Gruber Lucinda M, Young William F, Bancos Irina
Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA.
Curr Cardiol Rep. 2021 May 7;23(6):60. doi: 10.1007/s11886-021-01485-4.
Pheochromocytoma and paraganglioma (PPGL) in pregnancy is a rare entity and management of these patients is fraught with uncertainty. Our objective is to review current literature and discuss diagnosis and management of these patients.
Outcomes of PPGL in pregnancy have improved in recent years. The greatest risk for adverse maternal and fetal outcomes is the diagnosis of PPGL after delivery. Alpha- and beta-adrenergic blockade is well tolerated and is associated with less adverse outcomes. Antepartum surgery is not associated with improved maternal or fetal outcomes. Biochemical testing and cross-sectional imaging should be performed prior to conception for patients with a known germline variant associated with PPGL.
Medical therapy should be initiated when PPGL is diagnosed in pregnancy. Antepartum surgery should be reserved for special circumstances. Case detection testing in high-risk patients can identify PPGL before pregnancy.
妊娠期间的嗜铬细胞瘤和副神经节瘤(PPGL)是一种罕见疾病,对这些患者的管理充满不确定性。我们的目的是回顾当前文献并讨论这些患者的诊断和管理。
近年来,妊娠期间PPGL的预后有所改善。分娩后诊断出PPGL是母婴不良结局的最大风险。α和β肾上腺素能阻滞剂耐受性良好,且不良结局较少。产前手术与改善母婴结局无关。对于已知与PPGL相关的种系变异患者,应在受孕前进行生化检测和横断面成像。
妊娠期间诊断出PPGL时应开始药物治疗。产前手术应保留用于特殊情况。对高危患者进行病例检测可在妊娠前识别PPGL。