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法洛四联症:选择性手术治疗可将手术死亡率降至最低。

Tetralogy of Fallot: selective surgical management can minimize operative mortality.

作者信息

Hammon J W, Henry C L, Merrill W H, Graham T P, Bender H W

出版信息

Ann Thorac Surg. 1985 Sep;40(3):280-4. doi: 10.1016/s0003-4975(10)60042-2.

Abstract

Although tetralogy of Fallot can be completely corrected at any age, it appears that preliminary shunting may be of benefit in infancy. To better define the optimum age for shunting, the following patient data were reviewed. One hundred five patients with tetralogy of Fallot were treated between December, 1975, and July, 1984. Eight of 100 patients undergoing intracardiac repair died. Operative mortality was 14.3% (4 of 28 patients) when intracardiac repair was done before the patient was a year old, but only 5.6% (4 of 72 patients) after that age. Seven (13%) of 54 patients requiring transannular patch reconstruction of the right ventricular outflow tract died, whereas only 1 (2.2%) of 46 having repair without this type of reconstruction died (p less than 0.05). Twenty-one patients were initially palliated with systemic-pulmonary artery shunts, with 1 noncardiac death related to associated congenital neurological anomalies (4.8%). There was no statistically significant difference in mortality for primary versus secondary intracardiac repair. Left ventricular end-diastolic volume determination in 26 patients less than 2 years old demonstrated that 14 patients had small left ventricles and that 3 of them less than 1 year of age died at corrective operation. The remaining 12 had a left ventricle of normal size, and 1 of them died at operation. Palliative shunts can be performed safely in the very young child with no increased mortality at subsequent intracardiac repair. Diffuse hypoplasia of the right ventricular outflow tract and pulmonary arteries requiring transannular patch reconstruction, and age less than 1 year both alter operative mortality, their combined impact being additive.

摘要

虽然法洛四联症可在任何年龄得到完全矫正,但似乎在婴儿期进行初步分流可能有益。为了更好地确定分流的最佳年龄,对以下患者数据进行了回顾。1975年12月至1984年7月期间,对105例法洛四联症患者进行了治疗。100例行心内修复的患者中有8例死亡。患者1岁前进行心内修复时,手术死亡率为14.3%(28例中的4例),但1岁后手术死亡率仅为5.6%(72例中的4例)。54例需要经环修补重建右心室流出道的患者中有7例(13%)死亡,而46例未进行此类重建的修复患者中只有1例(2.2%)死亡(p<0.05)。21例患者最初采用体肺分流术进行姑息治疗,1例非心脏死亡与相关先天性神经异常有关(4.8%)。一期与二期心内修复的死亡率无统计学显著差异。对26例2岁以下患者的左心室舒张末期容积测定表明,14例患者左心室较小,其中3例1岁以下患者在矫正手术时死亡。其余12例左心室大小正常,其中1例在手术时死亡。在非常年幼的儿童中进行姑息性分流术是安全的,后续心内修复时死亡率不会增加。右心室流出道和肺动脉弥漫性发育不全需要经环修补重建,以及年龄小于1岁均会改变手术死亡率,它们的综合影响是累加的。

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