Sakamoto Shun-Ichiro, Amitani Ryosuke, Motoji Yusuke, Yamaguchi Takako, Hiromoto Atsushi, Suzuki Kenji, Ishii Yosuke
Department of Cardiovascular Surgery, Nippon Medical School Musashikosugi Hospital, 1-396 Kosugimachi, Nakahara-ku, Kawasaki-shi, Kanagawa, 211-8533, Japan.
Department of Cardiovascular Surgery, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
Surg Case Rep. 2022 Jul 27;8(1):140. doi: 10.1186/s40792-022-01504-8.
Scoliosis is one of the symptoms manifested by patients with Marfan syndrome (MFS). Deformity of the thoracic cavity due to severe scoliosis may cause difficulty during cardiac surgery in terms of the surgical approach and instrument manipulation; however, only a few reports have been available regarding the surgical case of MFS with severe scoliosis. Here, we report a case of combined aortic valve replacement and left atrial appendage closure in a patient with MFS who had severe scoliosis using lower hemisternotomy.
A 62-year-old female with MFS was referred to our hospital after being diagnosed with severe aortic regurgitation and paroxysmal atrial fibrillation with a history of cerebral thromboembolism. The aortic valve showed severe insufficiency due to cusp prolapse, whereas the aortic root was moderately dilated (42 mm). Echocardiography revealed severe regurgitation with reduced left ventricular ejection function (32%) and massive left ventricular diastolic dimension (88 mm). Moreover, combined aortic valve replacement and left atrial appendage closure was indicated. However, the patient had chest deformity due to severe scoliosis. Thus, conventional full sternotomy or thoracotomy was considered an inappropriate surgical approach. Lower hemisternotomy was selected on the basis of three-dimensional reconstruction imaging of the aorta, left atrial appendage, sternum, and rib. Sternal elevation and rib retraction with the costal arch folded back provided enough surgical field for the combined procedures to be safely conducted. The postoperative course was uneventful, except for predicted prolonged mechanical ventilation with the assistance of intraaortic balloon pumping. Thereafter, the patient has been free from any cardiac and cerebrovascular event.
Lower hemisternotomy can be useful for combined cardiac surgery in MFS with severe scoliosis.
脊柱侧弯是马方综合征（MFS）患者表现出的症状之一。严重脊柱侧弯导致的胸腔畸形可能会在心脏手术的手术入路和器械操作方面造成困难；然而，关于患有严重脊柱侧弯的MFS患者的手术病例报告较少。在此，我们报告一例使用低位半胸骨切开术对患有严重脊柱侧弯的MFS患者进行主动脉瓣置换和左心耳闭合联合手术的病例。
一名62岁患有MFS的女性在被诊断为严重主动脉瓣反流和阵发性心房颤动且有脑血栓栓塞病史后被转诊至我院。主动脉瓣因瓣叶脱垂而显示严重关闭不全，而主动脉根部中度扩张（42毫米）。超声心动图显示严重反流，左心室射血功能降低（32%），左心室舒张末期内径增大（88毫米）。此外，需要进行主动脉瓣置换和左心耳闭合联合手术。然而，该患者因严重脊柱侧弯导致胸部畸形。因此，传统的全胸骨切开术或开胸术被认为是不合适的手术入路。基于主动脉、左心耳、胸骨和肋骨的三维重建成像选择了低位半胸骨切开术。胸骨抬高和肋弓折返的肋骨牵开为联合手术提供了足够的手术视野，使其能够安全进行。术后过程顺利，除了预计在主动脉内球囊泵辅助下机械通气时间延长。此后，患者未发生任何心脏和脑血管事件。
低位半胸骨切开术对于患有严重脊柱侧弯的MFS患者进行联合心脏手术可能是有用的。
