Approach to additional testing in patients without a suspected cause of restrictive cardiomyopathy

RCM: restrictive cardiomyopathy; CMR: cardiovascular magnetic resonance; LGE: late gadolinium enhancement; CIED: cardiac implantable electronic device; PYP: 99mTc-labeled pyrophosphate; DPD: 99mTc-labeled 3,3-diphosphono-1,2-propanodicarboxyclic acid; HMDP: 99mTc-labeled hydroxymethylene diphosphonate; EMB: endomyocardial biopsy.
* Refer to UpToDate content for the initial evaluation and etiologies of RCM.
¶ Relative contraindications to CMR imaging with LGE include the presence of ferromagnetic material in the body and severe kidney dysfunction. The presence of a CIED typically results in nondiagnostic CMR images. Refer to UpToDate content on patient evaluation prior to CMR imaging and gadolinium administration for further details.
Δ Refer to UpToDate content for details on the diagnostic evaluation of cardiac sarcoidosis, amyloidosis, and infiltrative cardiomyopathies.
◊ We obtain a PYP scan, if available. If a PYP scan is not available, alternatives include DPD and HMDP scans.
§ Given the large number of pathologic gene variants associated with RCM, we refer patients for genetic counseling or consultation with a geneticist to help guide the selection and interpretation of genetic testing.
¥ Patients whose EMB does not reveal an identifiable cause of RCM, or those who do not undergo EMB, are considered to have idiopathic RCM.