Rare case of severe non-calcific aortic stenosis in an achondroplastic dwarf: surgical consideration

Interact Cardiovasc Thorac Surg. 2022 Feb 21;34(3):495-497. doi: 10.1093/icvts/ivab335.

Abstract

A 41-year-old patient with achondroplasia who had undergone surgery for congenital aortic stenosis >20 years ago presented with dyspnoea due to severe aortic stenosis. Computed tomography confirmed a small (16-mm) aortic annulus, thickened aortic valve leaflets without calcification and subaortic fibrous tissues. Intraoperatively, 3 non-calcific aortic leaflets were observed. Enlargement of the aortic root using a modified Manouguian technique for good exposure enabled the resection of subaortic tissues and replacement of the valve with a mechanical valve. The aortic root can be extremely small in patients with aortic stenosis and achondroplasia. The anatomy of the aortic root should be carefully assessed to enable appropriate surgical planning.

Keywords: Achondroplasia; Aortic root enlargement; Aortic stenosis; Aortic valve replacement.

Publication types

  • Case Reports

MeSH terms

  • Achondroplasia* / complications
  • Achondroplasia* / diagnostic imaging
  • Achondroplasia* / surgery
  • Adult
  • Aortic Valve / diagnostic imaging
  • Aortic Valve / surgery
  • Aortic Valve Stenosis* / complications
  • Aortic Valve Stenosis* / diagnostic imaging
  • Aortic Valve Stenosis* / surgery
  • Calcinosis*
  • Heart Valve Prosthesis*
  • Humans
  • Tomography, X-Ray Computed