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

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  • Sugiyama Kayo
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Matsuyama Katsuhiko
    Department of Cardiovascular Surgery, Aichi Medical University Hospital
  • Koizumi Nobusato
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Maruno Keita
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Muromachi Yukio
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Iwahori Akinari
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Takahashi Satoshi
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Iwahashi Toru
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Nishibe Toshiya
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital
  • Ogino Hitoshi
    Department of Cardiovascular Surgery, Tokyo Medical University Hospital

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Other Title
  • 重症心不全を伴ったマルファン症候群に対する部分体外循環補助下胸部下行大動脈置換術および腹部大動脈置換術

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Abstract

<p>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.</p>

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