高度肺高血圧症を伴う乳児期心室中隔欠損症の肺病理組織所見と臨床像 : Septitisの電子顕微鏡的、組織計測学的検索と手術予後との関連について
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In a ventricular septal defect (VSD) with severe pulmonary hypertension (PH) in infancy, respiratory failure is one of the most frequent complications and causes of death. The prognosis of VSD+PH has been believed to depend mostly upon the degree of the pulmonary vascular obstructive disease (PVOD). However, recent studies have indicated that in infants under 12 months of age the alveolar septal pathological changes were more remarkable than the PVOD and were related to respiratory failure. Such alveolar changes are characterized by lymphoid cellular infiltration and by the thickening of the alveolar septum, which is called "septitis". The purpose of this study is to clarify the importance of alveolar septal change (septitis) as a factor in determining the prognosis of VSD+PH in infancy. Lung biopsy specimens were taken from 21 infants younger than 12 months of age with VSD+PH (Pp/Ps≧0.75). Light microscopic study was done in 21 cases and the septitis was classified into three categories according to the degree of alveolar septal pathological changes : mild, moderate and severe. In 16 out of the 21 cases, a more detailed study was made histologically and histometrically with aid of an electron microscope. Both the light and the electron microscopic findings were compared with clinical pictures, hemodynamic data and operative prognoses, and the following results and conclusions were obtained. 1) Septitis was observed in all cases with VSD+PH in infancy but severe septitis was mostly found in infants under 6 months of age. The degree of septitis was the most important factor in determining the prognosis of VSD+PH in infancy. 2) There seemed to be a tendency that the higher the degree of septitis was, the higher the ratio of pulmonary to systemic resistence (Rp/Rs) was. The increase of Rp/Rs was closely related to Dulmonarv capillary destruction.