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单心室合并体循环流出道梗阻:经原发性达姆斯手术进行姑息治疗。

Univentricular heart with systemic outflow obstruction: palliation by primary Damus procedure.

作者信息

Brawn W J, Sethia B, Jagtap R, Stümper O F, Wright J G, De Giovanni J V, Silove E D, Jackson M, Sreeram N

机构信息

Heart Unit, Birmingham Children's Hospital, England.

出版信息

Ann Thorac Surg. 1995 Jun;59(6):1441-7. doi: 10.1016/0003-4975(95)00147-d.

DOI:10.1016/0003-4975(95)00147-d
PMID:7539607
Abstract

In 24 consecutive infants (19 male and 5 female) with complex forms of single-ventricle physiology and systemic outflow obstruction, a modified Damus operation without the use of exogenous material was undertaken in conjunction with creation of an aortopulmonary shunt 3.5 mm in diameter. The median age at operation was 6 days (range, 1 to 170 days) and the median weight, 3.4 kg (range, 2.6 to 4.6 kg). There were nine early deaths. All 15 survivors (median follow-up, 6.5 months) were clinically well without major systemic ventricular dysfunction or atrioventricular or arterial valve regurgitation. Ten of them have undergone a superior vena cava-pulmonary shunt (one death), and 1 has required patch angioplasty of the aortic arch and innominate artery with revision of the aortopulmonary shunt. The 4 other survivors are awaiting a cavopulmonary shunt. Univariate analysis yielded the chronologic rank for an individual procedure (higher risk of death early in the series), presence of aortic arch atresia, and presence or absence of transposition of the great arteries as predictors of death. This aggressive surgical approach provides excellent early palliation, and because the operation prevents abnormal ventricular hypertrophy from pressure or volume overload, systemic ventricular function is optimally conserved for a future Fontan-type procedure.

摘要

对24例(19例男性,5例女性)患有单心室生理复杂形式和体循环流出道梗阻的连续婴儿,在不使用外源材料的情况下进行改良达穆斯手术,并同时建立直径3.5毫米的主肺动脉分流。手术时的中位年龄为6天(范围1至170天),中位体重为3.4千克(范围2.6至4.6千克)。有9例早期死亡。所有15名幸存者(中位随访时间6.5个月)临床状况良好,无严重的体循环心室功能障碍或房室瓣或动脉瓣反流。其中10例接受了上腔静脉-肺动脉分流术(1例死亡),1例需要对主动脉弓和无名动脉进行补片血管成形术并修正主肺动脉分流。另外4名幸存者正在等待进行腔肺分流术。单因素分析得出个体手术的时间顺序排名(系列早期死亡风险较高)、主动脉弓闭锁的存在以及大动脉转位的有无作为死亡的预测因素。这种积极的手术方法提供了良好的早期姑息治疗,并且由于该手术可防止心室因压力或容量过载而出现异常肥厚,因此体循环心室功能可得到最佳保留,以便未来进行Fontan型手术。

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