Abstract
Pheochromocytomas and paragangliomas (PPGLs) are rare neuro-endocrine tumors. The catecholamine surge causes paroxysmal or chronic secondary hypertension. PPGLs may present as hypertensive- or PPGL-crisis with severe life-threatening cardiac and cerebrovascular complications. PPGLs-induced cardiac manifestations have been reported with diagnoses as PPGLs-induced electrocardiogram (ECG) changes “mimicking acute myocardial infarction”, arrhythmias, myocarditis, acute coronary syndrome, dilated cardiomyopathy, and lately as takotsubo syndrome. Critical analysis of these reports reveals that most of these cardiac manifestations have certain features in common. They have a dramatic clinical presentation and are reversible if the disease is treated with appropriate medical therapy and surgical resection of the PPGL tumor. They may have the same repolarization ECG changes irrespective of the clinical cardiac diagnosis, usually associated with mild to moderate elevations of myocardial biomarkers as troponins and normal coronary arteries. The histopathological findings are usually focal or multifocal in the form hypercontracted sarcomeres and contraction band necrosis (myofibrillar degeneration) with subsequent secondary mononuclear cell infiltration. Evidences argue the PPGL caused surge of catecholamines triggers hyperactivation of the sympathetic nervous system with cardiac sympathetic nerve terminal disruption with norepinephrine spillover causing the cardiac complications. A comprehensive review of various reported cardiovascular manifestations and complications of PPGLs are presented.
Highlights
Pheochromocytomas and paragangliomas (PPGLs) are rare neuro-endocrine tumors where the first arise from chromaffin tissues in the adrenal medulla and the second develop from chromaffin tissues in the extra-adrenal sympathetic and parasympathetic nervous system [1,2]
In the early reports on PPGLs, cardiac manifestations were reported with abnormal electrocardiogram (ECG) [31] or catecholamine-induced ECG changes mimicking ischemia induced by PPGLs [32]
The diagnosis of PPGLs-induced Dilated cardiomyopathy (DCM) were in most cases based on a clinical presentation with congestive heart failure, pulmonary edema or cardiogenic shock associated with diffuse left ventricular dysfunction documented by cardiac image studies [64,65]
Summary
Pheochromocytomas and paragangliomas (PPGLs) are rare neuro-endocrine tumors where the first arise from chromaffin tissues in the adrenal medulla and the second develop from chromaffin tissues in the extra-adrenal sympathetic and parasympathetic nervous system [1,2]. There are several familial syndromes (inherited tumor syndromes) with disease-causing mutations that are associated with PPGL (e.g., RET, SDHx, VHL, NF1, MAX and TMEM127) [2]. 123I-MIBG functional imaging is not useful in patients with SDHx mutations These patient groups require lifelong surveillance for both PPGLs and other related tumors. Severe cardiovascular complications in PPGLs are usually associated with hypertensive crisis. Zhang et al reported on the association of pheochromocytoma and various types of cardiomyopathies [16] They studied 163 case from 150 published articles between 1991 and 2016 (dilated cardiomyopathy n = 63, classical-apical takosubo syndrome (TS) n = 38, inverted TS n = 30, hypertrophic obstructive cardiomyopathy n = 10, myocarditis n = 8, and unspecified cardiomyopathy n = 14). The various cardiovascular manifestations and complication induced by PPGLs are reviewed and a short description of the pathogenetic mechanism and management are presented
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