Heart Institute, Children's Hospital Los Angeles, Keck School of Medicine of the University of Southern California, Los Angeles, California; Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of the University of Southern California, Los Angeles, California.
Heart Institute, Children's Hospital Los Angeles, Keck School of Medicine of the University of Southern California, Los Angeles, California; Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of the University of Southern California, Los Angeles, California; Department of Pediatrics, Keck School of Medicine of the University of Southern California, Los Angeles, California.
Semin Thorac Cardiovasc Surg. 2019 Summer;31(2):234-241. doi: 10.1053/j.semtcvs.2018.09.019. Epub 2018 Sep 29.
A subset of neonates with single ventricle (SV) physiology has antegrade pulmonary blood flow that is deemed unlikely to be reliable until Glenn. We have used systemic to pulmonary shunt (SPS) with pulmonary artery banding (PAB) to optimize pulmonary blood flow while maintaining reserve antegrade flow. We hypothesize that this is an effective strategy that can be accomplished without the routine need for cardiopulmonary bypass. We retrospectively reviewed the records of 60 neonates who underwent combined SPS + PAB between 2004 and 2015. Data are presented as median with quartiles. Children were 8 (4-19) days old at surgery and included 38 (63%) boys. Atresia or severe stenosis of the subpulmonary atrioventricular (AV) valve associated with pulmonary blood flow across a bulboventricular foramen was present in 37 (62%). In 20 (33%), heterotaxy-associated unbalanced AV canal with pulmonary stenosis with or without anomalous pulmonary venous drainage was present. First-stage palliation was accomplished without cardiopulmonary bypass in 44 patients (73%). There were 7 (12%) hospital deaths, 4 among the 20 (20%) with heterotaxy. Fifty-three children were followed for a median 5.1 (1.8-8.2) years. Three early reinterventions were required after initial palliation (1 PAB adjustment, 2 SPS balloon angioplasties). Five additional heterotaxy patients experienced late mortality during follow-up. There were no early or emergent Glenn. Thirty-nine patients have reached Fontan circulation with a median pre-Fontan PA pressure of 14 (12-18) mm Hg. One patient converted to biventricular physiology and the remaining await completion Fontan. Heterotaxy was the only independent predictor of mortality (hazard ratio 10 (2.3-44, P < 0.001). In SV patients with unreliable antegrade PA flow, SPS + PAB is an effective first-stage palliation. SV patients with heterotaxy are at increased risk for mortality.
一组患有单心室（SV）生理功能的新生儿中，有一部分存在前向肺血流，但直到 Glenn 手术前，这种血流被认为是不可靠的。我们使用体肺分流（SPS）加肺动脉环扎术（PAB）来优化肺血流，同时保持前向血流储备。我们假设这是一种有效的策略，可以在不常规需要心肺转流的情况下完成。我们回顾性分析了 2004 年至 2015 年间接受联合 SPS+PAB 手术的 60 例新生儿的病历资料。数据以中位数和四分位数表示。患儿手术时年龄为 8（4-19）天，其中 38 例（63%）为男性。37 例（62%）存在亚肺动脉瓣下房室（AV）瓣狭窄或闭锁，伴有经卵圆孔的肺血流。20 例（33%）存在非均衡性 AV 心房间隔缺损伴或不伴异常肺静脉引流，其中 4 例为右位心。44 例（73%）患儿在无体外循环的情况下完成一期姑息手术。7 例（12%）患儿院内死亡，其中 4 例来自于 20 例（20%）右位心患儿。53 例患儿获得中位 5.1（1.8-8.2）年的随访。初次姑息手术后需再次干预 3 例（1 例 PAB 调整，2 例 SPS 球囊血管成形术）。5 例右位心患儿在随访期间发生晚期死亡。无早期或紧急 Glenn 手术。39 例患儿已进入 Fontan 循环，Fontan 前肺动脉压中位数为 14（12-18）mmHg。1 例患儿转为双心室生理功能，其余患儿等待完成 Fontan 手术。右位心是唯一的独立死亡预测因素（风险比 10[2.3-44]，P<0.001）。在存在不可靠前向肺动脉血流的 SV 患儿中，SPS+PAB 是一种有效的一期姑息治疗方法。患有右位心的 SV 患儿死亡率较高。
