This case highlights the surgical challenges and potential electrophysiological complications, such as SA node dysfunction, associated with the resection of cardiac paragangliomas involving coronary anatomy.
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Warrants syndromic evaluation in young patients with cardiac PGL; leaves open optimal surgical and surveillance strategies.
Cardiac paraganglioma (PGL) is a rare catecholamine-secreting tumor forming 1% to 3% of cardiac tumors. Although most PGL occur sporadically, evidence exists that 40% of them may be related to familial cancer predisposition syndromes. We present a unique case of a 14-year-old female who presented with persistent hypertension and was found to have a cardiac PGL. During surgical resection, even though the main right coronary was preserved, the sinoatrial (SA) nodal artery was surrounded by the tumor and required resection with the tumor. The patient subsequently developed SA node dysfunction and is currently being evaluated for placement of a permanent pacemaker.
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Bansal et al. (2019) studied this question.
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