Total Aortic Replacement for a 9-Year-Old Boy With Loeys-Dietz Syndrome.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 26897206.
- Also identified by DOI 10.1016/j.athoracsur.2015.05.071.
- No licence information is recorded for this record.
- Because redistribution is not established, this page shows the abstract only. Follow the links below for the full text.
Abstract
Loeys-Dietz syndrome (LDS) is a recently identified rare connective tissue disorder caused by mutations of the transforming growth factor-β receptors and first described in 2005. It is an autosomal dominant syndrome with 2 different phenotypic expressions-LDS I and II. LDS is characterized by the triad of arterial tortuosity and aneurysm, hypertelorism, and a bifid uvula or cleft palate. We present a case of a 9-year-old boy diagnosed with LDS who underwent urgent thoracoabdominal aortic aneurysm repair followed by total arch replacement and aortic valve-sparing root replacement (AVSRR).
Medical subject headings
- Aortic Dissection
- Aortic Aneurysm, Thoracic
- Blood Vessel Prosthesis Implantation
- Imaging, Three-Dimensional
- Loeys-Dietz Syndrome