de Leval M, Bull C, Stark J, Anderson R H, Taylor J F, Macartney F J
Circulation. 1982 Aug;66(2):272-80. doi: 10.1161/01.cir.66.2.272.
Sixty patients with pulmonary atresia and intact ventricular septum (PA:IVS) presenting from 1970 to 1980 are reviewed. Three groups of patient are discussed: those with tripartite right ventricles, those with no trabecular portion to the cavity, and those with neither trabecular nor infundibular portions. The decrease in early mortality for neonates with PA:IVS since 1977 (one death in 15 patients) supports our current management policy of preoperative prostaglandin E1 infusion with transpulmonary valvotomy (for patients with an infundibular cavity) combined with a left modified Blalock-Taussig shunt using a Gore-Tex prosthesis. Tricuspid valve growth, estimated by serial angiograms in 12 patients, was greater if right ventricle-to-pulmonary artery continuity was established. Later definitive repair was attempted in nine patients, with two early deaths; five underwent right ventricular outflow tract reconstruction and four had modified Fontan procedures. Neonates with critical pulmonary stenosis are also discussed. Their neonatal mortality (nine deaths in 20 patients) was similar to that of comparable patients with PA:IVS, but their actuarial survival at 5 years (55%) was superior (36% at 5 years).
对1970年至1980年期间收治的60例肺动脉闭锁合并完整室间隔（PA:IVS）患者进行了回顾性研究。讨论了三组患者：具有三部分结构右心室的患者、腔室无小梁部分的患者以及既无小梁部分也无漏斗部的患者。自1977年以来，PA:IVS新生儿的早期死亡率有所下降（15例患者中有1例死亡），这支持了我们目前的治疗策略，即术前输注前列腺素E1并进行经肺动脉瓣切开术（适用于有漏斗腔的患者），同时使用戈尔特斯（Gore-Tex）假体进行左改良布莱洛克-陶西格分流术。通过对12例患者进行连续血管造影评估三尖瓣的生长情况，结果显示如果建立了右心室与肺动脉的连续性，三尖瓣生长更为明显。随后对9例患者尝试进行最终修复，其中2例早期死亡；5例接受了右心室流出道重建，4例接受了改良Fontan手术。还讨论了患有严重肺动脉狭窄的新生儿。他们的新生儿死亡率（20例患者中有9例死亡）与PA:IVS的类似患者相似，但他们5年时的预期生存率（55%）更高（5年时为36%）。
