日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 35(1): 46-51 (2019)
doi:10.9794/jspccs.35.46

症例報告Case Report

褐色細胞腫を合併したEisenmenger症候群の剖検例An Autopsy Case of Eisenmenger Syndrome with Pheochromocytoma

1弘前大学大学院医学研究科小児科学講座Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine ◇ Aomori, Japan

2弘前大学大学院保健学研究科Hirosaki University School of Health Sciences and Graduate School of Health Sciences ◇ Aomori, Japan

3弘前大学大学院医学研究科病理診断学講座Department of Anatomic Pathology, Hirosaki University School of Medicine and Graduate School of Medicine ◇ Aomori, Japan

受付日:2018年7月4日Received: July 4, 2018
受理日:2018年11月14日Accepted: November 14, 2018
発行日:2019年3月1日Published: March 1, 2019
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Eisenmenger症候群(ES)に褐色細胞腫(pheochromocytoma: PCC)を合併した稀な症例を経験した.症例は女性で,新生児期に両大血管右室起始(double outlet right ventricle: DORV)と診断したが,乳児期よりESの病態を呈した.30歳時にPCCの合併を診断した.外科的切除は周術期のリスクから適応外とされた.32歳時に心不全,腎不全のため死亡した.病理解剖でDORV,高度の肺血管閉塞性病変,左副腎のPCC,膀胱などの多発性のパラガングリオーマ(paraganglioma: PGL), チアノーゼ性腎症を認めた.近年,PCC/PGL発症のリスク因子として低酸素が報告されている.本症例でもDORVやESがPCC/PGLの誘因になった可能性がある.PCC/PGLの症状は心疾患の症状とオーバーラップしており,先天性心疾患患者のPCC/PGLの診断は遅れがちになりやすい.さらに本症例ではESによるPCCの治療制限,PCCによる高血圧と頻脈の心血行動態への悪影響など治療と管理にも難渋した.先天性心疾患の合併症としてPCC/PGLも念頭に置くべきである.

We report a rare case of a patient with pheochromocytoma (PCC) and Eisenmenger syndrome (ES). The patient was a female born to healthy parents. Although double outlet right ventricle (DORV) was diagnosed in the neonatal period, she displayed ES pathology from infancy. At the age of 30 years, PCC complications were diagnosed. An operation was contraindicated because of high perioperative risk. She died at the age of 32 years owing to advanced heart failure and renal failure. Her pathologic anatomy revealed DORV, severe pulmonary vascular obstruction, PCC in the left adrenal gland, multiple paraganglioma (PGL) in the bladder, and cyanotic nephropathy. In recent years, hypoxia has been reported as a risk factor for PCC/PGL. In our case, PCC/PGL was associated with DORV and ES. The diagnosis of PCC/PGL in congenital heart disease patients tends to be delayed as the symptoms of PCC/PGL overlap with those of heart disease. The harmful effects of hypertension and/or tachycardia on cardiac hemodynamics due to PCC were difficult to manage as she was contraindicated for surgery. PCC/PGL should be considered as a complication of congenital heart disease.

Key words: Eisenmenger syndrome; pheochromocytoma; double outlet right ventricle; hypoxia; SDH

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This page was last modified on 2019-03-12T16:00:10.000+09:00


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