全肺静脈接続異常に対する外科的修復の前に安定化を行わない緊急手術は，生存率を低下させずに人工呼吸の期間を短縮する．

Emergency surgery without stabilization prior to surgical repair for total anomalous pulmonary venous connection reduces duration of mechanical ventilation without reducing survival.

抄録

背景:

緊急手術が必要な全肺静脈還流異常症（TAPVC）患者に対する2種類の手術タイミングを検討し、2種類の手術戦略の効果をまとめること。

BACKGROUND: To examine two different operation timing for treating patients with a total anomalous pulmonary venous connection (TAPVC) who need emergency surgery and to summarize the effects of the two operation strategies.

方法:

2010年12月から2019年7月までに1施設で来院後72h以内に手術を受けたTAPVC患者54例のレトロスペクティブレビューを実施した。全例が機械的換気と強心剤によるサポートを必要とする呼吸または血行動態の不安定性を示した。44例は、患者の状態が安定したため、24～72hの間に緊急手術を受けた。血行動態が安定し、手術前に安定した体内環境が維持されていた。これらの患者をStable群(SG)とした。安定した血行動態の獲得と安定した内的環境の維持に努めるのではなく、診断後24時間以内に直ちに緊急手術を受けた患者や、修正不可能なアシドーシスや進行性の心血管系虚脱により直ちに緊急手術が行われた患者が10例ある。これらの患者をUnstable群（UG）とした。病院での経過、手術データ、外来患者記録を検討した。

METHODS: A retrospective review of 54 patients with TAPVC who underwent operations within 72 h of presentation between December 2010 and July 2019 at a single institution was conducted. All patients exhibited respiratory or hemodynamic instability that required mechanical ventilation and inotropic support. Forty-four patients received emergency operations between 24 to 72 h due to stabilization of the patient's condition. Stable hemodynamics were achieved, and a stable internal milieu was maintained before the operation. These patients comprised the Stable group (SG). Rather than being subjected to efforts to obtain stable hemodynamics and maintain a stable internal milieu, ten patients received emergency operations immediately within 24 h of diagnosis or an emergency operation is performed immediately due to uncorrectable acidosis or progressive cardiovascular collapse. These patients comprised the Unstable group (UG). The hospital course, operative data, and outpatient records were reviewed.

結果:

SG群では、心外膜型が23例、心臓型が15例、心臓型が4例、混合型が2例で、未熟児が3例、残りは期産児、PDAが最も多い合併症（28例）、次いで重度の三尖弁逆流（21例）であった。UG群では、心外膜型が3例、心内膜型が4例、心内膜型が3例、心内膜型混合型は1例で、未熟児は1例のみで、残りは成熟児であった。合併症はPDA（6例）、重症三尖弁閉鎖不全症（5例）が上位2位であった。体重中央値、手術時年齢中央値、CPB（Cardiopulmonary Bypass）時間、ACC（Aortic Cross Clamp）時間の平均値は両群間に有意差はなかった。術後の人工呼吸器支持期間の中央値はSG群8.1±4.6（2～13）日、UG群4.9±2.1（2～18）日であり、有意差（p＝0.術後ICU滞在日数，入院日数はSG群8.64±4.04日，19.9±4.27日，UG群5.6±2.01日，14.7±1.75日（P＝0.026，0.002）であり，有意差は認められなかった。）院内死亡はSG群12例（27.3％），UG群2例（20％）で，死亡率に有意差はなかった（p＝0.636）。術後合併症である低心拍出量や不整脈も両群間に有意差はなかった。5年後の生存率はUG群87.5％，SG群89.9％であった．最新の追跡調査でも両群の生存率に差はなかった（SG群89.9％ vs UG群87.5％，p＝0.8115）．

RESULTS: In SG group, there were 23 exhibited the supracardiac type, 15 exhibited the cardiac type, 4 exhibited the cardiac type, and 2 exhibited the mixed cardiac type,3 patients were premature, the rest was term infant, PDA was the most common comorbidities (28 patients), the next is severe tricuspid valve regurgitation (21 patients). In UG group, there were 3 exhibited the supracardiac type, 4 exhibited the cardiac type, 3 exhibited the cardiac type, and no patient exhibited the mixed cardiac type, only 1 patient was premature, the rest were term infant. PDA (6 patients) and severe tricuspid valve regurgitation (5 patients) were the top two comorbidities. The median weight, median age at surgery, mean cardiopulmonary bypass (CPB) duration and mean aortic cross-clamp (ACC) duration were not significantly different between the two groups. The median postoperation durations of ventilator support were 8.1 ± 4.6 (2-13) days in the SG group and 4.9 ± 2.1 (2-18) days in the UG group, resulting in a significant difference (p = 0.008), the Post-op days in ICU and Days of hospitalization were 8.64 ± 4.04 days and 19.9 ± 4.27 days in the SG group and 5.6 ± 2.01 days and 14.7 ± 1.75 days in the UG group (P = 0.026 and 0.002). There were 12 hospital mortalities (27.3%) in the SG group and 2 hospital mortalities (20%) in the UG group, resulting in no significant difference in mortality (p = 0.636). Postoperative complications, such as low cardiac output and arrhythmia, were not significantly different between the two groups. The survival rates in the UG and SG groups at 5 years were 87.5 and 89.9%, respectively. There was no difference in survival between the two groups at the latest follow-up (SG group 89.9% versus UG group 87.5%, p = 0.8115).

結論:

緊急手術は遅滞なく直ちに行うべきであり、死亡率を低下させることなく、人工呼吸の期間と入院日数を短縮することができる。

CONCLUSION: An emergency operation should be performed immediately without any delay, it can reduce duration of mechanical ventilation and Days of hospitalization without reducing mortality.