The Role of Extracorporeal Membrane Oxygenation Support in Early Management of Unexplained Life-Threatening Acute Heart Failure Due to Left Atrial Cardiac Paraganglioma

Paragangliomas/pheochromocytomas are uncommon neuroendocrine tumors that arise from chromaffin cells located outside of the adrenal gland. Although cardiac paragangliomas have been observed in all heart chambers, the most prevalent are left-atrial paragangliomas, followed by aortic body tumors. Diagnosis of paragangliomas/pheochromocytomas is mostly achieved with a multimodality approach because of her clinical presentation ranging from incidental findings to refractory acute heart dysfunction. The role of extracorporeal membrane oxygenation support in the early management and diagnosis of unexplained life threatening cardiogenic shock is rapidly increasing worldwide. However, its clinical utility remains still unclear in intractable heart failure due to primary cardiac paraganglioma. We reported a case of a primary left atrial paraganglioma/pheochromocytoma measuring 34 mm at the maximum diameter in a 58-year-old male patient. The patient presented with acute cardiogenic shock, pulmonary edema, and bilateral stroke. Peripherical mechanical circulatory support, in veno-arterial mode, was rapidly instaured for early management in a life-threatening situation. After normal myocardial function recovery and accurate diagnosis, a surgical approach through aortic and pulmonary artery transection for radical tumor resection and left atrial wall reconstruction was performed. Postprocedural recovery and follow-up at six months were uneventful with excellent neurological recovery.