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黑丁格综合征:类癌综合征罕见的心脏表现。

Hedinger Syndrome: A Rare Cardiac Manifestation of Carcinoid Syndrome.

作者信息

Ghukasyan Hayk

机构信息

Internal Medicine, University of Hawaii, Honolulu, USA.

出版信息

Cureus. 2022 Jul 3;14(7):e26528. doi: 10.7759/cureus.26528. eCollection 2022 Jul.

Abstract

Carcinoid syndrome can cause desmoplastic reactions to nearby tissues. When it involves the heart, it causes carcinoid heart disease (CHD) or Hedinger syndrome and usually involves the right-sided heart valves, causing tricuspid insufficiency and pulmonary stenosis (TIPS) and eventually leading to right-sided heart failure. The management of patients with CHD is complex, as both the systemic malignant disease and the heart involvement have to be addressed. Its prompt diagnosis and early treatment is paramount as CHD is associated with increased morbidity and mortality. A 61-year-old Caucasian male with a recently diagnosed metastatic neuroendocrine tumor presented to the heart failure clinic with decompensated heart failure, anasarca, flushing and diarrhea. This case highlights the common clinical features of carcinoid syndrome, its cardiac manifestations and the pathophysiology underlying the manifestations and treatment decisions that involve addressing both systemic and cardiac manifestations.

摘要

类癌综合征可导致对附近组织的促纤维增生反应。当累及心脏时,会引发类癌性心脏病(CHD)或黑丁格综合征,通常累及右侧心脏瓣膜,导致三尖瓣关闭不全和肺动脉狭窄(TIPS),最终导致右侧心力衰竭。CHD患者的管理很复杂,因为既要处理全身性恶性疾病,又要处理心脏受累情况。由于CHD与发病率和死亡率增加相关,其及时诊断和早期治疗至关重要。一名61岁的白种男性,最近被诊断为转移性神经内分泌肿瘤,因失代偿性心力衰竭、全身性水肿、潮红和腹泻就诊于心力衰竭门诊。该病例突出了类癌综合征的常见临床特征、其心脏表现以及这些表现背后的病理生理学,以及涉及处理全身和心脏表现的治疗决策。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78e6/9250807/8a38f1c7e69f/cureus-0014-00000026528-i01.jpg

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