Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Roma, Italy.
Clinical Internal, Anesthesiological and Cardiovascular Sciences, Sapienza University of Rome, 00185 Roma, Italy.
Int J Mol Sci. 2020 Jul 17;21(14):5047. doi: 10.3390/ijms21145047.
Cardiomyopathies are myocardial disorders in which heart muscle is structurally and/or functionally abnormal. Previously, structural cardiomyocyte disorders due to adrenal diseases, such as hyperaldosteronism, hypercortisolism, and hypercatecholaminism, were misunderstood, and endomyocardial biopsy (EMB) was not performed because was considered dangerous and too invasive. Recent data confirm that, if performed in experienced centers, EMB is a safe technique and gives precious information about physiopathological processes implied in clinical abnormalities in patients with different systemic disturbances. In this review, we illustrate the most important features in patients affected by primary aldosteronism (PA), Cushing's syndrome (CS), and pheochromocytoma (PHEO). Then, we critically describe microscopic and ultrastructural aspects that have emerged from the newest EMB studies. In PA, the autonomous hypersecretion of aldosterone induces the alteration of ion and water homeostasis, intracellular vacuolization, and swelling; interstitial oedema could be a peculiar feature of myocardial toxicity. In CS, cardiomyocyte hypertrophy and myofibrillolysis could be related to higher expression of atrogin-1. Finally, in PHEO, the hypercontraction of myofilaments with the formation of contraction bands and occasional cellular necrosis has been observed. We expect to clear the role of EMB in patients with cardiomyopathies and adrenal disease, and we believe EMB is a valid tool to implement new management and therapies.
心肌病是心肌结构和/或功能异常的心肌疾病。以前,由于肾上腺疾病引起的结构性心肌细胞疾病,如醛固酮增多症、皮质醇增多症和儿茶酚胺增多症,被误解了,并且由于被认为是危险和过于侵入性的,因此没有进行心内膜心肌活检(EMB)。最近的数据证实,如果在有经验的中心进行,EMB 是一种安全的技术,可以提供有关不同全身性疾病患者临床异常中涉及的生理病理过程的宝贵信息。在这篇综述中,我们说明了原发性醛固酮增多症(PA)、库欣综合征(CS)和嗜铬细胞瘤(PHEO)患者最重要的特征。然后,我们批判性地描述了最新的 EMB 研究中出现的微观和超微结构方面。在 PA 中,醛固酮的自主过度分泌导致离子和水稳态的改变、细胞内空泡化和肿胀;间质水肿可能是心肌毒性的一个特殊特征。在 CS 中,心肌细胞肥大和肌原纤维溶解可能与 atrogin-1 的更高表达有关。最后,在 PHEO 中,观察到肌原纤维的强烈收缩伴有收缩带的形成和偶尔的细胞坏死。我们期望明确 EMB 在心肌病和肾上腺疾病患者中的作用,并且我们相信 EMB 是实施新的管理和治疗方法的有效工具。