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///2013 Abstract Details
2013 Abstract Details2019-08-02T16:57:45-05:00

Anesthetic management in parturient with severe Marfan syndrome

Abstract Number: T 79
Abstract Type: Case Report/Case Series

Mary DiMiceli MD1

19y/o G2P1 female with Marfan syndrome complicated by severe progressive scoliosis s/p posterior spinal fusion from T3 to L3 with rods and bone grafts, thoracic aortic aneurysm s/p composite graft replacement of ascending aorta and aortic valve with St. Jude valve and mild to moderate stenosis of the prosthetic aortic valve on anticoagulation therapy presents for delivery of singleton gestation. At time of initial consultation, patient strongly requested trial of labor. Given the patient's significant medical history, the risks of neuraxial analgesia far outweigh the benefits. Furthermore, Marfan syndrome in a parturient significantly increases the risks of severe complications and mortality, with aortic dissection being the most frequent cause of death among these patients. According to the ZAHARA study, the presence of congenital heart disease carries a very high risk for peripartum cardiac events, including CHF and arrhythmias. This patient's Marfan syndrome is even further complicated by the presence of moderate stenosis of her prostethic valve, which could result in severe hemodynamic compromise if patient experiences significant increase in her afterload as occurs with valsalva or laboring. Medical management of these patients, therefore, may necessitate the use of invasive monitors. Finally, given this patient's prior cardiac surgical history being without anticoagulation carries a high risk of thromboembolic events, however maintaining anticoagulation therapy carries a higher risk of severe hemorrhage during the peripartum period. All of these factors, when examined together, argue for a very well controlled, planned elective cesarean section under general anesthesia with preparation for significant blood loss and invasive hemodynamic monitoring, and the patient must be strongly advised against neuraxial analgesia and especially against a trial of labor. Drenthen et al. Predictors of pregnancy complications in women with congenital heart disease. Eur Heart J (2010) 31 (17): 2124-2132.

SOAP 2013