School of Medicine, National Yang-Ming University.
Division of Cardiovascular Surgery, Department of Surgery, Taipei Veterans General Hospital.
Int Heart J. 2022;63(4):786-792. doi: 10.1536/ihj.18-241.
Cardiac paragangliomas are exceedingly rare. Herein, we describe a patient with a large dopaminesecreting cardiac paraganglioma who had a history of pheochromocytoma after right adrenalectomy. The cardiac surgery was uneventful and without blood pressure fluctuations.The measurement of plasma-free metanephrines or urinary fractionated metanephrines is used as an initial screening test for pheochromocytoma or paraganglioma detection. However, these results must be combined with those of a plasma 3-methoxytyramine test to accurately establish the rare dopaminergic phenotype of pheochromocytomas or paragangliomas, if suspected. F-FDOPA (6-[18F]-L-fluoro-L-3, 4-dihydroxyphenylalanine)-based positron emission tomography (PET) and PET-computed tomography are relatively sensitive and specific; therefore, these techniques are recommended for patients with pheochromocytomas or paragangliomas before operation or during postoperative follow-up.
心脏副神经节瘤极为罕见。本文报道了 1 例右肾上腺嗜铬细胞瘤切除术后患者，其患有大量多巴胺分泌型心脏副神经节瘤。心脏手术顺利，血压无波动。检测血浆游离甲氧基肾上腺素或尿分馏甲氧基肾上腺素可作为嗜铬细胞瘤或副神经节瘤检测的初始筛查试验。然而，如果怀疑存在罕见的多巴胺能表型，则必须将这些结果与血浆 3-甲氧基酪胺试验相结合，以准确建立嗜铬细胞瘤或副神经节瘤。基于 F-FDOPA（6-[18F]-L-氟-L-3,4-二羟基苯丙氨酸）的正电子发射断层扫描（PET）和 PET-计算机断层扫描具有相对较高的灵敏度和特异性；因此，这些技术推荐用于手术前或术后随访期间的嗜铬细胞瘤或副神经节瘤患者。
