12．肺動脈高度低形成症例における周術期管理

Post-operative Management After Surgery for Diminutive Branch Pulmonary Arteries

分枝肺動脈の高度低形成（diminutive branch pulmonary arteries）を伴う胎児が生存出生するには，動脈管ないし主要体肺動脈側副血行（MAPCAs）と心室中隔欠損の併存が必須である．心室中隔欠損・肺動脈閉鎖やFallot四徴症で動脈管を有する場合，プロスタグランジンE1製剤の持続投与をすみやかに開始して肺血流の維持に努めつつ，体肺動脈短絡や動脈管ステント留置によるチアノーゼ緩和と分枝肺動脈成長促進の時期と方法を検討する．肺血流源がMAPCAsに依存している場合は高度に低形成な中心肺動脈（分枝肺動脈の縦隔内部分）の灌流区域数と狭窄の程度により，中心肺動脈の成長をうながすか，MAPCAsの統合により肺血流源とするか，あるいは両者を組み合わせるかに応じて手術方法を検討する．手術時期はチアノーゼの程度（総肺灌流血流量）による．

Congenital severe peripheral pulmonary artery stenosis is usually associated with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral artery. Complications mainly related to respiratory system frequently occur after peripheral pulmonary artery patch augmentation with or without uniforcalization. Pulmonary hemorrhage and airway bleeding can be fatal. Airway compression from surrounding structures such as reconstructed central pulmonary artery, pulmonary hypertension, ventilation/perfusion mismatch, and pulmonary ischemia reperfusion injury are also life-threating complications. Long mechanical ventilator support with positive airway pressure under the deep sedation is a key to success to prevent major complications. Extra corporeal membrane oxygen support should not be hesitated if respiratory and/or hemodynamic conditions are critical. Chylothorax is a risk factor for prolonged intensive care unit stay. Several treatment options are reported, then multidisciplinary treatment with nil per oral and total parenteral nutrition is recommended.