成人期にtotal cavopulomonary connectionによるFontan手術を施行した症例の中期成績と効果の検討

Taku Ishii; 石井卓; Tadahiro Yoshikawa; 嘉川忠博; Satoshi Yazaki; 矢崎諭; Takumi Kobayashi; 小林匠; Kanako Kishiki; 吉敷香菜子; Akio Inage; 稲毛章郎; Yuji Hamamichi; 浜道裕二; Tomomi Ueda; 上田知実; Naoki Wada; 和田直樹; Makoto Ando; 安藤誠; Yukihiro Takahashi; 高橋幸宏

doi:10.9794/jspccs.34.143

成人期にtotal cavopulomonary connectionによるFontan手術を施行した症例の中期成績と効果の検討Midterm Prognosis and Effectiveness of Fontan Procedure with Total Cavopulmonary Connection Performed in Adults

石井卓¹,，嘉川忠博¹，矢崎諭¹，小林匠¹，吉敷香菜子¹，稲毛章郎¹，浜道裕二¹，上田知実¹，和田直樹²，安藤誠²，高橋幸宏²Taku Ishii¹,, Tadahiro Yoshikawa¹, Satoshi Yazaki¹, Takumi Kobayashi¹, Kanako Kishiki¹, Akio Inage¹, Yuji Hamamichi¹, Tomomi Ueda¹, Naoki Wada², Makoto Ando², Yukihiro Takahashi²

¹ 公益財団法人　日本心臓血圧研究振興会附属榊原記念病院　小児科Department of Pediatric Cardiology, Sakakibara Heart Institute ◇ Tokyo, Japan

² 公益財団法人　日本心臓血圧研究振興会附属榊原記念病院　心臓血管外科Department of Cardiovascular Surgery, Sakakibara Heart Institute ◇ Tokyo, Japan

現所属先：東京医科歯科大学医学部附属病院　小児科〔Current affiliation: Department of Pediatrics, Tokyo Medical and Dental University, Medical Hospital, Tokyo, Japan〕

受付日：2018年3月17日Received: March 17, 2018

受理日：2018年5月18日Accepted: May 18, 2018

発行日：2018年9月1日Published: September 1, 2018

背景：本研究の目的は，成人期TCPC（total cavopulmonary connection）の中期成績を小児期TCPCと比較することで明らかにするとともに，合併症のリスク因子および手術による血行動態への影響を検討することである．

方法：当院でTCPCを行った症例のうち，手術時18歳以上の25症例を対象とし，診療録を用いて後方視的に検討を行った．また，比較対象として，当院でTCPCを行った小児例（5歳未満，75例）を用いた．

結果：周術期合併症を13例に認めたが，周術期死亡は1例のみだった．術後の累積生存率は5年時96.0％で，小児期TCPCと有意差を認めなかった．一方，退院後の心血管イベントは小児期TCPCと比べて有意に高かった．観察期間内における手術後の総死亡は3例で，術前の心室拡張末期圧と平均肺動脈圧の上昇が共通していた．退院後の心血管イベントは心房内臓錯位症候群症例で頻度が高かった．また，周術期，退院後ともに成人期TCPC症例では上室頻拍の頻度が小児例に比べて高かった．手術前後では，酸素飽和度の有意な上昇と心胸郭比の有意な改善が見られた．一方，手術後の心係数は平均1.9 L/min/m²と低値であった．

結論：成人期TCPCの生存率は良好で，チアノーゼの改善と心拡大の改善が得られるため，成人期であってもTCPCによるFontan手術を行う意義はある．ただ，上室頻拍をはじめとした術後の合併症頻度が高いことや心拍出量低下などは念頭におく必要がある．

Background: This study aimed to ascertain midterm prognosis by comparing adult and child patients while accounting for the risk factors of complications and the effectiveness of the total cavopulmonary connection (TCPC) procedure.

Method: We analyzed data for 25 patients aged 18 or older who underwent TCPC procedure. The data for 75 patients aged 5 years or lower were analyzed for comparing the results.

Results: We observed perioperative complications in 13 patients including one perioperative death. The overall 5-year survival ratio after performing the TCPC procedure in adults was 96.0％, which was not different from that in child patients. On the contrary, cardiac events after discharge occurred more frequently in adult patients. Three patients died after performing the Fontan procedure in the observation period, all of whom had high end-diastolic pressure of the main ventricle and high pulmonary artery pressure. Cardiac events after discharge occurred more frequently among the patients with heterotaxy. Supraventricular tachyarrhythmia occurred more frequently after conducting the TCPC procedure in adult patients than in child patients both in the hospital and after discharge. Percutaneous oxygen saturation and cardiothoracic ratio improved significantly after the Fontan procedure; however, the mean cardiac index remained low at 1.9 L/min/m²．

Conclusion: The overall survival after performing the Fontan procedure with TCPC was satisfactory in adults. TCPC improved cyanosis and reduced volume overload in adult patients. However, certain patients may develop supraventricular tachyarrhythmia, and some may have high central venous pressure and low cardiac output after the surgery.

Key words: adult Fontan; total cavopulmonary connection; effectiveness; midterm prognosis; risk factors

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

原著Original

成人期にtotal cavopulomonary connectionによるFontan手術を施行した症例の中期成績と効果の検討Midterm Prognosis and Effectiveness of Fontan Procedure with Total Cavopulmonary Connection Performed in Adults