Dysplasia of the Systemic and Pulmonary Arterial System with Tortuosity and Lengthening of the Arteries

Abstract

This article describes the case of a boy, 1 year and 5 months old, with generalized tortuosity and lengthening of all major arteries, including the coronary arteries and the pulmonary artery. The early death of the patient was attributed to coronary insufficiency and multiple severe peripheral pulmonary stenoses. The pathological changes were confined to the elastic arteries and the first part of the muscular arteries. The wall of the aorta was thickened and there was an increase of the elastic fibers. The same changes were present in the main pulmonary artery. In the large muscular arteries, the characteristic changes were thickening of the intima with hyperplasia of the elastic fibers and degenerative fragmentation of the internal elastic membrane. The walls of the coronary arteries were thickened and their lumina were narrow.