Aortic and pulmonary root aneurysms in a child with loeys-dietz syndrome

Stefania Rizzo, Giovanni Stellin, Ornella Milanesi, Massimo Padalino, Luca A. Vricella, Gaetano Thiene, Duke E. Cameron, Cristina Basso, Vladimiro L. Vida

Research output: Contribution to journalArticlepeer-review

Abstract

We report the case of an 11-year-old boy with Loeys-Dietz syndrome, with both aortic and pulmonary aneurysms requiring cardiac operation because of progressive valve incompetence resulting from loss of coaptation of the cusps. Arterial medial changes, consisting of disarray of elastic fibers and increased collagen deposition, were observed in surgical specimens from both the aorta and the pulmonary artery of our patient, and the strong pSmad2 nuclear staining of smooth muscle cells of both aortic and pulmonary tunica media are the best evidence of transforming growth factor-β pathway activation in Loeys-Dietz syndrome.

Original languageEnglish (US)
Pages (from-to)1193-1195
Number of pages3
JournalAnnals of Thoracic Surgery
Volume101
Issue number3
DOIs
StatePublished - Mar 1 2016

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

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