Pheochromocytoma Presenting as Hypertrophic Obstructive Cardiomyopathy

Wani, Adil; Adil, Amina; Gardezi, Syed A.A.; Jain, Renuka; Galazka, Patrycja; Waples, Mark J; Tajik, A. Jamil

doi:10.1001/jamacardio.2021.0944

Cited by 4 publications

(4 citation statements)

References 6 publications

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“…In advanced stages, patients may experience orthopnea and/or fluid retention with peripheral/pulmonary edema ( 63 ). The typical symptoms of patients with hypertrophic CICMPP may also be complemented with PPGL-suggestive symptoms, namely profuse sweating, and palpitations ( 64 , 65 ). ECG alterations often meet the criteria of left ventricle hypertrophy ( 64 ).…”

Section: Predisposing Factors and Clinical Presentationmentioning

confidence: 99%

“…The typical symptoms of patients with hypertrophic CICMPP may also be complemented with PPGL-suggestive symptoms, namely profuse sweating, and palpitations ( 64 , 65 ). ECG alterations often meet the criteria of left ventricle hypertrophy ( 64 ). Echocardiography shows systolic anterior motion of the anterior mitral valve leaflet, increased left ventricle outflow tract gradient with persisted ejection fraction (EF), and septal and posterior wall hypertrophy ( 64 , 66 , 67 ).…”

Section: Predisposing Factors and Clinical Presentationmentioning

confidence: 99%

“…ECG alterations often meet the criteria of left ventricle hypertrophy ( 64 ). Echocardiography shows systolic anterior motion of the anterior mitral valve leaflet, increased left ventricle outflow tract gradient with persisted ejection fraction (EF), and septal and posterior wall hypertrophy ( 64 , 66 , 67 ).…”

Section: Predisposing Factors and Clinical Presentationmentioning

confidence: 99%

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Pheochromocytoma/paraganglioma-associated cardiomyopathy

2023

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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.

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Section: Predisposing Factors and Clinical Presentationmentioning

confidence: 99%

Section: Predisposing Factors and Clinical Presentationmentioning

confidence: 99%

Section: Predisposing Factors and Clinical Presentationmentioning

confidence: 99%

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Pheochromocytoma/paraganglioma-associated cardiomyopathy

2023

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“…Pheochromocytoma is a rare form of secondary hypertension characterized by excessive production of catecholamines typically due to a benign tumor in the adrenal medulla 1 . Among the potential cardiac repercussions of pheochromocytoma and paraganglioma (PPGL), previous studies described echocardiographic abnormalities such as dynamic left ventricular outflow tract obstruction, 2 cardiomyopathy, 3–5 reversible dilatation of the left ventricle 6 and left ventricular functional changes 7 in patients with pheochromocytoma. More recent publications highlighted the effect on left ventricular hypertrophy and ventricular remodeling 8,9 .…”

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confidence: 99%

What is the effect of catecholamines on diastolic function in patients with pheochromocytoma and paraganglioma?

Macedo

Drager

Vieira

2022

J of Clinical Ultrasound

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Pheochromocytoma is a rare form of secondary hypertension characterized by excessive production of catecholamines typically due to a benign tumor in the adrenal medulla. Among the potential cardiac repercussions of pheochromocytoma and paraganglioma, previous studies described echocardiographic abnormalities such as dynamic left ventricular outflow tract obstruction (i), cardiomyopathy (ii, iii, iv), reversible dilatation of the left ventricle (v) and left ventricular functional changes (vi) in patients with pheochromocytoma.

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Catecholamine-induced cardiomyopathy: an endocrinologist’s perspective

Kumar¹,

Pappachan²,

Fernandez³

2021

Rev. Cardiovasc. Med.

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Although many endocrine diseases can be associated with acquired cardiomyopathy and heart failure, conditions except hypothyroidism, hyperthyroidism, phaeochromocytoma-paraganglioma (PPGL), and primary hyperaldosteronism are rare. PPGL is a rare catecholamine-secreting neuroendocrine tumour arising from the adrenal gland in 80–85% or extra-adrenal chromaffin cells of the autonomic neural ganglia in the remainder. The annual incidence of PPGL is 3–8 cases per million per year in the general population. Catecholamine-induced cardiomyopathy (CICMP) has got a prevalence of 8–11% among patients with PPGL. Hypertension, either sustained or episodic, is present in the vast majority (95%) of PPGL patients. However, among patients with CICMP, hypertension is present only in 65% of cases and the classical triad of paroxysmal headache, sweating, and palpitation is present only in 4%. Based on the cardiac remodelling in response to endogenous catecholamine excess, PPGL patients might present with one of the three CICMPs, including dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), or Takotsubo cardiomyopathy (TCM). Regardless of the subtypes, all CICMPs have many features in common — a dramatic clinical presentation, reversible cardiomyopathy, similar repolarisation electrocardiography changes, mild-moderate cardiac biomarker elevation, and normal coronary arteries on coronary angiography. CICMP should be suspected in patients with non-ischaemic, non-valvular forms of cardiomyopathy, even in those without definite features of catecholamine excess. PPGL associated TCM should be suspected in all acute coronary syndrome (ACS) patients exhibiting pronounced blood pressure variability with no culprit lesions on coronary angiography. This article will provide a review of the various CICMPs, their pathophysiology, clinical features, and the management options.

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Pheochromocytoma Presenting as Hypertrophic Obstructive Cardiomyopathy

Cited by 4 publications

References 6 publications

Pheochromocytoma/paraganglioma-associated cardiomyopathy

Pheochromocytoma/paraganglioma-associated cardiomyopathy

What is the effect of catecholamines on diastolic function in patients with pheochromocytoma and paraganglioma?

Catecholamine-induced cardiomyopathy: an endocrinologist’s perspective

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