体-肺动脉分流术后早期分流失效的危险因素分析

目的分析影响体-肺动脉分流术后早期分流失效的危险因素,以提高手术效果。方法回顾性分析2002年2月至2010年12月期间沈阳军区总医院189例行体-肺动脉分流术患者的临床资料,其中男87例,女102例;年龄3个月～50(5.3±6.2)岁;体重3～56(17.7±11.0)kg。术前临床诊断为法洛四联症合并肺动脉狭窄94例,室间隔缺损合并肺动脉闭锁51例,室间隔完整的肺动脉闭锁4例,功能性单心室合并肺动脉狭窄10例,功能性单心室合并肺动脉闭锁6例,右心室双出口合并肺动脉狭窄6例,完全型大动脉转位合并肺动脉狭窄8例,完全型大动脉转位合并肺动脉闭锁10例。手术方式包括中央分流术105例,改良Blalock-Taussig分流术61例,墨尔本分流术23例。结果术后早期死亡13例(6.9%),术中发生严重低血压和心律失常12例(6.3%),术后严重低心排血量10例(5.3%),术后24 h内分流失效10例(5.3%)。单因素分析结果显示,低体重(P=0.027)、分流管直径<4 mm(P=0.025)、术中不良事件(严重低血压和心律失常,P=0.002)是24 h内分流失效的危险因素。多因素logistic逐步回归分析结果显示,术中不良事件(P=0.017)是24 h内分流失效的独立危险因素。结论对于年龄小、低体重和肺动脉发育细小的患者,术中预防严重低血压和心律失常,采用4 mm以上的分流管,可明显提高分流术后效果。     

体-肺动脉分流术治疗伴有室间隔缺损的肺动脉闭锁

目的讨论体-肺动脉分流术的手术方式,评价其治疗效果。方法自2003年9月至2005年5月对10例伴有室间隔缺损的肺动脉闭锁患者施行了体-肺动脉分流术,6例手术为右锁骨下动脉与右肺动脉吻合术,3例行升主动脉和右肺动脉人工血管连接术,1例3个月行中心分流术。结果10例手术均顺利完成,患者右上臂无缺血及运动障碍。末梢血氧饱和度由术前的(60±11)%上升至术后的(90±3)%。紫绀显著减轻,活动耐力明显增强,能从事正常的学习与生活。术后随访3~18个月,心功能Ⅰ级8例,Ⅱ级2例。McGoon指数由术前的0.91±0.15增至术后的1.15±0.20。NaKata指数由术前的(92±41)mm2/m2增至术后的(121±38)mm2/m2。结论体-肺动脉分流术是治疗伴有室间隔缺损且左右肺动脉发育较差的肺动脉闭锁的一种较好的姑息性手术。     

改良B-T分流术治疗复杂先天性心脏病的临床效果

目的分析改良B-T分流术治疗复杂先天性心脏病的临床效果。方法回顾性分析2006年7月至2013年1月上海市新华医院对143例复杂先天性心脏病患者行150例次改良B-T分流术的临床资料,其中男100例次、女50例次,手术年龄2~756(20.17±80.37)个月;体重4~63(8.86±9.69)kg。结果住院死亡5例(死亡率3.50%),3例患者因异常出血行开胸止血术(2.10%),5例(3.50%)因人造血管闭塞再次开胸行改良B-T分流术。该组患者129例(88.99%)获得随访,随访时间6~48(14.38±10.05)个月,随访期间6例患者因人造血管闭塞再次行改良B-T分流术7例次(5.43%),随访过程中死亡3例(死亡率2.33%),生存患者中88例再次行根治性手术或二期姑息性手术(68.22%)。行改良B-T分流术后,主肺动脉直径显著增大(t=-15.18,P=0.00),左肺动脉直径较术前有明显增大(t=-13.27,P=0.00),右肺动脉直径较术前明显增大(t=-15.94,P=0.00)。改良B-T分流术后,手术侧肺血管较对侧肺血管发育好(t=2.44,P=0.02),Mc Goon比值较术前有明显上升(t=10.10,P=0.00),射血分数值较术前稍有降低(t=2.77,P=0.00),左心室舒张期末容积指数较术前明显上升(t=-9.26,P=0.00)。结论改良B-T分流术治疗复杂先天性心脏病安全、有效,能显著促进双侧肺动脉发育,手术侧肺血管较对侧发育更好。术前Mc Goon比值小、肺动脉闭锁是限制肺动脉进一步发育的危险因素。根据患者术前肺动脉发育情况及体重选择合适的分流血管是保证手术成功及良好预后的基础。     

