積極的にPrimary Norwood手術を選択した治療戦略でのFontan手術到達への手術成績の検討

Hidenori Hayashi; 林秀憲; Koichi Sughimoto; 杉本晃一; Yuta Tsuchida; 𡈽田勇太; Tsuyoshi Yoshii; 吉井剛; Shin Kondo; 近藤真; Sumito Kimura; 木村純人; Eri Mineo; 峰尾恵梨; Atsushi Kitagawa; 北川篤史; Hisashi Ando; 安藤寿; Masahiro Ishii; 石井正浩; Kagami Miyaji; 宮地鑑

doi:10.9794/jspccs.33.197

積極的にPrimary Norwood手術を選択した治療戦略でのFontan手術到達への手術成績の検討Primary Norwood Operation Strategy for Hypoplastic Left Heart Syndrome and its mid-term outcome up to the Fontan Operation

林秀憲^1,2，杉本晃一¹，𡈽田勇太¹，吉井剛¹，近藤真¹，木村純人³，峰尾恵梨³，北川篤史³，安藤寿³，石井正浩³，宮地鑑¹Hidenori Hayashi^1,2, Koichi Sughimoto¹, Yuta Tsuchida¹, Tsuyoshi Yoshii¹, Shin Kondo¹, Sumito Kimura³, Eri Mineo³, Atsushi Kitagawa³, Hisashi Ando³, Masahiro Ishii³, Kagami Miyaji¹

¹ 北里大学心臓血管外科Department of Cardiovascular Surgery, Kitasato University School of Medicine ◇ Tokyo, Japan

² 和歌山県立医科大学心臓血管外科Department of Cardiovascular Surgery, Wakayama Medical University ◇ Wakayama, Japan

³ 北里大学小児科Department of Pediatrics, Kitasato University School of Medicine ◇ Tokyo, Japan

受付日：2017年1月13日Received: January 13, 2017

受理日：2017年4月10日Accepted: April 10, 2017

発行日：2017年5月1日Published: May 1, 2017

背景：左心低形成症候群（HLHS）では近年初回に両側肺動脈絞扼術が姑息術として行われるようになったが，遠隔期の問題が指摘されている．初回手術として積極的にNorwood手術を行い，Fontan手術に至る中期遠隔成績と危険因子を解析した．

方法：2004年10月から2014年5月までにHLHS等の症例に対しNorwood手術を受けた連続する16名に対し後方視的解析を行った．

結果：1例を除く15例に初回Norwood手術を行った．Norwood手術後1年生存率は69％，3年生存率は56％であり，遺伝子異常（p＝0.037），Heterotaxy（p＝0.026）が死亡の危険因子であった．BCPS後1.0±0.5年で，全例がFontan手術に到達した．

結論：初回Norwood手術は遺伝子異常やHeterotaxyを除き有効な治療戦略であった．同病態への治療方針は再考の余地がある．

Background: Bilateral pulmonary artery banding for patients with Hypoplastic Left Heart Syndrome (HLHS) prevails as the first palliation in Japan, however, concerns remain about the long-term outcomes. Primary Norwood operation, nevertheless, has advantages such as fewer inter-stage palliations. This study’s goal is to review the mid-term results of primary Norwood operations up to the Fontan operations and to analyze risk factors.

Methods: Sixteen consecutive patients with HLHS (male/female: 6/10) were included from October 2004 to May 2014. Of those, fifteen patients underwent the primary Norwood operations. Survival was assessed by the Kaplan–Meier method and compared between groups using the Mantel–Cox log-rank test. A Cox regression model was used for risk factor analysis.

Results: Estimated survival rate was 69％ at one year and 56％ at three years after the Norwood operations. Prior to Fontan operations, palliations were performed in 2.5±0.8 times. Hazard ratio for death of genetic disorder/malformation and heterotaxy was 14.03 (95％CI, 1.17–167.8, p＝0.037) and 18.66 (95％CI, 1.43–244.4, p＝0.026), respectively. Nine patients (9/16＝56％) achieved Fontan operations. All survivors after the Fontan operations except one patient who suffered from protein-losing enteropathy, are in NYHA class 1.

Conclusion: The strategy of primary Norwood operation for patients with HLHS was feasible up to the Fontan operation with fewer inter-stage palliations, except for genetic disorder and heterotaxy. More investigations are required to justify the operational strategy for genetic disorder and heterotaxy patients.

Key words: Norwood operation; Hypoplastic Left Heart Syndrome; Fontan operation; congenital heart disease

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

原著Original

積極的にPrimary Norwood手術を選択した治療戦略でのFontan手術到達への手術成績の検討Primary Norwood Operation Strategy for Hypoplastic Left Heart Syndrome and its mid-term outcome up to the Fontan Operation