Istratoaie Sabina, Kovacs Emese, Manole Simona, Inceu Andreea Ioana, Axente Dan Damian, Bungărdean Raluca Maria, Șerban Adela Mihaela
Department of Pharmacology, Toxicology, and Clinical Pharmacology, "Iuliu Haţieganu" University of Medicine and Pharmacy, 400337 Cluj-Napoca, Romania.
Department of Cardiology, "Niculae Stăncioiu" Heart Institute, 400001 Cluj-Napoca, Romania.
J Clin Med. 2023 Jul 14;12(14):4694. doi: 10.3390/jcm12144694.
Paraganglioma is a rare neuroendocrine tumor derived from chromaffin cells. The overproduction of catecholamines accounts for the presenting symptoms and cardiovascular complications. The clinical presentation frequently overlaps with the associated cardiac diseases, delaying the diagnosis. Multimodality imaging and a multidisciplinary team are essential for the correct diagnosis and adequate clinical management.
A 37-year-old woman with a personal medical history of long-standing arterial hypertension and radiofrequency ablation for atrioventricular nodal reentry tachycardia presented with progressive exertional dyspnea and elevated blood pressure values, despite a comprehensive pharmacological treatment with six antihypertensive drugs. The echocardiography showed a bicuspid aortic valve and severe aortic regurgitation. The computed tomography angiography revealed a retroperitoneal space-occupying solid lesion, with imaging characteristics suggestive of a paraganglioma. The multidisciplinary team concluded that tumor resection should be completed first, followed by an aortic valve replacement if necessary. The postoperative histopathology examination confirmed the diagnosis of paraganglioma. After the successful resection of the tumor, the patient was asymptomatic, and the intervention for aortic valve replacement was delayed.
This was a rare case of a late-detected paraganglioma in a young patient with resistant hypertension overlapping the clinical presentation and management of severe aortic regurgitation. A multimodality imaging approach including transthoracic and transesophageal echocardiography, computed tomography, and magnetic resonance imaging had an emerging role in establishing the diagnosis and in guiding patient management and follow-up. The resection of paraganglioma was essential for the optimal timing of surgical correction for severe aortic regurgitation. We further reviewed various cardiovascular complications induced by pheochromocytomas and paragangliomas.
副神经节瘤是一种罕见的起源于嗜铬细胞的神经内分泌肿瘤。儿茶酚胺分泌过多导致了其出现的症状和心血管并发症。临床表现常与相关心脏疾病重叠，从而延误诊断。多模态成像和多学科团队对于正确诊断和恰当的临床管理至关重要。
一名37岁女性，有长期动脉高血压病史，曾因房室结折返性心动过速接受射频消融治疗，尽管使用了六种抗高血压药物进行全面药物治疗，但仍出现进行性劳力性呼吸困难和血压升高。超声心动图显示二叶式主动脉瓣和严重主动脉瓣反流。计算机断层血管造影显示腹膜后占位性实性病变，影像学特征提示为副神经节瘤。多学科团队得出结论，应首先完成肿瘤切除，必要时随后进行主动脉瓣置换。术后组织病理学检查确诊为副神经节瘤。肿瘤成功切除后，患者无症状，主动脉瓣置换干预被推迟。
这是一例在患有难治性高血压的年轻患者中晚期发现副神经节瘤的罕见病例，其临床表现和管理与严重主动脉瓣反流重叠。包括经胸和经食管超声心动图、计算机断层扫描和磁共振成像在内的多模态成像方法在确立诊断以及指导患者管理和随访方面发挥着越来越重要的作用。切除副神经节瘤对于严重主动脉瓣反流手术矫正的最佳时机至关重要。我们进一步回顾了嗜铬细胞瘤和副神经节瘤引起的各种心血管并发症。
