Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.

Hemiarch replacement during pregnancy (19 weeks) utilizing normothermic selective cerebral perfusion.

Matsuda H, Ogino H, Neki R, Kitamura S

Department of Cardio-Vascular Surgery, National CardioVascular Center, Suita, Osaka, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan. hitmat@hsp.ncvc.go.jp

A 20-year-old woman with Marfan syndrome in the 19 weeks of pregnancy was admitted for a dissecting aneurysm of the ascending aorta to the proximal arch. She wanted to continue with pregnancy. A cardiotocography and a trans-esophageal echo probe which allowed visualization of the fetal heart contraction were attached on the abdomen. A cardio-pulmonary bypass was established via the right axillar and femoral arteries with bicaval drainage. The aortic isthmus and arch branches were clamped. After opening the ascending aorta, selective cerebral perfusion was initiated by quick cannulation into the left carotid and left subclavian arteries. Peripheral pressure was maintained above 80 mmHg with the flow of the selective cerebral perfusion at 1.0 L/min and flow from the femoral artery at 3.5L/min. Hemi-arch replacement was performed using a Dacron graft. The body temperature passively descended to 35 degrees C. She normally delivered a healthy female baby after 37 weeks' of gestation.

Published 23 May 2006 in Eur J Cardiothorac Surg, 29(6): 1061-3.
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