Department of Medicine, University of Birmingham Medical School, B15 2TH Birmingham, UK.
Department of Endocrinology and Metabolism, Lancashire Teaching Hospitals NHS Trust, PR2 9HT Preston, UK.
Rev Cardiovasc Med. 2021 Dec 22;22(4):1215-1228. doi: 10.31083/j.rcm2204130.
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.
尽管许多内分泌疾病都可能与获得性心肌病和心力衰竭相关,但除了甲状腺功能减退症、甲状腺功能亢进症、嗜铬细胞瘤/副神经节瘤(PPGL)和原发性醛固酮增多症外,其他情况较为罕见。PPGL 是一种罕见的儿茶酚胺分泌性神经内分泌肿瘤,起源于肾上腺 80-85%的嗜铬细胞瘤或其余的自主神经节副神经节细胞中的肾上腺外嗜铬细胞。在普通人群中,PPGL 的年发病率为每百万人口 3-8 例。在患有 PPGL 的患者中,儿茶酚胺诱导的心肌病(CICMP)的患病率为 8-11%。高血压,无论是持续性还是间歇性,存在于绝大多数(95%)PPGL 患者中。然而,在患有 CICMP 的患者中,高血压仅存在于 65%的病例中,经典的三联征即阵发性头痛、出汗和心悸仅存在于 4%的病例中。根据内源性儿茶酚胺过多引起的心脏重构,PPGL 患者可能表现出三种 CICMP 中的一种,包括扩张型心肌病(DCM)、肥厚型心肌病(HCM)或 Takotsubo 心肌病(TCM)。无论亚型如何,所有 CICMP 都有许多共同特征——戏剧性的临床表现、可逆性心肌病、相似的复极心电图变化、轻度至中度心脏生物标志物升高以及冠状动脉造影正常的冠状动脉。即使没有明确的儿茶酚胺过多特征,患有非缺血性、非瓣膜性心肌病的患者也应怀疑存在 CICMP。在所有急性冠状动脉综合征(ACS)患者中,若存在冠状动脉造影无明显病变但血压变化明显的情况下,应怀疑患有与 PPGL 相关的 TCM。本文将对各种 CICMP 进行综述,包括其病理生理学、临床特征和治疗选择。