右心不全を伴う肺高血圧症として経過し剖検にて低形成右肺動脈の血栓塞栓症と肝の結節性再生過形成症（nodular regenerative hyperplasia）の合併が診断された1例

A Case of Nodular Regenerative Hyperplasia (NRH) of the Liver with Thromboembolism of the Hypoplastic Right Pulmonary Artery and Pulmonary Hypertension

　62歳，女性．約20年前より労作時息切れを自覚，近医より加療を受けていた．1991年7月当院初診，以後肺高血圧と右心不全にて入退院を繰り返した．右心カテーテル検査にて肺動脈圧115／33mmHg，肺動脈楔入圧29mmHg，造影検査にて右肺動脈は起始部で完全閉塞，右肺動脈は左上葉動脈の完全閉塞と左主幹部の拡張，また冠動脈造影検査で右冠動脈および回旋枝より右気管支動脈に対し，側副血行を認めた．1993年5月23日，呼吸不全にて死亡した．剖検で右肺動脈は低形成で血栓により起始部で完全閉塞，また左上葉動脈の閉塞，末梢肺動脈の内腔閉塞，多発性再疎通血管腔および軽度のplexiform lesionを認めた．肝に多数の数mm大の白色軟結節あり，結節性再生過形成症（NRH）と診断した．本症例は低形成の右肺動脈が，その末梢および左肺動脈分枝に二次血栓塞栓を合併して肺高血圧症を呈したものと考えられ，NRHの成因として肺高血圧による肝うっ血との関連性が疑われた．

We report a case of a 62-year-old woman treated for pulmonary hypertension and right-sided heart failure. The value of pulmonary pressure was 115/33mmHg and pulmonary arteries revealed occlusion of the right main pulmonary artery and left upper lobe arteries. Angio-gram of the coronary artery revealed collateral circula-tion from the right coronary artery and circumflex branch to the right bronchial artery. At autopsy, throm-hoembolic occlusion of the hypoplastic right pulmonary artery and its peripheral branches as well as of the left upper lobe arteries was detected. Histological examina-tion demonstrated diffusely severe stenosis or total occlusion of the arterial lumen by thrombi organization with multivessel recanalizations and mild plexiform lesions. The liver revealed many regenerative nodules without formation of fibrous septa. which is compatible with nodular regenerative hyperplasia (NRH). NRH is rarely reported in pulmonary hypertension. but patho-genetic association between them is suggested in some of the literature.