Ikeda Tadashi, Ikai Akio
Department of Cardiovascular Surgery, Kyoto University Hospital, Kyoto, Japan
Department of Cardiovascular Surgery, Iwate Medical University Hospital, Iwate, Japan.
Multimed Man Cardiothorac Surg. 2015 Aug 28;2015. doi: 10.1093/mmcts/mmv021. Print 2015.
Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (MAPCAs) is a complex lesion. Since the concept of primary one-stage unifocalization evolved in the 1990s, the results of surgical treatment have improved significantly. From the midline approach, most of MAPCAs are dissected in the posterior mediastinal space between the ascending aorta and the superior vena cava. Extensive dissection maximizes the length of each MAPCA, which makes direct anastomosis of native tissue feasible without use of prosthetic materials. Pulmonary blood supply is established by a systemic-pulmonary shunt. The procedure was performed in 13 patients with 7.7% of hospital mortality. There was 1 late death because of infection in a patient with deletion of chromosome 22q11.2. Nine patients underwent intracardiac repair without mortality. The ratio of right ventricular systolic pressure to the systemic pressure after intracardiac repair did not exceed 0.5, except for 1 patient. Although further follow-up is necessary, midline one-stage unifocalization is considered as the standard procedure for this lesion.
肺动脉闭锁合并室间隔缺损及大量主-肺动脉侧支血管（MAPCAs）是一种复杂病变。自20世纪90年代原发性一期单灶化概念提出以来，外科治疗效果有了显著改善。从中线入路，多数MAPCAs在升主动脉和上腔静脉之间的后纵隔间隙进行游离。广泛游离可使每条MAPCA的长度最大化，从而无需使用人工材料即可实现自体组织的直接吻合。通过体-肺分流建立肺血供。该手术应用于13例患者，住院死亡率为7.7%。有1例22q11.2染色体缺失患者因感染晚期死亡。9例患者接受心内修复且无死亡。除1例患者外，心内修复后右心室收缩压与体循环压力之比不超过0.5。尽管需要进一步随访，但中线一期单灶化被认为是该病变的标准术式。