主动脉与人工血管侧侧吻合技术在体肺动脉分流术中的应用

目的 探讨主动脉与人工血管侧侧吻合技术在体肺动脉分流术中的应用.方法 2010年4月至2011年6月,44例紫绀型先天性心脏病患儿行主动脉与人工血管侧侧吻合的体肺动脉分流术,其中升主动脉-主肺动脉38例,升主动脉-左肺动脉4例,升主动脉-右肺动脉2例.男、女各22例;＜1岁23例,1～3岁14例,＞3岁7例;体质量(9.2±3.6) kg.肺动脉闭锁32例(3例室间隔完整,29例伴室间隔缺损),法洛四联症10例,右室双出口合并肺动脉狭窄和矫正性大动脉转位并肺动脉狭窄各1例.术后定期随访.结果 全组无手术死亡.2例出现早期狭窄,1例因早期分流堵塞再次行中心分流术,1例分流过多.术后经皮血氧饱和度由0.67±0.10增加至0.81±0.07.术后动脉压增加0～ 25mm Hg(1 mm Hg =0.133 kPa),中位值6.5 mm Hg.所有患儿随访12 ～ 23个月.2例出院后第1、3个月因误吸死亡;2例人工血管狭窄;2例人工血管闭塞,其中1例术后14个月再次行体肺动脉分流术;7例肺动脉发育良好者行进一步手术治疗,其中4例行根治手术,3例行Glenn手术.余患儿经皮血氧饱和度0.82±0.05,超声心动图示分流血管通畅,血流束与人工血管口径一致.结论 主动脉与人工血管侧侧吻合技术的体肺动脉分流术操作简单,安全性高,人工血管堵塞率低,可以推广应用.     

上腔静脉-右肺动脉分流术治疗三尖瓣闭锁

目的总结上腔静脉-右肺动脉分流术治疗三尖瓣闭锁(TA)的临床经验。方法2004年10月至2008年4月,采用上腔静脉-右肺动脉分流术治疗TA10例,男8例,女2例;年龄2～13岁,平均年龄5.6岁;体重10.0～33.5kg,平均体重16.4kg。TA合并大动脉错位(右位心)2例,房室间隔缺损3例,永存左上腔静脉1例。均在常温非体外循环下手术,切断上腔静脉,近端缝合,远端与右肺动脉做端侧吻合;1例合并永存左上腔静脉患者行双侧上腔静脉肺动脉吻合术。结果全组无死亡患者,术后末梢血氧饱和度由74%±9%上升至92%±5%。所有患者紫绀明显减轻,顺利出院。随访10例,分别随访6～36个月,血氧饱和度86%±3%,活动能力明显改善。结论上腔静脉-右肺动脉分流术治疗TA效果满意,是比较理想的术式。     

姑息性右心室肺动脉连接术在肺动脉闭锁合并室间隔缺损治疗中的应用

目的 评价姑息性右心室肺动脉外管道连接术(Sano分流术)在肺动脉闭锁合并室间隔缺损(PA/VSD)分期矫治术中的应用价值. 方法 2009年9月至2011年5月期间,共17例PA/VSD小儿患者在阜外心血管病医院接受Sano分流术,其中男10例、女7例,中位月龄9.7 (2.5～73.8)个月,体重(8.3±3.4)kg.术前McGoon比为1.04±0.29,Nakata指数为(102.0±56.9) mm2/m2;5例合并左肺动脉开口重度狭窄,11例存在动脉导管未闭(PDA),1例伴存主要体肺侧枝,术前经皮血氧饱和度为72.6％±11.6％. 结果 17例患者均顺利接受姑息性Sano分流术.术中结扎未闭动脉导管11例,同期左肺动脉成形7例.10例肺动脉下拉后覆盖自体心包片,1例利用自体心包制作管道,6例使用Gore-Tex管道构建右心室到肺动脉连接.术中均经右心室切口施行流出道疏通.体外循环时间为(75.0±30.0) min,术后经皮血氧饱和度为89.8％±5.3％,较术前显著改善(P＜0.05).所有患者均生存,随访(12.1±6.7)个月,随访期间8例接受二次心血管影像学检查,McGoon比值和Nakata指数分别为2.05±0.37和(304.8±51.3) mm2/m2,较术前显著提高(P＜0.05).其中4例已接受了二期根治术. 结论 Sano分流术是分期矫治PA/VSD较为安全且可有效促进肺血管发育的术式.     

