Japanese Journal of Cardiovascular Surgery Vol47,No2

Graft Replacement with Partial Extra-corporeal Circulation of Descending Thoracic and Abdominal Aortic Aneurysms in Marfan Syndrome Combined with Severe Left Ventricular Dysfunction and Mitral Regurgitation
Kayo Sugiyama* Katsuhiko Matsuyama** Nobusato Koizumi*
Keita Maruno* Yukio Muromachi* Akinari Iwahori*
Satoshi Takahashi* Toru Iwahashi* Toshiya Nishibe*
Hitoshi Ogino*

(Department of Cardiovascular Surgery, Tokyo Medical University Hospital*, Tokyo, Japan, and Department of Cardiovascular Surgery, Aichi Medical University Hospital**, Aichi, Japan)

We report on a rare case of Marfan syndrome with giant dissecting descending thoracic and abdominal aortic aneurysms associated with poor left ventricular function and severe mitral regurgitation. Before the anesthetic induction, a partial extra-corporeal circulation was established to prevent a collapse of the circulation. Descending aortic graft replacement and following abdominal aortic graft replacement were performed safely using the partial extra-corporeal circulation to relief the afterload for the severely deteriorated left ventricle with severe mitral regurgitation. Intra-aortic balloon pumping was also promptly used to assist the poor circulation in the postoperative period. Despite the admission to a specialized institute, he died from irreversible heart failure with a developing renal failure. Even for a difficult patient with Marfan syndrome with severe left ventricular dysfunction and mitral regurgitation, graft replacement was feasible with meticulous perioperative circulatory management using partial extra-corporeal circulation and intra-aortic balloon pumping. However, a prompt registration for heart transplantation and an aortic surgery concomitant with implantation of left ventricular assisted device should have been considered to save the patient.

 

Jpn. J. Cardiovasc. Surg. 47:71-77(2018)

Keywords:Marfan syndrome;left ventricular dysfunction;aortic aneurysm;partial extra-corporeal circulation;intra-aortic balloon pumping


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