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 32(2): 141-153 (2016)
doi:10.9794/jspccs.32.141

ReviewReview

フォンタン術後患者のQOL向上をめざして:経時的な病態観察から学ぶSeeking a Better Quality of Life for Patients after the Fontan Operation: Lessons Learned from Serial Assessment of Fontan Pathophysiology

国立循環器病研究センター 小児循環器科 成人先天性心疾患科Departments of Pediatric Cardiology and Adult Congenital Heart Disease, 
National Cerebral and Cardiovascular Center ◇ Osaka, Japan

受付日:2015年11月23日Received: November 23, 2015
受理日:2016年1月28日Accepted: January 28, 2016
発行日:2016年3月1日Published: March 1, 2016
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フォンタン手術は単心室循環患者のQOLや生命予後を著しく改善したが,その術後病態は極めて多様で,他の先天性心疾患患者術後に比べ予期しない入院や死亡の頻度は依然として高い.最近の医療の進歩によりフォンタン手術の成績が格段に改善したが,術後遠隔期の高い臨床事故予防や軽減に向けた治療管理戦略がほとんど確立できていない.その高頻度の事故の背景には特異な循環の病態把握がいまだ不十分であることに加え,この特異な循環不全病態の長期予後(一生)の全容が不明である現状がある.我々の施設ではフォンタン術後患者の長期予後改善を目指し,定期的な血行動態評価,心肺運動負荷試験,さらには糖代謝や肝腎機能評価といった総合的な評価を継続している.これらの試みで,心血管機能に加え,多臓器機能を含めたフォンタン病態が徐々に明らかとなりつつある.しかし,一方で様々な新たな病態が明らかになっている現状がある.

The Fontan operation has certainly changed the quality of life in patients with congenital heart disease with functionally single ventricle, and the perioperative survival has dramatically improved owing to recent medical advances and modifications of the surgical technique. The postoperative course of these patients varies widely with relatively high morbidity and mortality compared with the course of patients in whom congenital heart disease has been treated with other types of definitive repairs. However, long-term management strategies of these patients have not yet been established because of a lack of information on the entire postoperative course of the unique hemodynamics and insufficient understanding of the pathophysiology of this type of heart failure. Our institute has developed a unique approach to overcome these clinical problems and has tried to provide these patients with better medical care using precise serial comprehensive assessments of the pathophysiology, including hemodynamics, cardiopulmonary exercise testing, hepatorenal conditions, and even metabolic abnormalities. Thanks to these daily practices, we have clarified some of the complicated heart failure pathophysiology to some extent, and some patients have benefited from these results. However, additional complications have emerged as these patients aged and raise many questions.

Key words: Fontan; hemodynamics; prognosis; heart failure

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This page was last modified on 2016-04-12T11:39:54.908+09:00


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