减状手术对肺血减少型先天性心脏病患者体质及肺动脉发育的影响

目的探讨3种不同减状手术对肺血减少型先天性心脏病患者术后体质和肺动脉发育的影响。方法回顾性分析28例曾分别行腔静脉-肺动脉连接术(n=9)、体-肺动脉分流术(n=8)和姑息性右心室流出道重建术(n=11)的肺血减少型先天性心脏病患者的临床资料。所有患者此次入院与第1次入院的间隔时间为5~54个月(19.07±10.06个月)。观察所有患者减状手术前和二次手术前的红细胞压积(HCT)、血红蛋白(Hb)、脉搏血氧饱和度(SpO2)、体表面积(BSA)和肺动脉指数(PA I)等指标。结果第2次入院后共死亡7例,死亡原因为出血、循环衰竭和体外循环意外等。第1次行姑息性右心室流出道重建术患者二次手术后呼吸机辅助时间、ICU停留时间及多巴胺总量均长于或大于行腔静脉-肺动脉连接术患者(P<0.05)。所有患者二次手术前HCT、Hb较减状手术前降低,而SpO2、BSA和PA I增高(P<0.01)。第1次行腔静脉-肺动脉连接术患者BSA、体-肺动脉分流术患者BSA和PA I、姑息性右心室流出道重建术患者SpO2、BSA和PA I二次手术前均较减状手术前增高(P<0.01),姑息性右心室流出道重建术患者HCT较减状手术前降低(P<0.05)。结论体-肺动脉分流术、姑息性右心室流出道重建术均能有效地改善肺血减少型先天性心脏病患者的肺血流量,促进左右肺动脉的发育,而腔静脉-肺动脉连接术患者未发现此种改变。     

用人造血管作改良Blalock-Taussig分流术的经验分析

传统的Ｂｌａｌｏｃｋ－Ｔａｕｓｉｇ分流术即将锁骨下动脉和肺动脉吻合，在临床上较其它方式的分流术效果更优，但这种分流术对锁骨下动脉给同侧上肢供血极为不利。因此，利用膨体聚四氟乙烯人造血管作改良的Ｂｌａｌｏｃｋ－Ｔａｕｓｓｉｇ分流术，可保留锁骨下动脉，并...     

婴儿颈内静脉置管后上腔静脉血栓栓塞一例

患儿,男,5个月,体重7.0 kg,身高68cm,因先天性心脏病(室隔完整的肺动脉闭锁、房间隔缺损、动脉导管未闭)在全麻体外循环下行右侧双向上腔静脉与右肺动脉吻合分流术、右室流出道补片扩大和动脉导管结扎术,手术顺利.     

体肺分流术在治疗肺血减少型先天性心脏病中的应用评价

目的分析体肺分流术在肺血减少型先天性心脏病治疗中的应用效果。方法对2005年4月至2009年2月我院20例肺血减少型心脏病患者行体肺分流手术,其中Waterston术（升主动脉吻合至右肺动脉）12例,中心分流术8例。结果围术期死亡1例,术后半年心功能衰竭1例,余病例元远期死亡。结论对肺动脉发育不良的患者,体肺分流术可减轻患者的缺氧症状,改善患者的心功能,促进肺血管的发育,并为进一步的手术创造条件。     

