Intracardiac Paraganglioma in the Left Atrium in the context of a Hereditary Syndrome due to SDHD Mutation: A Case Report

Abstract Background Cardiac paragangliomas are extremely rare accounting less than 0.3% of cardiac tumors. Their management may become in a clinical challenge. Case summary We present a case of a woman with adrenergic crisis due to a cardiac paraganglioma in the context of a familiar inherited paraganglioma syndrome. For the diagnosis, it was needed to integrate different imaging techniques, such as echocardiogram, MRI or PET-CT. A percutaneous embolization of the tumor and an adrenergic pharmacological blockade for a successful surgery was made. Discussion This case shows the importance of the use of different imaging techniques for the diagnosis of cardiac tumors, integrating MRI with nuclear medicine functional test. Due to the high hemorrhagic risk of cardiac paragangliomas, a percutaneous embolization prior to surgery was carried out in order to avoid hemorrhagic complications. The cardiac location of paragangliomas is highly related with inherited familiar syndromes.