Cardiac hydatid cyst is a rare disease with frequency of less than 2%. The left ventricle is the most common site. Symptoms depend upon site of involvement ranging from asymptomatic to acute coronary syndrome, arrhythmias, valvular lesions, etc. It is a surgical emergency, therefore early diagnosis and surgical treatment is required even in asymptomatic patients. Surgical excision on beating heart along with preparedness with cardiopulmonary bypass and fibrillator via left anterolateral thoracotomy can be done successfully, followed by medical treatment with albendazole. Increased awareness and high degree of suspicion among cardiac physicians in the endemic area is essential for early diagnosis. Echocardiography remains the gold standard for diagnosis and follow-up of patient.
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