Table 1.
Common types of infiltrative cardiomyopathies.
| CONDITION | EPIDEMIOLOGY | PATHOLOGY | ECG | ECHOCARDIOGRAM | CMR | TREATMENT |
|---|---|---|---|---|---|---|
| Cardiac amyloidosis | 6th or 7th decade acquired (AL, SSA) or inherited (ATTR) | Extracellular amyloid fibrils | Low-voltage QRS; pseudoinfarction; AV block | LV and RV hypertrophy; granular speckled myocardium; restricted basal longitudinal strain | Global LGE (Also consider radionuclide scanning) | AL: chemotherapy (CyBordD); TTR: difflunisal/tafamidis; ± heart-liver transplant |
| Cardiac Sarcoidosis | 3rd or 4th decade; African Americans, northern Europeans, Japanese; female>male | Noncaseating granulomas surrounded by fibrosis | High-grade AV block | Septal thinning/thickening; noncoronary segmental wall motion abnormalities | Pathy LGE, predominantly LV free wall and basal septum (Also consider FDG-PET) | Corticosteroids, PPM/ICD; ± cardiac transplant |
| Hemochromatosis/IOC | 4th or 5th decade; inherited (primary, HFE mutation) or acquired (secondary) | Intracellular iron | Nonspecific repolarization abnormalities | Diastolic disease global systolic dysfunction(Image) | Shortened T2* time | Phlebotomy; chelation |
| Fabry Disease | 2nd through 5th decade X1 linked error of glycosphingolipid metabolism | Perinuclear vacuoles and myocardial fibrosis | Increased voltage QRS | Concentric LV hypertrophy | LGE of the basal segments of the anterolateral and inferolateral walls | Enzyme replacement |
| Danon Disease | 2nd or 3rd decade; inherited (LAMP2 deficiency) | Myocyte hypertrophy with vacuolization | Increased voltage QRS; short PR with delta wave | Massive LV hypertrophy with possible outflow tract obstruction | Sunbendocardial LGE sparing the septum | Supportive |
| Friedreich’s Ataxia | 2nd and 3rd decade; inherited (frataxin mutation) | Nonspecific myocyte hypertrophy and fibrosis | Nonspecific repolarization abnormalities | Increased septal thickness | Not used | Supportive |
Abbreviations: CMR, cardiac magnetic resonance; ECG, electrocardiography; IOC, iron overload cardiomyopathy; LGE, late gadolinium enhancement; LV, left ventricle; RV, right ventricle.