Korean J Anesthesiol.  2021 Aug;74(4):342-349. 10.4097/kja.20181.

Anesthetic management of a parturient with Shone’s syndrome -a case report with review of literature-

• Bhatia K
• Eccles J
• Meessala DK

• KMID: 2520728
• DOI: http://doi.org/10.4097/kja.20181

Abstract

Background
Shone’s syndrome is a rare complex congenital cardiac condition, characterized by a supra-valvular mitral ring, parachute deformity of the mitral valve, aortic stenosis, and coarctation of the aorta. Case: A 26-year-old parturient with partial Shone’s syndrome presented to our delivery unit in pulmonary edema. She underwent a scheduled cesarean section performed under a combined spinal-epidural anesthetic at 33 weeks. She had multidisciplinary input from the cardiac, obstetric, and anesthetic teams, which led to a good outcome. A review of the five published case reports of Shone’s syndrome in pregnancy is presented along with key findings.
Conclusions
Our case report and the review highlight the successful use of combined spinal-epidural anesthetic and provides guidance to the multidisciplinary team on the varied presentation and the optimum management of women with Shone’s syndrome during the peripartum period.

Keyword

Anesthesia; Cesarean; Epidural; Labor; Shone’s Syndrome; Spinal