Watanabe S, Matsuda H, Nakano S, Shimazaki Y, Miura T, Kawashima Y, Sano T, Kishimoto H
First Department of Surgery, Osaka University Medical School.
Kyobu Geka. 1992 Jun;45(6):487-92.
Eleven patients with syndromes of asplenia and polysplenia associated with total anomalous pulmonary venous drainage (TAPVD) were underwent Blalock-Taussig (B-T) shunt operation for reduction of pulmonary blood flow. The age of patients at operation were 6 days to 5 years (average 19.5 +/- 18.7 month). There were seven patients in supracardiac type and four in cardiac type of TAPVD. All patients didn't present obstruction to pulmonary venous return (PVO) before B-T shunt operation. There were one early (9%) and two late deaths (20%) after surgery. Although the hospital death was related to perioperative errors. Two late deaths were not due to the PVO. One of infants had moderately pulmonary congestion and cardiac failure after shunt procedure. Two patients were measured pressure gradient (3 to 4 mmHg) between common pulmonary vein to atrium chamber before shunt procedure. Repeated catheterization revealed that 6 of them could be measured pressure gradient, 3 to 7 mmHg, and no patients had clinical sign of the PVO. Our results demonstrated that B-T shunt operation could be satisfactory for syndromes of asplenia and polysplenia associated with reduced pulmonary blood flow and TAPVD.
11例患有无脾综合征和多脾综合征并伴有完全性肺静脉异位引流（TAPVD）的患者接受了Blalock-Taussig（B-T）分流手术，以减少肺血流量。手术时患者年龄为6天至5岁（平均19.5±18.7个月）。心上型TAPVD患者7例，心内型4例。所有患者在B-T分流手术前均未出现肺静脉回流梗阻（PVO）。术后有1例早期死亡（9%）和2例晚期死亡（20%）。尽管医院死亡与围手术期失误有关。2例晚期死亡并非由于PVO。1例婴儿在分流手术后出现中度肺充血和心力衰竭。2例患者在分流手术前测量了肺总静脉至心房腔之间的压力梯度（3至4 mmHg）。重复导管检查显示，其中6例可测量到压力梯度，为3至7 mmHg，且无患者有PVO的临床体征。我们的结果表明，B-T分流手术对于伴有肺血流量减少和TAPVD的无脾综合征和多脾综合征可能是令人满意的。
