Double-valve surgery in Shprintzen-Goldberg syndrome

Asian Cardiovasc Thorac Ann. 2014 Sep;22(7):842-5. doi: 10.1177/0218492313485070. Epub 2013 Oct 9.

Abstract

We describe the challenging case of a 28-year-old Jehovah's Witness patient who presented with symptomatic mitral and tricuspid valve disease and Shprintzen-Goldberg syndrome. This is the first reported double-valve surgery in such a patient who, apart from chest deformity, had a small body size, severe lung disease, difficult airway and vascular access, and to add to the complexity, refused blood and blood product use. The patient underwent a successful mitral valve replacement and tricuspid valve repair through a right thoracotomy. Apart from atrial fibrillation, he had a smooth hospital course and was discharged home on postoperative day 9.

Keywords: Heart valve prosthesis; Jehovah’s Witnesses; Shprintzen-Goldberg syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anti-Arrhythmia Agents / therapeutic use
  • Arachnodactyly / complications*
  • Arachnodactyly / diagnosis
  • Atrial Fibrillation / drug therapy
  • Atrial Fibrillation / etiology
  • Cardiac Valve Annuloplasty* / adverse effects
  • Craniosynostoses / complications*
  • Craniosynostoses / diagnosis
  • Heart Valve Prosthesis Implantation* / adverse effects
  • Humans
  • Jehovah's Witnesses
  • Male
  • Marfan Syndrome / complications*
  • Marfan Syndrome / diagnosis
  • Mitral Valve / surgery*
  • Mitral Valve Insufficiency / diagnosis
  • Mitral Valve Insufficiency / etiology
  • Mitral Valve Insufficiency / surgery*
  • Religion and Medicine
  • Thoracotomy
  • Time Factors
  • Treatment Outcome
  • Tricuspid Valve / surgery*
  • Tricuspid Valve Insufficiency / diagnosis
  • Tricuspid Valve Insufficiency / etiology
  • Tricuspid Valve Insufficiency / surgery*

Substances

  • Anti-Arrhythmia Agents

Supplementary concepts

  • Shprintzen Golberg craniosynostosis