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

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.