Imai Y, Sawatari K, Hoshino S, Ishihara K, Nakazawa M, Momma K
Department of Pediatric Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women's Medical College.
J Thorac Cardiovasc Surg. 1994 May;107(5):1272-83.
Since June 1989, 18 patients with atrioventricular discordant anomalies under 15 years of age underwent anatomic correction, or double switch operation, with 2 hospital deaths (11%). Ages ranged from 1 year 4 months to 12 years (mean 7.2 years) and body weights ranged from 7.1 to 32.5 kg (mean 20.1 kg) at operation. Visceral situs was solitus in 12 and inversus in 6. Ventriculoarterial connection was discordant in 4 and double-outlet in 14. All had associated congenital cardiac anomalies consisting of ventricular septal defect in 17, atrial septal defect in 9, pulmonary atresia in 10, pulmonary stenosis in 6, systemic tricuspid regurgitation in 9, mitral regurgitation in 6, bilateral superior venae cavae in 6, patent ductus arteriosus in 6, Wolff-Parkinson-White syndrome in 2, and congenital atrioventricular block in 1. Previous palliation had been performed 19 times in 11 patients including systemic-pulmonary shunts in 10 patients and pulmonary banding in 1 patient. Anatomic correction consisted in the Mustard procedure in 12 or the Senning procedure in 6 at the atrial level and in external conduit repair in 14, arterial switch operations in 3, or direct anastomosis between the pulmonary artery and right ventricle in 1 at the ventriculoarterial level. The arterial switch operation was indicated in patients with a normal pulmonary valve, and external conduit repair was indicated for patients with pulmonic stenosis or atresia. Anatomic right ventricular end-diastolic volume showed significant reduction from 122.2% +/- 46.3% of normal to 78.8% +/- 24.0%, mainly owing to unloading of the ventricle after the operation (p < 0.0003). Right ventricular ejection fraction remained unchanged (56% +/- 8% to 56% +/- 6%). Anatomic left ventricular end-diastolic volume and left ventricular ejection fraction remained unchanged before and after the operation: volume 134.6% +/- 50.0% of normal to 127.3% +/- 32.8% and ejection fraction from 61% +/- 8% to 56% +/- 8%. Cardiac index averaged 3.1 +/- 0.5 L/min per square meter after the operation. The double switch operation is best indicated in patients with atrioventricular discordance associated with systemic tricuspid regurgitation and/or with poor systemic right ventricular function.
自1989年6月以来，18例15岁以下的房室不协调畸形患者接受了解剖矫正术，即双调转手术，其中2例患者在医院死亡（11%）。手术时年龄范围为1岁4个月至12岁（平均7.2岁），体重范围为7.1至32.5千克（平均20.1千克）。内脏位置正常者12例，内脏反位者6例。心室-动脉连接不一致者4例，双出口者14例。所有患者均伴有先天性心脏畸形，其中室间隔缺损17例，房间隔缺损9例，肺动脉闭锁10例，肺动脉狭窄6例，三尖瓣反流9例，二尖瓣反流6例，双侧上腔静脉6例，动脉导管未闭6例，预激综合征2例，先天性房室传导阻滞1例。11例患者曾接受过19次姑息性手术，其中10例患者行体-肺分流术，1例患者行肺动脉环缩术。解剖矫正术在心房水平采用Mustard手术12例或Senning手术6例，在心室-动脉水平采用外管道修复术14例、动脉调转手术3例或肺动脉与右心室直接吻合术1例。肺动脉瓣正常的患者行动脉调转手术，肺动脉狭窄或闭锁的患者行外管道修复术。解剖学上右心室舒张末期容积从正常的122.2%±46.3%显著降至78.8%±24.0%，主要是由于术后心室负荷减轻（p<0.0003）。右心室射血分数保持不变（56%±8%至56%±6%）。解剖学上左心室舒张末期容积和左心室射血分数在手术前后保持不变：容积从正常的134.6%±50.0%降至127.3%±32.8%，射血分数从61%±8%降至56%±8%。术后心脏指数平均为3.1±0.5升/分钟每平方米。双调转手术最适合于伴有三尖瓣反流和/或体循环右心室功能不良的房室不协调患者。
