Marfan syndrome presented as asymmetrical aortic root aneurysm and spontaneous isolated aortic abdominal dissection

29^th World Cardiology Conference

November 19-20, 2018 | Edinburgh, Scotland

Cun-Tao Yu and Jin-Lin Wu

National Center for Cardiovascular Disease and Fuwai Hospital, China

Posters & Accepted Abstracts: J Cardiovasc Dis Diagn

Abstract :

Isolated abdominal aortic dissection (IAAD) refers to aortic dissection (AD) inferior to the diaphragm, which is very rare. Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder, which is mostly presented as garlic-like aortic aneurysm in cardiovascular system. To the best of our knowledge, no such case concerning MFS was presented as IAAD has been reported before. A 37-year-old female with no history of hypertension was referred to our hospital for chest tightness. She had been initially diagnosed as spontaneous IAAD one month before with successful analgesic and antihypertensive treatment at a local hospital. On physical examination, the patientâ??s blood pressure was 138/80 mm Hg. Laboratory tests showed D-dimer of 2.13 ug/ml (<0.5 ug/ml), FDP of 8.42 ug/ml (0-5 ug/ml). Echocardiography revealed severe aortic regurgitation. CT showed an obvious compression of the left atrium by asymmetrical dilatation of non-coronary sinus (Figure 1-A). Abdominal aortic dissection originated distally to the superior mesenteric artery (SMA) ostium and extended downward to the bilateral common internal iliac artery (Figure 1-B). Then FBN1 mutation was found by gene analysis, thus the diagnosis of Marfan syndrome (MFS) was confirmed. She underwent Bentall procedure only and discharged on calcium channel blocker and warfarin. On follow-up at one year, the patient was doing well physically. CT scanning found no obvious extension of IAAD or aortic growth in diameter. First, this is the first case report of MFS combined with both asymmetrical aortic root aneurysm and spontaneous IAAD, which enriches our understanding of the clinical manifestations of MFS. Second, we should take aorta as a whole organ in which multiple levels of lesions may occur simultaneously, so itâ??s necessary to assess the whole aorta in order to prevent serious missed diagnosis. Third, for MFS patient with IAAD, conservative treatment under careful surveillance seems satisfactory on a short-term follow up. Further follow-up is still needed to confirm the long-term effect.

Biography :

E-mail: jinlinhorsy@outlook.com