Yamaguchi T, Kazui T, Sakai E, Watanabe A, Inoue N, Tsukamoto M, Inaoka M, Yamada O, Komatsu S
Department of Surgery (Section 2), Sapporo Medical College, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1992 Mar;40(3):393-8.
This report is concerned with results of surgical treatment for Marfan's syndrome combined with annulo-aortic ectasia (AAE) and mitral regurgitation (MR). Of the 23 patients with Marfan's syndrome who received Bentall's procedure during 14 year period, seven (30%) of these patients had both AAE and MR. The MR grade of seven patients by cardiac Doppler or left ventriculographic studies were grade 1 in 2, 2 in 1, 3 in 1, and 4 in 3. Atrial fibrillation was present in 4 patients. New York Heart Association Functional Class on admission in these 7 patients were II in 1, III in 4, and IV in 2. The mitral valve was replaced with mechanical valve in 4 patients by left atrial approach whose MR grade were over 3. In the 4 patients the mitral annuli were extremely dilated, both valve leaflets were massively redundant, and all chordae were elongated and turned chordae and vegetation were detected due to infective endocarditis. Only Bentall's procedure was performed in 3 patients whose MR were minimal. There were no early death, but two late deaths. One of them died of cardiac failure 2.3 years after Bentall's procedure because grade 2 MR was increased. Another one died from ventricular arrhythmia 1.6 years after MVR and Bentall's procedure. The remaining 5 patients are doing well for 3 months to 11.5 years after operation. For Marfan's syndrome combined with AAE and MR, early operation is recommended before left ventricular impairment. Mitral valve repair was not performed, both to save time and because anticoagulant therapy was need for aortic valve replacement. Concomitant MVR was to be done for moderate to severe MR.
本报告关注马方综合征合并主动脉瓣环扩张（AAE）及二尖瓣反流（MR）的外科治疗结果。在14年期间接受Bentall手术的23例马方综合征患者中，7例（30%）同时患有AAE和MR。通过心脏多普勒或左心室造影研究，这7例患者的MR分级为：2例为1级，1例为2级，1例为3级，3例为4级。4例患者存在心房颤动。这7例患者入院时纽约心脏协会心功能分级：1例为Ⅱ级，4例为Ⅲ级，2例为Ⅳ级。4例MR分级超过3级的患者经左心房途径行二尖瓣机械瓣置换术。这4例患者二尖瓣环极度扩张，两个瓣叶均明显冗长，所有腱索均延长，且因感染性心内膜炎检测到腱索和赘生物。3例MR极轻微的患者仅行Bentall手术。无早期死亡病例，但有2例晚期死亡。其中1例在Bentall手术后2.3年死于心力衰竭，原因是2级MR加重。另1例在二尖瓣置换术和Bentall手术后1.6年死于室性心律失常。其余5例患者术后3个月至11.5年情况良好。对于马方综合征合并AAE和MR，建议在左心室功能受损前尽早手术。未进行二尖瓣修复，一是为了节省时间，二是因为主动脉瓣置换需要抗凝治疗。对于中重度MR应同时行二尖瓣置换术。
