C59. Management of Subacute Painless Aortic Dissection Stanford A Debakey II in Patient With Undiagnosed Marfan Syndrome During Pregnancy

Abstract

Abstract Background Pregnant women with Marfan’s syndrome needed multidisciplinary approach to evaluate pregnancy status, fetal and maternal outcome. Marfan’s syndrome was an inherited autosomal dominant with mutation of fibrillin-I (FBN1) gene. Pregnant woman with MFS can lead to aortic dissection. It’s increase 23% of maternal mortality and 35% of fetal death. Case Summary a 30 year-old pregnant woman referred from secondary hospital care with valvular heart disease (severe aortic regurgitation due to dilatation of the aortic root and severe mitral regurgitation). Due to her severe myopia, history of aortic dissection in her family, enlarged of aortic root, pulnus excavatum, scoliosis and arachnodactyly, de novo MFS was suspected. She was diagnosed with marfan’s syndrome according her systemic score was 10 (posive >7), and calculated aortic z score was 6.2 (positive >2). Intimal tears and dilatation of the aortic root were considered to terminate the pregnancy. It’s can result bad neonatal outcome. For the management of a potential vascular catastrophe , monitoring of delivery and repairing the aortic root, we needed to multidisciplinary approach. Discussion Patient with marfan’s syndrome during pregnancy, may develop painless aortic dissection because massive dilatation of aortic arch prevent compression of pain sensitive structure. Less evidence to manage pregnant woman with MFS with aortic root dilatation more than 45 mm and or progressively dilatation of aortic root ( more than 0.5 mm/year). This patient was categorized as class IV modified WHO classification with high risk maternal mortality and severe morbidity.