ABSTRACT

Restrictive cardiomyopathy is a heart muscle disease characterized by diastolic filling dysfunction, reduced end diastolic volume with normal ventricular systolic function and normal or near-normal wall thickness. Idiopathic restrictive cardiomyopathy is the least commonly observed reason of childhood cardiomyopathies. Restrictive cardiomyopathy has a variable level of atrial dilatation, and atrial thrombus is more frequently seen in cases with marked biatrial dilatation. The thrombus can be seen in both of the atriums, but generally occurs in the appendix by little size and its course is usually asymptomatic. Risk of atrial thrombus is increased in patients who have restrictive cardiomyopathy with biatrial dilatation. Idiopathic restrictive cardiomyopathy rarely causes giant atrial thrombus in children. We describe a very rare pediatric case who developed giant atrial thrombus, and herein summarize restrictive cardiomyopathy and its complications.