Marfan Syndrome (See also Chap. 357) This is an autosomal dominant disease, associated with a high risk of maternal morbidity. Approximately 15% of pregnant women with Marfan syndrome develop a major cardiovascular manifestation during pregnancy, with almost all women surviving. | Chapter 007. Medical Disorders during Pregnancy Part 4 Marfan Syndrome See also Chap. 357 This is an autosomal dominant disease associated with a high risk of maternal morbidity. Approximately 15 of pregnant women with Marfan syndrome develop a major cardiovascular manifestation during pregnancy with almost all women surviving. An aortic root diameter 40 mm is considered to be associated with a favorable outcome of pregnancy. Prophylactic therapy with beta blockers has been advocated although large-scale clinical trials in pregnancy have not been performed. Pulmonary Hypertension See also Chap. 244 Maternal mortality in the setting of severe pulmonary hypertension is high and primary pulmonary hypertension is a contraindication to pregnancy. Termination of pregnancy may be advisable in these circumstances to preserve the life of the mother. In the Eisenmenger syndrome . the combination of pulmonary hypertension with right-to-left shunting due to congenital abnormalities Chap. 229 maternal and fetal death occur frequently. Systemic hypotension may occur after blood loss prolonged Valsalva maneuver or regional anesthesia sudden death secondary to hypotension is a dreaded complication. Management of these patients is challenging and invasive hemodynamic monitoring during labor and delivery is generally recommended. In patients with pulmonary hypertension vaginal delivery is less stressful hemodynamically than cesarean section which should be reserved for accepted obstetric indications. Deep Venous Thrombosis and Pulmonary Embolism See also Chap. 256 A hypercoagulable state is characteristic of pregnancy and deep venous thrombosis DVT occurs in about 1 in 2000 pregnancies. Pulmonary embolism is one of the most common causes of maternal death in the United States. In pregnant women DVT occurs much more commonly in the left leg than in the right leg due to the compression of the left iliac vein by the iliac artery and the uterus. Activated protein C resistance caused
