Management of Hypertension in Intrapericardial Paraganglioma

Abstract

Functioning paraganglioma is extra-adrenal catecholamine-secreting tumours that may cause secondary hypertension. Primary intrapericardial paragangliomas are very rare and are located adjacent to the great vessels or heart, typically near the left atrium. These tumours are an exceptionally uncommon finding during the investigation of refractory hypertension. However, in recent years, intrapericardial paragangliomas have been diagnosed incidentally with increased frequency, due to the extensive use of radiologic chest imaging. The mainstay of treatment of functioning intrapericardial paraganglioma is surgical removal, which usually achieves blood pressure normalization. Due to the locations of these tumours, the surgical approach is through a median sternotomy or posterolateral thoracotomy, and manipulation-induced catecholamine release may cause paroxysmal hypertension. Typically in these patients, blood pressure fluctuates dramatically intra- and post-operatively, increasing the risk of cardiovascular complications. We review here the current modalities of perioperative fluid and hypotensive drug administration in the setting of surgery for functioning intrapericardial paraganglioma and discuss the recently proposed paradigm shift that omits preoperative preparation.

Paraganglioma of the carotid body and intrapericardium

“…About 20% of cardiac PGLs are functionally inactive [7], and in cases such as with the present patient, the tumor is found incidentally. It is difficult to resect surgically pericardial paraganglioma because of the risk of bleeding caused by the tumor's high vascularity, and there were few useful data obtained from the symptoms and routine blood and urine tests.…”

Role of Multimodality Imaging and Preoperative Management in Intrapericardial Paragangliomas

“…Intrapericardial PGLs are extremely rare tumors and are most often located adjacent to the left atrium. 1 They can be functional and secrete catecholamines in up to 80% of cases. 1 …”

“…On the other hand, around 20% of intrapericardial PGLs are nonfunctioning and diagnosed due to mass effect (eg, chest/back pain, cough, hoarseness) or metastatic disease, or incidentally on imaging. 1 Owing to the distinctive features of PGLs including vascularity and location, imaging characteristics are often enough for presumptive diagnosis before proceeding with surgical resection. 3 On CT scan, findings include intense enhancement with contrast and delayed washout.…”

“…Intrapericardial PGLs are extremely rare. 1 These tumors are highly vascular and frequently adhere to adjacent structures, rendering surgical resection challenging. 2 Therefore, intrapericardial PGLs often necessitate cardiopulmonary bypass (CPB) for surgical resection.…”

The Intra-Pericardial Paraganglioma Presenting as Ascites and Hemopericardium with Impending Tamponade

“…In the literature, most cardiac paragangliomas are large, ranging in size from 3 to 8 cm and about 20% of them are functionally inactive. 2) Most patients with functionally-inactive cardiac paraganglioma present with chest pain and dysphagia due to obstruction. It is rare to present with symptoms and signs, such as heart failure and hemopericardium.…”