Intracardiac paraganglioma in the left atrium in the context of a hereditary syndrome due to SDHD mutation: a case report

Cardiac paragangliomas are extremely rare, accounting for less than 0.3% of cardiac tumours. Their management may become a clinical challenge.

We present a case of a woman with an adrenergic crisis due to a cardiac paraganglioma in the context of a familial inherited paraganglioma syndrome. For the diagnosis, it was necessary to integrate different imaging techniques, such as echocardiogram, MRI, or PET-CT. A percutaneous embolization of the tumour and an adrenergic pharmacological blockade for a successful surgery was made.

This case shows the importance of the use of different imaging techniques for the diagnosis of cardiac tumours, integrating MRI with nuclear medicine functional test. Due to the high haemorrhagic risk of cardiac paragangliomas, a percutaneous embolization prior to surgery was carried out to avoid haemorrhagic complications. The cardiac location of paragangliomas is highly related with inherited familiar syndromes.