典型的左心低形成症候群の僧帽弁／大動脈弁サブタイプ別による小児期予後

Hirofumi Fukunaga; 福永啓文; Makoto Nakamura; 中村真; Shiro Ishikawa; 石川司朗; Yoshihiko Kodama; 兒玉祥彦; Koichi Sagawa; 佐川浩一; Toshihide Nakano; 中野俊秀; Hideaki Kado; 角秀秋; Ichiro Sakamoto; 坂本一郎

doi:10.9794/jspccs.34.3

典型的左心低形成症候群の僧帽弁／大動脈弁サブタイプ別による小児期予後Comparison of Childhood Outcome by Mitral Valve and Aortic Valve Subtypes of Typical Hypoplastic Left Heart Syndrome

福永啓文¹，中村真¹，石川司朗¹，兒玉祥彦¹，佐川浩一¹，中野俊秀²，角秀秋²，坂本一郎³Hirofumi Fukunaga¹, Makoto Nakamura¹, Shiro Ishikawa¹, Yoshihiko Kodama¹, Koichi Sagawa¹, Toshihide Nakano², Hideaki Kado², Ichiro Sakamoto³

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

² 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

³ 九州大学病院循環器内科Department of Cardiology, Kyushu University Hospital ◇ Fukuoka, Japan

受付日：2017年10月13日Received: October 13, 2017

受理日：2017年12月18日Accepted: December 18, 2017

発行日：2018年1月1日Published: January 1, 2018

背景：左心低形成症候群（HLHS）は周術期管理や外科治療の進歩に伴い生存率は向上したが，現在でもFontan到達率が低く予後は悪い．HLHSが心室中隔欠損のない典型的左心低形成症候群（typical HLHS）だけでなく非典型的左心低形成症候群なども包含し複雑な血行動態を有するため，予後因子の解析が困難である．typical HLHSを僧帽弁／大動脈弁の狭窄または閉鎖の組み合わせによる4群に分類し，生存率や死亡の危険因子を検討することで，今後のFontan到達および予後向上に役立てることを目的とした．

方法：typical HLHS＝119例（MA/AA＝61, MA/AS＝4, MS/AA＝24, MS/AS＝30例）を対象にした．症例の少ないMA/ASを除くMA/AA, MS/AAおよびMS/ASの3群間別生存率，死亡数，死因を検討し，胎内診断，MS/AA, 低出生体重，房室弁閉鎖不全，心外合併症，狭小心房間交通，初回術後ICU滞在日数および初回術式を変量として解析した．

結果：typical HLHS全体の5年生存率は58％，サブタイプ別にMS/AS 72％, MA/AA 59％, MS/AA 33％で有意差を認めた（p＝0.002）．多変量解析でMS/AA（p＝0.002）と胎内診断陽性（p＝0.03）が予後に関する有意な危険因子とされた．

結論：typical HLHSにおいてMS/AAは予後不良因子であった．TCPC後予後は他群と変わらないことから，術前診断の向上，右心バイパス前の治療戦略の改善が必要である．

Background: Although progress in perioperative management and surgical treatment has improved the survival rate of patients with hypoplastic left heart syndrome (HLHS), the Fontan completion rate remains low and prognosis is poor. Analysis of prognostic factors is challenging because HLHS complicates hemodynamics in patients with typical HLHS without ventricular septal defect and nontypical HLHS. This study aimed to categorize typical HLHS into four groups with combinations of stenosis/atresia of the mitral valve/aortic valve and to investigate survival rates and mortality risk factors to improve Fontan completion and prognosis.

Methods: We enrolled 119 patients with HLHS [mitral valve atresia (MA)/aortic valve atresia (AA): 61 patients; MA/aortic valve stenosis (AS): 4; mitral valve stenosis (MS)/AA: 24; and MS/AS: 30)]. After excluding the MA/AS group because of a small number of patients, we investigated the survival rate, number of mortalities, and cause of death in the MA/AA, MS/AA, and MS/AS groups by using prenatal diagnosis, MS/AA, low birth weight, atrioventricular valvular insufficiency, noncardiac complications, restricted foramen ovale, and length of intensive care unit stay following initial surgery as variables.

Results: The overall 5-year survival rate for typical HLHS was 58％, which significantly varied by subtype (MS/AS: 72％; MA/AA: 59％; and MS/AA: 33％; p＝0.002). Multivariate analysis identified MS/AA (p＝0.002) and positive prenatal diagnosis (p＝0.03) as significant prognostic risk factors.

Conclusion: In typical HLHS, MS/AA was found to be an adverse prognostic factor. Because prognosis after total cavopulmonary connection was similar to those of other groups, preoperative diagnosis and treatment strategies before right heart bypass must be improved.

Key words: hypoplastic left heart syndrome; congenital heart disease; cardiac surgery; outcome; mitral valve stenosis aortic valve atresia

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

原著Original

典型的左心低形成症候群の僧帽弁／大動脈弁サブタイプ別による小児期予後Comparison of Childhood Outcome by Mitral Valve and Aortic Valve Subtypes of Typical Hypoplastic Left Heart Syndrome