肺動脈閉鎖病人接受瓣膜擴張術擴右心室出口整形治療發育不良之右心室成長之研究

Abstract

目的: 本研究之目的在於探討肺動 脈閉鎖且無心室中隔缺損之病人在 瓣膜整型後其原先發育不良的右心 室是否會生長。 方法與結果： 從1995 年6 月至2001 年8 月，在本院有44 位新生兒患有肺動 脈瓣閉鎖且心室中隔無缺損，接受 心導管攝影及檢查，其中7 例由於 右心室嚴重發育不全(三尖瓣Z 值< -4)或有冠狀動脈管無法做肺動脈 瓣整形，成為對照組，其餘37 例接 受右心室減壓，包括原先使用心導 管31 例，右心室出口整形手術6 例，31 例初期成功28 例(4 例使用引 導線，24 例使用高周波導線)，失敗 3 例，最終成功20 例，(6 例需開刀， 2 例死亡)，因此手術共有3 例死亡， 34 例包括20 例心導管治療，14 例 手術，供作右心室成長之分析，34 例病人之血氧飽和度均在75%以 上。34 例，追蹤平均1.4 年，三尖 瓣平均Z 值由原先的–1.7 + 1.2 變 成， –0.8 + 1.3 (P <0.01, parted -t)， 而對照組7 例的三尖瓣的Z 值沒有 改變，平均值由原先–3.2 + 0.6 成為 -3.6 + 0.8。(P >0.05) 結論： 肺動脈瓣閉鎖且無心室中隔 缺損的病人，若在新生兒期建立右 心室與肺動脈的通路，發育不良的 右心室是有機會成長。

Between June 1995 and August 2001, 44 neonates with PA/IVS underwent cardiac catheterization and angiography. Thirty-seven patients tricuspid valve Z-value ＞-4 (ranging from –3.2 to 0.6, mean -1.4 ± 0.8) without significant sinusoid underwent right ventricle decompression in the neonatal period. Seven patients in whom decompression of right ventricle was considered as contraindicated because of severe hypoplasia of right ventricle tricuspid valve Z < -4 or sinusoid-coronary communications were used as controls. Of the 37 patients, transcatheter pulmonary valvotomy was attempted in 31 and was successful in 28 patients: 4 with a guidewire and 24 with a radiofrequency guidewire. Mean while, 6 neonates with PA/ IVS underwent right ventricular outflow tract reconstruction with or without a shunt. Six of the 28 patients further required a right ventricular out flow tract patch, despite an initial success in pulmonary valvotomy. Of the 37 patients, there were 3 mortalities.Therefore, a total of 34 patients who underwent decompression of right ventricle (14 RVOT patch and 20 transcatheter valvotomy) were available for follow up. A total of 34 patients who were discharged with systemic O2 saturation above 75% were enrolled in this sutudy. After a follow-up period ranging from 2 to 62 months, the mean Z value of tricuspid value measured an echocardiography increased from –1.7 + 1.2 to –0.8 + 1.3 during follow-up. However, of the 7 patients managed with a modified B-T shunt, the Z value of tricuspid valve remained unchanged. (–3.2 + 0.6 vs. –3.6 + 0.8, P >0.05) Conclusion：Right ventricle could grow in PA-IVS patients if patients decompression could be performed in early infancy.