural Terminology codes. Chest Radiography Chest radiography can provide information on HF and vascular abnormalities; however, there is no specific role for radiography in characterizing the different types of NICM. Echocardiography Echocardiography provides information on ventricular function (global/regional, systolic/diastolic), volumes, mass, thickness, as well as valvular function.
Nonischemic Myocardial Disease with Clinical Manifestations Ischemic Cardiomyopathy Already Excluded . nWisconsin Heart Hospital, Milwaukee, Wisconsin; Nuclear cardiology expert. oJohns Hopkins Medical Institute, Baltimore, Maryland. pSpecialty Chair, UT Southwestern Medical Center, Dallas, Texas. Reprint requests to: publications@acr.org Nonischemic Myocardial Disease treated with chelation therapy; cardiac sarcoidosis is treated with high-dose corticosteroids; cardiac amyloidosis is treated with chemotherapy for light-chain amyloidosis (AL type) and novel therapies for transthyretin type; Fabry disease is treated with enzyme replacement therapy; and severe HCM or endomyocardial fibrosis is treated with surgery [2]. An endomyocardial biopsy may be required for definitive diagnosis in some cases; however, it is an invasive procedure and the yield may be low because of the patchy nature of disease processes. In unexplained cardiomyopathy, the final diagnosis based on biopsy differed from initial diagnosis in 31% of patients, and endomyocardial biopsy made the final diagnosis in 75% of these cases [9]. Imaging is also helpful for quantification of the disease process, risk stratification, prognosis, and monitoring response to therapy. All elements are essential: 1) timing, 2) reconstructions/reformats, and 3) 3-D renderings. Standard CTs with contrast also include timing issues and reconstructions/reformats. Only in CTA, however, is 3-D rendering a required element. This corresponds to the definitions that the CMS has applied to the Current Procedural Terminology codes. Chest Radiography Chest radiography can provide information on HF and vascular abnormalities; however, there is no specific role for radiography in characterizing the different types of NICM. Echocardiography Echocardiography provides information on ventricular function (global/regional, systolic/diastolic), volumes, mass, thickness, as well as valvular function.