Background: Cardiac amyloidosis refers to the extracellular deposition of fibrils in cardiac tissue resulting in restrictive cardiomyopathy. There has been reported that various delayed hyperenhancement (DHE) patterns appeared in cardiac magnetic resonance imaging (CMR) in cardiac amyloidosis. However, the data regarding relationship between the patterns of DHE and clinical parameters of the patients are lacking. We evaluated the relationships between the pattern of DHE and left ventricular function in the patients with biopsy-proven cardiac amyloidosis. Methods: Among 40 patients who diagnosed as cardiac amyloidosis by positive Congo red stain in cardiac tissue, 32 (80%) patients had evaluated with both CMR and echocardiography. The patients were grouped according to the pattern of DHE (transmural versus non-transmural DHE) and we compared the CMR and echocardiographic parameters as well as survival between groups. Results: Seventeen patients showed global transmural DHE pattern and the other 15 patients showed diffuse subendocardial (DHE thickness less than 50% of myocardium) or patchy DHE in CMR. Left ventricular ejection fraction was not different between groups when evaluated by echocardiography (48짹13% vs 51짹11%, p= 0.526) and by CMR (46짹19% vs 45짹14%, p=0.959), respectively. However, the diastolic function parameters in echocardiography was worse in the patients with global trasmural DHE; E/A was significantly higher (3.4짹1.3 vs 2.1짹1.8, p=0.020) and deceleration time was shorter (150짹34 msec vs 233짹138 msec, p=0.015). During follow-up periods (30.3 짹 23.9 months), overall survival rate was not significantly different between groups. Conclusions: The pattern of DHE was related with diastolic function rather than systolic parameters. Large scale study is warranted for the evaluation of relationship between DHE and long-term survival.