Open Access Open Access  Restricted Access Subscription or Fee Access

Successful Surgical Treatment of Intramural Aortoatrial Fistula, Severe Aortic Regurgitation, Mitral Prolapse, and Tricuspid Insufficiency in a Patient with Ehlers-Danlos Syndrome Type IV

Shengli Jiang, Changqing Gao, Chonglei Ren, Tao Zhang

Abstract


Patients with Ehlers-Danlos syndrome (EDS) type IV, an inherited connective tissue disorder, are predisposed to vascular and digestive ruptures, and arterial ruptures account for the majority of deaths. A 31-year-old man with EDS presented with an intramural aortoatrial fistula, severe aortic regurgitation, mitral valve prolapse, and severe tricuspid valve insufficiency combined with a severely dilated left ventricle. Determining the best surgical option for the patient was not easy, especially regarding the course of action for the aortic root with a tear in the sinus of Valsalva. The fistula tract was closed at the aorta with suture and with a patch in the right atrium, the mitral valve was repaired with edge-to-edge suture and then annuloplasty with a Cosgrove ring, the aortic valve was replaced with a mechanical prosthesis, and a modified De Vega technique was used for the tricuspid valvuloplasty. The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. The considerations made to arrive at the chosen surgical course of action in this complex case are reviewed.

Full Text:

PDF


DOI: http://dx.doi.org/10.1532/HSF98.20111133

Refbacks

  • There are currently no refbacks.


Copyright 2016 Forum Multimedia Publishing, LLC. All rights reserved. The material available at this site is for educational purposes only and is NOT intended for any diagnostic, clinically related, or other purpose. Forum Multimedia Publishing, LLC, assumes no responsibility for any use or misuse of this material and makes no warranty or representation of any kind with respect to the material available at this site.