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要旨
症例は肺血管拡張薬を要する門脈肺高血圧症を合併した9歳,女児。先天性門脈欠損症に対し肝移植術が施行された。肺動脈カテーテル,経食道心エコーによるモニタリングや,一酸化窒素吸入,ミルリノンの持続投与を用いた周術期管理を行い,門脈再灌流時を含め,周術期に肺血管抵抗増大による循環破綻を来すことなく安全に管理しえた。
A liver transplant was performed in a 9-year-old girl with congenital portal insufficiency and pulmonary hypertension. A pulmonary artery catheter and transesophageal echocardiography were used during the surgery. After the start of the surgery, the patient’s mean pulmonary pressure rose to 30 mmHg. Nitric oxide inhalation and milrinone administration were therefore started, and the patient’s Fio2 and respiratory rate then increased. The nitric oxide inhalation and milrinone treatment were continued postoperatively, and the patient’s cardiac function was monitored by echocardiography. Her case was safely managed without acute right heart failure in the perioperative period, including the period of portal vein reperfusion.

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