長期酸素療法および血管拡張剤が有効であった肝硬変症に合併した肺高血圧症

Favorable effect of long term oxygen and vasodilator therapy in a patient with pulmonary hypertension associated with liver cirrhosis

　症例は56歳男性。肝硬変症に合併した肺高血圧症で，長期酸素および血管拡張剤併用療法にて著明に改善した。肺高血圧症の改善は胸部X線写真，心電図，心エコー図でも確認された。右心カテーテル検査で肺動脈圧は92／35 mmHg（mPA 56）より，75／24 mmHg（mPA 43）と低下した。治療後約2カ月にて日常生活にはほぼ無症状となり，18ヵ月を経た現在でも安定した状態が続いている。心音図および心機図で確認されたHegglin症候群は，肝硬変症のため心筋のエネルギー代謝性異常に起因する過心運動状態と考えられた。肝硬変症に合併した肺高血圧症においても，原発性肺高血圧症と同様に長期酸素および血管拡張剤併用療法が有効であることが認められた。

A 56-year-old male with moderate pulmonary hy-pertension associated with liver cirrhosis was treated successfully by long term oxygen and vasodilator therapy. Improvement of pulmonary hypertension and clinical symptoms were proved by chest X-ray, ECG, echocardiography and right heart catheteriza-tion. Hegglin's phenomenon confirmed by phono-and mechanocardiography may suggest a hyperkine-tic state as well as the so-called “energischemeta-bolische Herzinsufficienz” related to liver cirrhosis.

The patient was treated for 18 months. After 2 months of therapy the patient became asymptomatic during modest daily activity, and apparent improve-ments of hemodynamic parameters were observed thereafter. Long term oxygen and vasodilator ther-apy in this patient with pulmonary hypertension associated with liver cirrhosis proved as effective as it has been in patients with primary pulmonary hypertension.