Surgical Management of a Left Main Coronary Trunk-Adjacent Functional Cardiac Paraganglioma Using Proactive Coronary
Kenichi Morimoto1, Yuichiro Kishimoto1, Takeshi Onohara1
1Department of Cardiovascular Surgery, Tottori University Faculty of Medicine, Yonago, Tottori, Japan.
Introduction:
Cardiac paragangliomas are exceptionally rare and often functional tumors that frequently arise near the aortic root and proximal coronary arteries. Complete excision is the only potentially curative treatment, but lesions adjacent to the left main coronary trunk (LMT) pose a major surgical dilemma: achieving oncologic radicality while preserving coronary perfusion and controlling massive bleeding.
Case Presentation:
A 33-year-old man presented with episodic postprandial chest/abdominal pain, paroxysmal hypertension, and cold sweating. Imaging revealed a hypervascular cardiac mass located between the ascending aorta and main pulmonary artery, extending to the left atrial roof; coronary angiography demonstrated tumor-feeding branches from the left anterior descending artery (LAD) and right coronary artery. After preoperative α-adrenergic blockade with doxazosin, surgery was performed via median sternotomy with cardiopulmonary bypass (CPB). Given the tumor's proximity to the LMT and the anticipated risk of compromised coronary perfusion to achieve macroscopic complete resection, planned coronary artery bypass grafting was performed before tumor excision. Under cardioplegic arrest, both the ascending aorta and main pulmonary artery were transected for exposure. The LMT and LAD were preserved, whereas the left circumflex artery coursed through the tumor and was sacrificed. En bloc resection including part of the left atrial roof was required, followed by bovine pericardial patch reconstruction. Diffuse massive bleeding from the left atrial patch suture line and the dissection surface required a second CPB run for hemostasis, and recurrent ventricular tachycardia/fibrillation after weaning from CPB necessitated temporary rescue peripheral veno-arterial extracorporeal membrane oxygenation, which was weaned off on POD3. Postoperative catecholamine levels normalized, and 123I-metaiodobenzylguanidine scintigraphy demonstrated no abnormal uptake at the cardiac operative site, consistent with complete resection.
Conclusions:
For functional cardiac paragangliomas adjacent to the LMT, integrating planned revascularization before tumor manipulation can provide a myocardial "safety net" that enables oncologically oriented en bloc resection when coronary sacrifice becomes unavoidable. This operation carries an exceptionally high risk of massive bleeding; therefore, meticulous hemostatic planning and preparedness-including a low threshold for prompt re-institution of CPB-may be crucial for the safe completion of radical resection.
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