Double-valve surgery in Shprintzen-Goldberg syndrome. (September 2014)

• Record Type: Journal Article
• Authors: Elmistekawy, Elsayed; Hudson, Christopher C; Williams, Anne; Mesana, Thierry
• Is Part Of: Asian cardiovascular & thoracic annals. Volume 22:Number 7(2014)
• Page Start: 842
• Page End: 845
• Publication Date: 2014-09
• Subjects: Heart valve prosthesis -- Jehovah's Witnesses -- Shprintzen-Goldberg syndrome; Heart -- Diseases -- Asia -- Periodicals; Heart -- Diseases -- Pacific Area -- Periodicals; Heart -- Diseases -- Periodicals; Heart -- Surgery -- Asia -- Periodicals; Heart -- Surgery -- Pacific Area -- Periodicals; Heart -- Surgery -- Periodicals; Chest -- Surgery -- Asia -- Periodicals; Chest -- Surgery -- Pacific Area -- Periodicals; Chest -- Surgery -- Periodicals; 617.412
• DOI: 10.1177/0218492313485070
• Languages: English
• ISSNs: 0218-4923

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.