Total intravenous anaesthesia for caesarean section in a patient with Marfan's syndrome

The case is described of a pregnant patient with Marfan's syndrome scheduled at 39 weeks' gestation for elective caesarean section carried out for the first time by total intravenous anaesthesia (TIVA) with continuous intravenous (i.v.) infusion of propofol. The diagnosis was based on a positive family history, classic phenotype, scoliosis, arachnodactyly, high narrow palate, hyperextensible joints, ectopia lentis and mitral valve prolapse, with a secondary low mitral insufficiency. Maternal and fetal surveillance did not detect complications during the course of pregnancy. Elective caesarean section was performed at 39 weeks due to high-risk pregnancy and to avoid the risk of haemodynamic alterations that take place during labour and delivery. The patient was given general anaesthesia with continuous i.v. infusion of propofol and boluses of atracurium and fentanyl after delivery. The haemodynamics and oxygen saturation remained stable during surgery. Apgar scores were 9 at 1 and 5 min. The post-delivery course was unremarkable and post partum echocardiography showed no changes from before caesarean section. The cardiovascular problems of Marfan's syndrome, the risk of haemodynamic changes associated with pregnancy and delivery, its anaesthetic implications and the possible advantages of TIVA with continuous i.v. infusion of propofol in the anaesthetic management of caesarean section in patients with this disease are discussed.