Three of the leading causes …  |  Thus the heart is restricted from stretching and filling with blood properly. In this review, an overview of RCMs will be presented followed by a detailed discussion on 3 major causes of RCM, for which tailored interventions are available: cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis. Biventricular chamber size and systolic function are usually normal or near-normal until later stages of the disease. The prevalence of ATTRm CA is not known, reflecting both the global variation in mutation prevalence and disease awareness. Among the women enrolled in the Nurse Health Study II, the average annual incidence rate was 11 per 100 000.55 The incidence rate increased with age from 9 per 100 000 in women aged <35 years to 15 per 100 000 in women aged ≥55 years. The pattern of LGE can be global or focal, subendocardial or transmural. Figure 4. Subcutaneous fat pad aspirate is positive in almost half of the patients although the yield is higher in AL>ATTRm>ATTRwt (80% versus 67% versus 14%, respectively).19,20 When a monoclonal protein is found in blood\urine studies or if the serum free LC assay is abnormal, suggesting AL amyloidosis, a bone marrow biopsy performed for the evaluation of plasma cell dyscrasia is an additional tissue source for Congo red staining, and when coupled with fat aspirate, is diagnostic in 85% to 90%.20a When these tissues fail to demonstrate amyloid deposits, EMB is a rational approach, but other soft tissue sources can still be biopsied (lip, rectum) if EMB is deemed unsafe. The heart, liver, spleen, nervous system, bone marrow, kidneys, bones, joints, muscles, and other organs may also be involved. ApoA-I amyloidosis, caused by a mutation in the APOA1 gene, should also be considered in the differential diagnosis of CA although infrequently seen. RV aneurysms are occasionally observed. If this cannot be achieved within 2 to 3 months of initial treatment, an alternative regimen is warranted. In 1 study of 58 patients with suspected CS, LGE had a sensitivity of 100% and a specificity of 78% compared with the JMWH criteria.76 The low specificity likely reflects the poor sensitivity of the JMHW criteria and the ability of CMR to detect small areas of scarring. Newer T2 mapping techniques may overcome some of the limitations of current techniques and provide an additional method to identify and quantify myocardial inflammation. Cardiac involvement in AL amyloidosis can be seen without thickened walls, so awareness for amyloidosis and screening for serum free LCs is required when seeing a patient with heart failure and preserved LVEF, regardless of wall thickness. The primary site of involvement is the myocardium, clinically manifesting with conduction defects and arrhythmias. Courtesy of Martha Grogan, MD, Mayo Clinic, Rochester, MN. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Most importantly, these guidelines have not been clearly validated against a reference standard. These systems may prove to especially helpful in the future to assess response to immunosuppressive therapy, but need to be systematically studied before routine use in clinical practice is indicated. IMiD-treated patients frequently experience a rise of NT-proBNP/BNP, which is usually asymptomatic, but was reported to be associated with a shortened survival.31 It is unclear whether the biomarker rise indicates direct cardiac toxicity, fluid retention, or impaired renal clearance. The rational for use of CLEAR is based on evidence that some cases of sarcoidosis may be caused by an unidentified mycobacterium and that fluoroquinolones and macrolides both have anti-inflammatory properties. Cardiac response was achieved in 57%, with no organ progression seen.35 A phase 3 trial is currently recruiting, evaluating the efficacy and safety of NEOD001 plus standard chemotherapy versus placebo and standard chemotherapy in untreated AL amyloidosis with cardiac involvement.36. Torsemide has better bioavailability than furosemide and a longer half-life. The Mayo eligibility criteria for autologous stem cell transplantation are reported.27 It is reasonable in patients with poor organ function to initiate induction chemotherapy in anticipation of improvement in organ function, which then might restore eligibility to autologous stem cell transplantation. Cardiac positron emission tomography enhances prognostic assessments of patients with suspected cardiac sarcoidosis. Conduction abnormalities, but some of these 3 RCMs is challenging to diagnose CS: 10.1016/j.tcm.2017.07.004 clinical,! 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