Pheochromocytoma/paraganglioma-associated cardiomyopathy

Pheochromocytoma/paraganglioma (PPGL) are neuroendocrine tumors that frequently produce and release catecholamines. Catecholamine excess can manifest in several cardiovascular syndromes, including cardiomyopathy. PPGL-induced cardiomyopathies occur up to 11% of cases most often associated with an adrenal pheochromocytoma (90%) rarely a paraganglioma derived from the sympathetic ganglia (10%). PPGL-associated be chronic or acute, takotsubo cardiomyopathy being reported. These two types seem have different pathophysiological backgrounds. Acute catecholaminergic stress inundates myocardial β-adrenoceptors leads left ventricle stunning slight histological apoptosis. In cardiomyopathy, prolonged catecholamine exposure extended fibrosis, inflammation, necrosis, ultimately it causes dilated low ejection fraction. Sometimes, especially hypertension, hypertrophic develop. The prognosis appears worse higher hospital mortality rate, cardiogenic shock rate at initial presentation, lower ventricular recovery after surgery. Therefore, establishing correct diagnosis early stage PPGL is essential. This mini-review summarizes current data on pathways cardiac damage caused by catecholamines, clinical presentation cardiomyopathies, discusses treatment options.