Cardiac echinococcosis is a rare manifestation of cystic echinococcosis (CE) caused by the tapeworm Echinococcus granulosus. Among all patients suffering from CE, only 0.5%–2% exhibit a cardiac involvement.

Diagnosis of cardiac echinococcosis requires a detailed evaluation of the patients’ case history, specific laboratory analyses and radiological imaging methods. Ultrasound, MRI and CT are key imaging tools for diagnosis, therapy control, prognosis estimation and disease course control. For the therapy of cardiac echinococcosis, a combination of surgical removal and drug treatment should be applied to symptomatic as well as asymptomatic patients. The complete surgical removal of the cyst(s) is the major prognosis factor of the cardiac manifestation of CE.