Kjernsmo A
Medicinska kliniken i Torsby.
Tidsskr Nor Laegeforen. 1991 Sep 10;111(21):2640-3.
Pheochromocytoma may mimic a variety of clinical syndromes which present a diagnostic challenge when blood pressure is normal. Occasionally the predominant manifestation of such a tumour is congestive heart failure. The article describes a 19 year-old adolescent with an advanced dilated cardiomyopathy (ejection fraction 0.20) which was finally proven to be induced by a noradrenaline-secreting tumour (urinary noradrenaline: 88,130 nmol/24 hours, ref. interval: less than 350) localized in his right adrenal medulla. In spite of normalization of urinary noradrenaline levels postoperatively the patient died within two weeks due to intractable biventricular failure. Even though pheochromocytoma-induced cardiomyopathy is regarded as potentially reversible after tumour resection, careful consideration is imperative to optimize the patients preoperative status. The article reviews the mechanisms involved in the development of the myocardial changes induced by pheochromocytoma, as well as therapeutic principles relating to the pre- and intraoperative stages of the disease.
嗜铬细胞瘤可能会模拟多种临床综合征，当血压正常时，这些综合征会带来诊断挑战。偶尔，此类肿瘤的主要表现是充血性心力衰竭。本文描述了一名19岁的青少年，患有晚期扩张型心肌病（射血分数0.20），最终被证实是由位于其右肾上腺髓质的分泌去甲肾上腺素的肿瘤引起的（尿去甲肾上腺素：88,130 nmol/24小时，参考区间：小于350）。尽管术后尿去甲肾上腺素水平恢复正常，但患者在两周内死于难治性双心室衰竭。尽管嗜铬细胞瘤诱发的心肌病在肿瘤切除后被认为可能是可逆的，但必须仔细考虑以优化患者的术前状态。本文回顾了嗜铬细胞瘤诱发心肌改变的发病机制，以及与该疾病术前和术中阶段相关的治疗原则。
