Anesthetic management of the pregnant patient with Marfan syndrome.

Two patients with Marfan syndrome who presented for labor and delivery are reviewed. The characteristics of Marfan syndrome and their significance in relation to the physiologic changes due to pregnancy and the implications for anesthetic management are discussed. Marfan syndrome can present with musculoskeletal, cardiovascular, ocular, and pulmonary complications believed to be secondary to an inheritable disorder of connective tissue metabolism. These patients must be carefully evaluated, monitored, and managed during the prepartum and peripartum period. The physiology of pregnancy, particularly the hemodynamic changes, can have catastrophic consequences for the parturient with Marfan syndrome. Serial echocardiographic studies evaluating aortic root size and aortic and mitral valve function are essential during pregnancy. Anesthetic management focuses on minimizing aortic root shear forces and wall stress through invasive monitoring, pharmacologic intervention, and pain treatment.