Cardiovascular abnormalities in cutis laxa.
Cutis laxa is manifest histologically by fragmentation of elastic tissue throughout the body. The aorta and other systemic vessels show dilatation, elongation and even aneurysm formation. We report multiple pulmonary artery branch stenoses in a child who had cutis laxa. Pulmonary artery stenoses may be the cause of the pulmonary hypertension and right heart failure observed in patients with this condition.
D002318:Cardiovascular Diseases / Q000150:complications*; D002675:Child, Preschool; D003251:Constriction, Pathologic; D003483:Cutis Laxa / Q000150:complications*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications; D011651:Pulmonary Artery