General anesthesia using remifentanil for Cesarean delivery in a parturient with Marfan's syndrome.

PURPOSE: Due to cardiovascular and skeletal abnormalities, anesthetic management of parturients with Marfan's syndrome can be particularly challenging. Parturients with aortic root dilatation are at risk for aortic dissection. We describe the anesthetic management of a parturient with Marfan's syndrome and aortic root dilatation, who required general anesthesia for Cesarean delivery.

CLINICAL FEATURES: At 26 weeks gestation, a nulliparous woman with Marfan's syndrome presented to the Anesthesia Clinic. Her history revealed asymptomatic aortic root dilatation of 41 mm, and partial correction of scoliosis with Harrington rods. Her cardiologist advised metoprolol, serial echocardiograms, and Cesarean delivery to decrease the risk of aortic dissection. At a multidisciplinary conference, a decision was made to proceed with Cesarean delivery, at term, at the cardiac surgery centre. After placement of arterial and central lines, general anesthesia was induced with remifentanil, propofol, and succinylcholine. Anesthesia was maintained with N(2)O, sevoflurane, and remifentanil (0.02-0.08 microg x kg(-1) x min(-1)). Transesophageal echocardiography examination confirmed stable aortic root dilatation. The patient remained hemodynamically stable. The baby's Apgars were 4 and 8, at one and five minutes, respectively. At the end of the procedure, the patient's trachea was extubated when she was awake. Initial postoperative care was in the intensive care unit. Both mother and baby recovered uneventfully.

CONCLUSIONS

Peripartum hemodynamic changes can be life-threatening to the parturient with Marfan's syndrome and aortic dilatation. Anesthetic goals for delivery included preparation for possible aortic dissection, and avoidance of increased aortic root shear stress, through careful hemodynamic monitoring, and general anesthesia using remifentanil.