González López, María TeresaGonzález González, SergioSarria García, EstebanGonzález Romero, StellaGutiérrez de Loma, Julio2013-12-192013-12-192013-01-29González López MT, González González S, Sarria García E, González Romero S, Gutierrez de Loma J. Surgical excision with left atrial reconstruction of a primary functioning retrocardiac paraganglioma. J Cardiothorac Surg. 2013; 8:22http://hdl.handle.net/10668/1437Case Reports; Journal Article;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.enParagangliomaPheochromocytomaCardiac tumorLeft atriumCardiopulmonary bypassProcedimientos quirúrgicos cardíacosDerivación cardiopulmonarCatecolaminasAtrios cardíacosNeoplasias cardíacasProcedimientos quirúrgicos reparadoresMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Surgical Procedures, Operative::Extracorporeal Circulation::Cardiopulmonary BypassMedical Subject Headings::Chemicals and Drugs::Organic Chemicals::Amines::CatecholaminesMedical Subject Headings::Check Tags::FemaleMedical Subject Headings::Anatomy::Cardiovascular System::Heart::Heart AtriaMedical Subject Headings::Diseases::Neoplasms::Neoplasms by Site::Thoracic Neoplasms::Heart NeoplasmsMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Named Groups::Persons::Age Groups::Adult::Middle AgedMedical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Nerve Tissue::Neuroectodermal Tumors::Neuroendocrine Tumors::ParagangliomaMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Surgical Procedures, Operative::Reconstructive Surgical ProceduresMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Surgical Procedures, Operative::Thoracic Surgical Procedures::SternotomyMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Surgical Procedures, Operative::Cardiovascular Surgical Procedures::Cardiac Surgical Proceduresresearch article23360571open access10.1186/1749-8090-8-221749-8090PMC3599281