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Review
. 2023 Jul 13:14:1204851.
doi: 10.3389/fendo.2023.1204851. eCollection 2023.

Pheochromocytoma/paraganglioma-associated cardiomyopathy

Alicja Szatko  1   2 , Piotr Glinicki  1   2 , Małgorzata Gietka-Czernel  1
Affiliations
Review

Pheochromocytoma/paraganglioma-associated cardiomyopathy

Alicja Szatko et al. Front Endocrinol (Lausanne). .

Abstract

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

Keywords: dilated cardiomyopathy; hypertrophic cardiomyopathy; paraganglioma; pheochromocytoma; takotsubo cardiomyopathy.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Binding affinities of epinephrine and norepinephrine to adrenoceptors and mechanisms leading to the development of subtypes of catecholamine-induced cardiomyopathy in pheochromocytoma-paraganglioma (CICMPP). Gi, inhibiting G protein; GRK, G protein–coupled receptor kinase.
Figure 2
Figure 2
Pharmacological management of acute cardiovascular complications in patients with pheochromocytoma-paraganglioma. The order of presented drugs does not correspond to first-, second-, and third-line therapy and the choice of administered drugs should be individualized. 1With alpha-adrenoceptor blockade, otherwise, it can precipitate a hypertensive crisis. 2Antiarrhythmic agent not recommended in systolic dysfunction. 3Used to restore sinus rhythm. 4In case of hypotension and suspected beta2-adrenoceptor overstimulation. NDHPCCB, Nondihydropyridine Calcium Channel Blockers; ST, Sinus Tachycardia; AF/AFL, Atrial Fibrillation/Atrial Flutter; VT, Ventricular Tachycardia; MCS, Mechanical Circulatory Support.
See this image and copyright information in PMC

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