Catecholamine-Induced Cardiomyopathy in Pheochromocytoma: How to Manage a Rare Complication in a Rare Disease?

Abstract

Pheochromocytomas and paragangliomas (PHEOs) are rare neuroendocrine tumors. Clinical manifestations include different cardiovascular signs and symptoms, which are related to excessive secretion of catecholamines. Catecholamine-induced cardiomyopathy in PHEO (CICMPP) is a rare but dreaded complication of PHEO. Once patient is diagnosed with this condition, the prognosis is worse and a surgical risk is much higher than expected. This article focuses on how catecholamines affect the heart and the pathophysiologic mechanism of CICMPP. The cardiovascular responses to catecholamine depend mostly on which catecholamine is released as well as the amount of catecholamine that is released. The acute release of norepinephrine and epinephrine from PHEO increases heart rate, systemic vascular resistance, myocardial contractility, and reduces venous compliance. The excessive adrenergic stimulation by catecholamine results in severe vasoconstriction and coronary vasospasm, myocardial ischemia, and subsequently damage, and necrosis. Chronically elevated catecholamine levels lead to significant desensitization of cardiac β-adrenoceptors. The increased levels of the enzyme β-adrenoceptors kinase (βARK) in the heart seems to mediate these biochemical and physiological changes that are consistently correlated with attenuated responsiveness to catecholamine stimulation. Through these mechanisms different types of cardiomyopathy (CMP) can be formed. This review discusses extensively the 3 types of cardiomyopathies that can be present in a PHEO patient. It also provides the clinical presentation and diagnostic and therapeutic algorithm in managing patients with CICMPP.

ST-Segment Elevation Myocardial Infarction Related to Potential Spontaneous Coronary Thrombosis in Pheochromocytoma Crisis

“…Second, the inflammatory cytokines and procoagulants secreted by tumor cells, such as the plasminogen activator inhibitor-1 which is stored in catecholamine storage vesicles and co-released with catecholamines, may play a crucial role in inducing hypercoagulability (9,10). Third, the coronary slow flow, possibly caused by microvascular dysfunction following vasospasm due to aberrant catecholamines secretion (11,12), may also participate in this pathologic process.…”

A case of catecholamine-induced cardiomyopathy treated with extracorporeal membrane oxygenation

“…CICMP should be suspected in all cases of heart failure or cardiomyopathy with unclear aetiology, an acute coronary syndrome with non-obstructive coronaries and multiple organ failure without a clear aetiology. A preoperative echocardiogram is recommended in all cases of PHEO and PGL 4. Biochemical diagnosis of PHEO and PGL is made by finding high levels of plasma free metanephrines or total urinary metanephrines.…”

“…Milrinone which acts through the calcium channel can be used. Refractory hypotension may necessitate the need for intraaortic balloon pump or VAECMO 4…”

“…Prompt recognition and management of CICMP are essential to reduce mortality and morbidity. Due to the relative rarity of this condition, there is a lack of evidence-based guidelines for the management of CICMP 4…”

Pheochromocytomas: Fabulous, Fascinating, and First (in everything)!

“…Complications of pheochromocytomas are frequent and one of the least understood and more clinically consequential is cardiomyopathy. Santos et al guides us through its detection and therapy to minimize its potentially ruinous effects [12].…”