Primary cardiac haemangioendothelioma is an uncommon vascular neoplasm with an infiltrative growth pattern and malignant potential. The treatment of this tumour alone is challenging, but the symptomatic presentation during late pregnancy creates a complex clinical situation for both the mother and physician. To the best of our knowledge, <40 cases of primary cardiac haemangioendothelioma have been reported worldwide, but none of these were associated with pregnancy. We report a case of symptomatic primary haemangioendothelioma in the right atrium of a pregnant woman in the 36th week of gestation. A primary caesarean section, followed by complete resection of the cardiac tumour, was carried out without any complications. The mother and child were discharged home 5 days after the surgical procedure and birth, respectively. At the 2-month follow-up, positron emission tomography showed signs of hypermetabolic activity in the right atrium, without signs of tumour recurrence on the subsequent computed tomography and magnetic resonance imaging, thus a close clinical follow-up was recommended.
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