Vnitr Lek 2024, 70(1):17-21 | DOI: 10.36290/vnl.2024.003

Catecholamine induced cardiomyopathies in patients with pheochromocytoma

Zora Lazúrová

IV. interná klinika UPJŠ LF a UNLP, Košice

Pheochromocytoma/paraganglioma (PPGL) is a rare neuroendocrine tumour arising from chromaffine cells of sympathetic ganglia. 90% of PPGLs are located in adrenal medulla (pheochromocytoma), the rest are extraadrenal paragangliomas. PPGLs produce catecholamines - adrenaline and/or noradrenaline, in case of extraadrenal forms, dopamine may be rarely secreted. An overproduction of these hormones usually manifests by the cardiovascular diseases, mostly with arterial hypertension. In 8-11% of patients, catecholamine excess leads to heart injury. Katecholamine-induced cardiomyopathies (CICMP) are severe, potentially fatal complications of PPGLs. Clinical manifestation may vary, from asymptomatic forms up to severe heart failure with pulmonary edema or cardiogenic shock. Three types of CICMPs associated with PPGL have been identified. Acute, takotsubo-like cardiomyopathy, typical for adrenalin producing tumours, and two types of chronic forms - dilated cardiomyopathy and hypertrophic cardiomyopathy, the last one typical for noradrenalin-secreting PPGLs. Early diagnosis and appropriate management leads to significant improvement in most of the patients. Though receidiving forms have worse prognosis.

Keywords: pheochromocytoma, cardiomyopathy, catecholamines, heart failure.

Accepted: February 9, 2024; Published: February 26, 2024  Show citation