Abstract

BackgroundCatecholamine excess arising from pheochromocytomas and paragangliomas (PPGLs) can cause a wide spectrum of cardiac manifestations, including acute cardiac complications (ACCs) and subclinical myocardial injuries (SMIs). In this study, we aimed to conduct a comprehensive analysis of ACCs and SMIs in a large cohort of patients with PPGLs.MethodsWe retrospectively analyzed the clinical data of consecutive patients with PPGLs admitted between January 2013 and July 2020 (n = 189). The prevalence of ACCs and SMIs and characteristics of patients identified with ACCs and SMIs were investigated. Moreover, comparisons were performed between patients with and without ACCs.ResultsFourteen patients (7.4%) fulfilled the criteria for ACCs, including nine (4.8%) who presented with Takotsubo-like cardiomyopathy, four (2.1%) with heart failure with preserved ejection fraction, and finally one (0.5%) with catecholamine-induced cardiomyopathy. Compared to those without ACCs (n = 175), patients with ACCs had a higher prevalence of epinephrine-producing PPGLs (81.8% vs 33.9%, P = 0.006) and were more likely to show invasive behavior (61.5% vs 27.3%, P = 0.022) or hemorrhage/necrosis (53.9% vs 17.4%, P = 0.005) on histology. The apical sparing pattern (5/7, 71.4%) was the dominant impairment pattern of longitudinal strain (LS) for patients displaying Takotsubo-like cardiomyopathy. In patients without cardiac symptoms, a fairly high proportion (21/77, 27.3%) of patients who underwent screening for troponin and/or natriuretic peptide and/or echocardiography had SMIs.ConclusionsOne in every fourteen PPGL patients presented with ACCs, and in the patients with Takotsubo-like cardiomyopathy, the apical sparing pattern was the primary impairment pattern of LS. Additionally, nearly one-third of patients without symptoms had SMIs. The diagnosis of PPGLs should be considered in patients with acute reversible cardiomyopathy, especially in those exhibiting an apical sparing pattern of LS.

Highlights

  • Catecholamine excess arising from pheochromocytomas and paragangliomas (PPGLs) can cause a wide spectrum of cardiac manifestations, including acute cardiac complications (ACCs) and subclinical myocardial injuries (SMIs)

  • PPGLs were incidentally found in 59.3% (112/189) of patients, who either had no symptoms or, in symptomatic cases, the symptoms were not considered to be related to the space-occupying effect of tumors or CA-related effects

  • Seventy (37.0%) patients were screened for tumors with CA secretion, as the plasma CA/MN/NMN test was unavailable until January 2018 in our center; the urine vanillylmandelic acid test was performed in 103 (54.5%) cases, resulting in 135 (71.4%) patients undergoing at least one of the aforementioned endocrine tests

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Summary

Introduction

Catecholamine excess arising from pheochromocytomas and paragangliomas (PPGLs) can cause a wide spectrum of cardiac manifestations, including acute cardiac complications (ACCs) and subclinical myocardial injuries (SMIs). A few retrospective series of PPGLs have been reported from cardiac perspectives, revealing that the prevalence of ACCs varies from 11% to 19.3% [4,5,6,7,8]. We retrospectively reviewed patients diagnosed with PPGLs in our center and screened carefully for patients with ACCs. The prevalence and features of PPGLs and outcomes of PPGL patients with ACCs were assessed; comparisons between patients with and without ACCs were conducted to identify the patient features associated with ACCs. subclinical myocardial injuries (SMIs) were investigated to reveal CA-induced cardiac damage that did not cause clinical symptoms

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