内臓錯位症候群・単心室患者に合併した食道裂孔ヘルニアの臨床経過と手術適応についての検討

Sayo Suzuki; 鈴木彩代; Yuichi Ishikawa; 石川友一; Katsuo Tao; 田尾克生; Ayako Kuraoka; 倉岡彩子; Kenichiro Yamamura; 山村健一郎; Yoshihiko Kodama; 兒玉祥彦; Makoto Hayashida; 林田真; Toshihide Nakano; 中野俊秀; Koichi Sagawa; 佐川浩一

doi:10.9794/jspccs.41.42

内臓錯位症候群・単心室患者に合併した食道裂孔ヘルニアの臨床経過と手術適応についての検討Clinical Course and Operative Indication of Hiatal Hernia in Patient with Heterotaxy Syndrome and Single Ventricle

鈴木彩代¹，石川友一¹，田尾克生¹，倉岡彩子¹，山村健一郎¹，兒玉祥彦²，林田真³，中野俊秀⁴，佐川浩一¹Sayo Suzuki¹, Yuichi Ishikawa¹, Katsuo Tao¹, Ayako Kuraoka¹, Kenichiro Yamamura¹, Yoshihiko Kodama², Makoto Hayashida³, Toshihide Nakano⁴, Koichi Sagawa¹

¹ 福岡市立こども病院循環器科Department of Cardiology, Fukuoka Children’s Hospital ◇ Fukuoka, Japan

² 宮崎大学医学部附属病院小児科Department of Pediatrics, University of Miyazaki Hospital ◇ Miyazaki, Japan

³ 福岡市立こども病院小児外科Department of Pediatric Surgery, Fukuoka Children’s Hospital ◇ Fukuoka, Japan

⁴ 福岡市立こども病院心臓血管外科Department of Cardiovascular Surgery, Fukuoka Children’s Hospital ◇ Fukuoka, Japan

受付日：2023年12月19日Received: December 19, 2023

受理日：2024年12月29日Accepted: December 29, 2024

発行日：2025年2月28日Published: February 28, 2025

背景：内臓錯位症候群・単心室患者の食道裂孔ヘルニア（HH: hiatal hernia）は，単心室循環へ悪影響を及ぼし得るが，このコホートでのHH修復術適応は定まっていない．

方法：2008年1月から2018年12月に当院で出生した内臓錯位症候群・単心室患者109人の，HH合併，修復術の有無等臨床経過を検討した．

結果：13例（12％）がHHを合併し，6例にHH手術を施行した．4例は滑脱した胃による肺静脈圧排が疑われ，いずれも滑脱した胃が心房椎体間にあり肺静脈を後方から圧排している形態であった．手術施行例のうち3例が術後十二指腸狭窄を合併，1例はHH手術周術期に死亡した．一方HH手術不要と判断した7例のうち4例はFontan手術に到達し追跡期間中（中央値104.5ヵ月，100–112ヵ月）も無症状であった．

結論：単心室患者のHH修復術は，特徴的な症状や術後経過を考慮し手術適応を決定するべきである．

Background: Hiatal hernia (HH) in patients with heterotaxy syndrome can potentially affect the circulation of a single ventricle. The indications for HH repair have not been clearly established in this cohort.

Methods: We conducted a comprehensive review of the clinical course, encompassing HH complications and repairs, of 109 patients diagnosed with heterotaxy syndrome and a single ventricle. Neonates treated at our hospital from January 2008 to December 2018 were enrolled in the present study.

Results: Among the cohort, 13 patients (12％) had HH, with 6 patients undergoing subsequent HH repair. Four patients exhibited pulmonary venous compression by a herniated stomach, all of whom presented with a herniation positioned between the atrium and vertebrae, impacting the pulmonary veins from behind. Three patients underwent surgery with postoperative duodenal stenosis, and one patient died in the perioperative period of HH surgery. Among the 7 patients who did not undergo HH surgery, 4 patients successfully completed the Fontan procedure and remained asymptomatic during follow-up (median, 104.5 months; range, 100–112 months).

Conclusion: The surgical indications for HH repair in patients with a single ventricle should be determined according to their characteristic symptoms and postoperative course.

Key words: heterotaxy syndrome; single ventricle physiology; hiatal hernia; pulmonary vein stenosis; non cardiac surgery

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

原著Original

内臓錯位症候群・単心室患者に合併した食道裂孔ヘルニアの臨床経過と手術適応についての検討Clinical Course and Operative Indication of Hiatal Hernia in Patient with Heterotaxy Syndrome and Single Ventricle