Key result
Right atrial appendage thrombus causes severe pulmonary embolism in idiopathic restrictive cardiomyopathy.
Why the study?
Idiopathic restrictive cardiomyopathy is rare and its natural history is not well known.
Case Report (n=1)
No
In patients with restrictive hemodynamic patterns, the right atrium should be routinely assessed for thrombi to prevent severe complications like pulmonary thromboembolism.
Hypothesis-generating for right atrial thrombus screening in restrictive cardiomyopathy; prospective studies needed before routine adoption.
Idiopathic restrictive cardiomyopathy (RCM) is rare, and its natural history is not well known. Its prognosis in infants is extremely poor, whereas patients with RCM occurring in middle age have comparatively good prognoses. Here, we report a case of idiopathic RCM with the disease onset at 10 years old. Echocardiography and cardiac catheterization revealed a biventricular restrictive pattern; however, the right ventricle showed more severe restriction. At 20 years old, severe pulmonary thromboembolism (PTE) occurred with circulatory collapse. The right atrium was extremely enlarged and the appendage was filled with moderate thrombi that migrated to pulmonary arteries. PTE is a rare complication of idiopathic RCM; however, this complication occurs more commonly in other secondary RCMs. In patients with restrictive hemodynamic pattern, the presence of thrombi in cardiac cavities should be routinely examined.<Learning objective: A patient with idiopathic restrictive cardiomyopathy, with disease onset at 10 years old, is described. Echocardiography and cardiac catheterization revealed biventricular restrictive pattern, however right ventricle showed more severe restriction. At 20 years old, he had severe pulmonary thromboembolism. The right atrial appendage was filled with moderate thrombi that migrated to pulmonary arteries. The right atrium should be assessed to determine the presence of thrombi in patients with restrictive hemodynamic pattern.>
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Yamamoto et al. (2020) conducted a case report in Idiopathic restrictive cardiomyopathy (n=1). Idiopathic restrictive cardiomyopathy was evaluated on Clinical course and complications. A patient with idiopathic restrictive cardiomyopathy developed severe pulmonary thromboembolism originating from right atrial appendage thrombi, demonstrating that thrombi should be routinely assessed in patients with restrictive hemodynamic patterns.
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