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一例由儿茶酚胺分泌肿瘤诱发肥厚型心肌病的罕见病例报告。

A rare case report of hypertrophic cardiomyopathy induced by catecholamine-producing tumor.

作者信息

Olmati Federica, Petramala Luigi, Bisogni Valeria, Concistré Antonio, Saracino Vincenza, Oliviero Gaia, Bonvicini Maria, Mezzadri Martina, Ciardi Antonio, Iannucci Gino, De Toma Giorgio, Frustaci Andrea, Letizia Claudio

机构信息

Secondary Hypertension Unit, Department of Internal Medicine and Medical Specialties, University of Rome.

Department of Radiological, Oncological and Anatomy-Pathological Sciences, University of Rome.

出版信息

Medicine (Baltimore). 2018 Dec;97(50):e13369. doi: 10.1097/MD.0000000000013369.

Abstract

RATIONALE

Catecholamine-producing tumors are rare, occurring in less than 0.2% of patients with hypertension, but can have relevant cardiovascular morbidity and mortality.

PATIENT CONCERNS

A 37-year-old woman presented with a history of dyspnea, chest pain, palpitations, and paroxysmal hypertension. Electrocardiogram, echocardiogram, and cardiac magnetic resonance showed severe LVH with a prevalent involvement of the anterior portion of interventricular septum. Endomyocardial biopsy found severe hypertrophy with disarray of cardiomyocytes and ultrastructural evidence of contraction and necrosis of myocytes. Hormone investigations revealed high values of 24-hours urinary metanephrines. Abdominal computed tomography (CT) showed an enlarged left adrenal gland with a strong uptake of I-metaiodobenzylguanidine at scintigraphy scan.

INTERVENTIONS

Thus, the adrenal tumor was surgically removed.

OUTCOMES

At follow-up examination, the patient's metanephrines levels were normalized and the transthoracic echocardiogram showed a reduction of LVH.

DIAGNOSIS AND LESSONS

We report a rare case of catecholamine-induced cardiomyopathy due to an adrenal adenoma mixed with nodules enriched in epinephrine-types secreting granules.

摘要

理论依据

分泌儿茶酚胺的肿瘤很罕见,在高血压患者中发生率低于0.2%,但可导致相关的心血管发病率和死亡率。

患者情况

一名37岁女性,有呼吸困难、胸痛、心悸和阵发性高血压病史。心电图、超声心动图和心脏磁共振显示严重左心室肥厚,以室间隔前部受累为主。心内膜活检发现严重肥厚,心肌细胞排列紊乱,并有心肌细胞收缩和坏死的超微结构证据。激素检查显示24小时尿间甲肾上腺素值升高。腹部计算机断层扫描(CT)显示左肾上腺增大,在闪烁扫描中对间碘苄胍摄取强烈。

干预措施

因此,手术切除了肾上腺肿瘤。

结果

在随访检查中,患者的间甲肾上腺素水平恢复正常,经胸超声心动图显示左心室肥厚减轻。

诊断与经验教训

我们报告了一例罕见的由肾上腺腺瘤引起的儿茶酚胺性心肌病,该腺瘤混合有富含肾上腺素型分泌颗粒的结节。

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