A family study of the biochemical defects in Wilson's disease

Abstract

The morbid anatomical factors in the production of right heart hypertrophy and failure in emphysema have been reviewed with reference to 44 cases studied by standard histological methods and by post-mortem pulmonary arteriography. The most important factors in raising the pulmonary vascular resistance in these cases are considered to be vascular deformity and pressure by the emphysematous spaces on the adjacent branches of the pulmonary artery. Precapillary bronchopulmonary arterial anastomoses may be of importance in those cases where areas of localized bronchiectasis are present. Intimal fibrosis of the pulmonary arterioles, destruction of the pulmonary vascular bed, and thromboembolic phenomena were thought to be of only minor importance in the production of cor pulmonale in emphysema.