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Cardiovascular abnormalities in cutis laxa
European Journal of Cardiology
|May 1, 1977
Insights
Cutis laxa, a connective tissue disorder, can cause pulmonary artery branch stenoses. This condition may lead to pulmonary hypertension and right heart failure in affected children.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Pediatric Cardiology
Background:
- Cutis laxa is a rare connective tissue disorder characterized by fragmentation of elastic tissue.
- Systemic vascular manifestations include aortic and other vessel dilatation, elongation, and aneurysm formation.
Abstract:
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.