Surgical excision with left atrial reconstruction of a primary functioning retrocardiac paraganglioma.
About 2% of all paragangliomas are located in the chest, and a few have been described to be found in the heart. Primary cardiac paragangliomas are extremely uncommon tumors and surgical experience with this neoplasm is limited. Treatment strategies described in the literature have included simple excision, excision with reconstruction, autotransplantation after excision of the tumor and even orthotopic cardiac transplantation, depending on the extent of disease. A primary retrocardiac paraganglioma catecholamine-productive was identified in an asymptomatic 49-year old female associated to familial pheochromocytoma-paraganglioma syndrome caused by germline mutation of the gen which codifies for the subunit B of succinate dehydrogenase enzyme (SDHB). The neoplasm was surgically excised from the posterior surface of the left atrium via median sternotomy using cardiopulmonary bypass. Direct ligation of feeding vessels of the tumor along with left atrial reinforcement using a pericardial patch was performed. The post-operative course was uneventful, with normalization of catecholamine secretion and no recurrence at three-month follow-up. We review the current literature about this exceptional cardiac tumor, pathophysiological conditions and options for surgical management.
Cardiovascular Surgery Department, Carlos Haya Regional Hospital, Carlos Haya Avenue, s/n, 29010 Málaga, Spain. draglezlopez@hotmail.com
María Teresa
MT
González López
Sergio González
SG
González
Esteban Sarria
ES
García
Stella González
SG
Romero
Julio Gutiérrez
JG
de Loma
D006348:Cardiac Surgical Procedures / Q000379:methods*; D002315:Cardiopulmonary Bypass; D002395:Catecholamines / Q000097:blood; D005260:Female; D006325:Heart Atria / Q000601:surgery*; D006338:Heart Neoplasms / Q000601:surgery*; D006801:Humans; D008875:Middle Aged; D010235:Paraganglioma / Q000097:blood / Q000601:surgery*; D019651:Plastic Surgery Procedures / Q000379:methods*; D056346:Sternotomy
D002395:Catecholamines