1．総肺静脈還流異常症

Emergent Operation for Total Anomalous Pulmonary Venous Return

先天性心疾患で生後すぐに緊急手術が必要な代表的疾患は，総肺静脈還流異常症（total anomalous pulmonary venous return：TAPVR），肺動脈閉鎖症，大動脈縮窄症/大動脈弓離断症であった．プロスタグランジン製剤の登場により動脈管開存の維持が可能となり，肺動脈閉鎖やductal shockに陥った大動脈縮窄症で緊急手術を要することはほとんどみなくなったが，肺静脈閉塞（pulmonary venous obstruction：PVO）と続発する肺うっ血が高度なTAPVRではPVOを解除する方法が外科手術しかなく，緊急手術の適応となる．

Total anomalous pulmonary venous return with pulmonary venous obstruction is one of the congenital heart diseases requiring an emergent operation just after birth. The patients with this condition have severe lung congestion and pulmonary hypertension, and cannot survive without relief of pulmonary venous obstruction.

During the emergent operation, an appropriate confluence between the common pulmonary venous chamber and left atrium needs to be created under cardiopulmonary bypass. The typical approaches for the anastomosis and the anastomosis techniques are summarized in this article. At the weaning from the cardiopulmonary bypass, the necessary treatments such as nitric oxide or open chest need to be applied.

The early surgical outcome for total anomalous pulmonary venous return is referenced from the Japanese National Clinical Database.