Alport syndrome: A case study of chronic type A aortic dissection

Hironobu Nishiori, Tomoki Sakata, Shin ichi Makino, Makio Kawakami, Goro Matsumiya

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

A 39-year-old woman with a history of Alport syndrome was admitted to our hospital for heart failure due to severe aortic regurgitation. Computed tomography revealed a chronic type A aortic dissection that required valve-sparing aortic root replacement. The pathological examination demonstrated that elastic fibers in the tunica media of the aortic wall are torn and severely disorganized. Immunostaining showed fragmented alpha 5 chains, indicating Alport syndrome. These findings imply Alport syndrome may have connective tissue vulnerability, rendering patients susceptible to the development of aortic disease at a young age.

Original languageEnglish
Pages (from-to)2134-2137
Number of pages4
JournalJournal of Cardiac Surgery
Volume37
Issue number7
DOIs
StatePublished - Jul 2022
Externally publishedYes

Keywords

  • aortic dissection
  • aortic root replacement
  • aortic valve repair

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