Pheochromocytomas originate from chromophores in the sympathetic nervous system. About 90% of pheochromocytomas occur in the adrenal medulla, about 10% occur outside the adrenal glands, and most are located in the abdominal cavity, while only 1%-2% of pheochromocytomas occur in the thoracic cavity, mostly in the posterior mediastinum. Pheochromocytomas originating in the heart are rare, but those originating in the heart originate from the paraganglia (atria or septum) and the visceral autonomic paraganglia, most often in the left atrium. Surgical resection is difficult because the tumor often involves the valve or coronary artery blood supply. Diagnostic strategy: Unlike adrenal pheochromocytoma, cardiac pheochromocytoma has a more aggressive growth pattern and generally has a poor prognosis, with onset ranging from extremely insidious to sudden death. Awareness and vigilance of the disease is the key to timely treatment. The diagnostic steps can be divided into four steps: clinical suspicion, qualitative diagnosis, local diagnosis and differential diagnosis. (1) Patients with clinical symptoms of increased catecholamine secretion such as palpitations, sweating and headache; (2) paroxysmal or persistent hypertension; (3) pheochromocytoma of multicenter origin; (4) occupying lesions in the middle mediastinum found at physical health with hypertension, palpitations and other uncomfortable symptoms. For such high-risk patients, comprehensive relevant examinations should be performed. The best qualitative diagnosis of pheochromocytoma depends on laboratory tests: there are at least 20 laboratory tests used to screen for pheochromocytoma. 99% of adrenal pheochromocytomas have higher than normal 24h urinary metanephrines or catecholamines, and most cardiac pheochromocytomas have significantly elevated blood or urinary catecholamines. Localization diagnosis In the localization diagnosis of pheochromocytoma: 131I-meso-iodobenzylguanidine (131I-MIBG) imaging (adrenal medullary imaging) is considered the test of choice for patients with suspected adrenal pheochromocytoma, but this test has a certain percentage of false negatives for ectopic, malignant or metastatic pheochromocytoma, while growth inhibitor receptor imaging (99mTc or 111In- octreotide) can reveal ectopic, malignant or metastatic pheochromocytoma that cannot be revealed by 131I-m-iodobenzylguanidine imaging. 4.Differential diagnosis: diseases that need to be differentiated from this disease include: hypertension with coronary artery disease; hypertension with paroxysmal tachycardia; hypertension with primary or secondary cardiac tumors, cardiac hemangioma, angiosarcoma, carcinoid tumor; these diseases can be differentiated by qualitative localization diagnostic methods. Perioperative management Most pheochromocytomas are benign, about 12% are malignant, but the probability of malignancy is significantly higher in ectopic pheochromocytomas, which can rise from 12% to 24%. The majority are located in the epicardium of the left atrial apex or posterior wall, other sites include the atrial septum, the atrial cavity, and rarely in the ventricles. Because of the malignant potential of cardiac pheochromocytoma, the degree of malignancy is determined by the long-term aggressive or metastatic nature of the disease, and therefore the principle of surgical treatment for cardiac pheochromocytoma is to remove it as radically as possible. The complexity of surgery is determined by this pathology, and depending on the tumor, surgical resection may include either tumor resection alone or simultaneous reconstruction of the cardiac structure, with consideration given to preparing the heart for transplantation if necessary before planning to remove the tumor. In the early stage of cardiac pheochromocytoma surgery, most of the operations were performed under lateral open-chest non-corporeal circulation, which resulted in large intraoperative hemodynamic fluctuations and the main cause of surgical death was hemorrhage. It helps to avoid and reduce large intraoperative hemodynamic fluctuations; it allows more complete resection of the tumor; it is conducive to structural reconstruction of the heart and avoids heavy bleeding. In our four patients, the hemodynamic changes were not as drastic as envisaged when the tumor was surgically removed under extracorporeal circulation, and the postoperative recovery was smooth. The choice of extracorporeal circulation for cardiac pheochromocytoma surgery is not absolutely necessary, but depends on the tumor site, scope and blood supply. Pheochromocytoma is a tumor characterized by the secretion of large amounts of catecholamines by chromophores, and cardiac pheochromocytoma is no exception. It is prone to violent hemodynamic fluctuations in the perioperative period, especially during anesthesia, which should be given attention and requires a smooth procedure throughout the operation. The intraoperative separation and extrusion can lead to a large amount of catecholamine release directly to the heart, resulting in drastic hemodynamic changes. It is required to make gentle movements during surgery to reduce the release of large amount of catecholamines into the blood caused by squeezing, and to closely observe the changes of vital signs and make timely and appropriate treatment. After tumor removal, catecholamines in patients’ bodies are suddenly reduced, so attention should be paid to supplementing catecholamine drugs during the perioperative period to strive for stable postoperative hemodynamics. The general condition was significantly improved when the drugs were discontinued or reduced after surgery, indicating that surgical resection of the tumor is an effective treatment for cardiac pheochromocytoma. Postoperatively, there was progression of high growth inhibitor receptor-expressing lesions in the original heart site, and it has been suggested that heart transplantation is the most effective treatment for cardiac pheochromocytoma, but routine resection of cardiac tumors is still a problem to be considered in cardiac surgery due to the difficulty of donor availability, which requires future experience. Heart transplantation is an effective treatment for patients with cardiac pheochromocytoma that cannot be directly resected by surgery.