The Blalock-Taussig Shunt

Abstract   Background and aim of the study: Even though the Blalock-Taussig (B-T) shunt, either classic or modified, has been advocated and successfully employed in clinical practice for more than half a century, a systemic review on this procedure is still scanty. This warrants us a zest in making a comprehensive survey on this subject. Methods: Articles were extensively retrieved from the MEDLINE database of National Library of Medicine USA if the abstract contained information relevant to the B-T shunt in terms of the conduit options, modified surgical techniques, surgical indications, short- and long-term results, complications, and prognosis. Further retrieval was undertaken by manually searching the reference list of relevant papers. Results: Classical or modified B-T shunts, either on ipsilateral or contralateral side to the aortic arch, can be performed on patients of any age with minimum postoperative complications and low operative mortality. Expended polytetrafluoroethylene has gained satisfactory long-term patency rate in the construction of the modified B-T shunt. Excellent pulmonary artery growth was observed in the patients with a modified B-T shunt, and it has shown superb prognosis over the classic with regard to hemodynamics, patency rate, and survival. Conclusions: The modified B-T shunt that was developed on basis of the classic fashion remains the preferable palliative procedure aiming at enhancing pulmonary blood flow for neonates and infants with complicated cyanotic congenital heart defects. The modified B-T shunt is technically simpler with less dissection, and blood flow to the respective arm is not jeopardized. It has been proved to be of low risk, excellent palliation, and is associated with excellent pulmonary artery growth, has become the most effective palliative shunt procedure of today.     

杂交技术治疗室间隔完整型肺动脉闭锁

目的 总结杂交技术经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉闭锁的即刻疗效及近、中期随访结果.方法 2005年3月至2010年3月,采用超声引导经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉膜性闭锁30例,年龄1天～48个月,平均(4.59±3.21)个月.胸骨正中切口,于右室流出道距离肺动脉瓣环下约2 cm缝荷包线,然后置入导丝.在超声引导下置入穿刺鞘管.确认穿刺针对准膜性闭锁的瓣膜后,在钢丝引导下放入球囊扩张管进行扩张,超声提示肺动脉瓣开放满意.＜3个月病婴行改良Blalock-Taussig(B-T)体肺分流术,并同期行动脉导管结扎术.＞3个月病婴行球囊扩张术后,如血氧饱和度改善明显,不常规行改良B-T分流术,并保留动脉导管开放,如血氧饱和度改善不明显,则考虑行改良B-T分流术,结扎或保留动脉导管.＞5个月病儿行球囊扩张后血氧饱和度改善不满意,且重度右心发育不良,则选择双向Glenn术.结果 30例行球囊扩张均取得成功,同期行动脉导管结扎术25例,改良B-T分流术8例,双向Glenn术2例.均未出现严重并发症.1例术后因低氧血症,术后第3天行动脉导管结扎术和改良B-T分流术;余者术后血流动力学稳定,顺利出院.术后随访1.5～62.0个月,平均(18.7±17.2)个月.血氧饱和度由术前0.73±0.08上升至0.94±0.04,心功能Ⅰ级.院外死亡5例,25例生长发育良好.结论 杂交技术经胸肺动脉瓣球囊扩张成形术是一种治疗新生儿及婴幼儿室间隔完整型肺动脉膜性闭锁的安全、有效的方法.

Abstract：

Objective In patients with pulmonary atresia and intact ventricular septum ( PAIVS) without right ventricular-dependent coronaries, catheter techniques including the use of a sniff wire, lasers, and radiofrequency have been the most widely used initial therapy. However, percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and serious complications. Methods We report our experience with a hybrid approach for pulmonary atresia with intact ventricular septum, combining surgery and interventional catheterization techniques. Between March 2005 and March 2010, hybrid procedure was carried out successfully in 30 newboms and infants with favorable anatomy. The age ranged from 1 day to 48 months with a mean of (4.59 ±3.21) months. The heart was exposed through median sternotomy. A pursestring suture was placed in the right ventricular outflow tract 2 cm away from the pulmonary trunk. Then a 16-gauge intravenous catheter was punctured through the right ventrical and perforated the atretic PV with the guidance of echocardiography. A guide wire was then inserted into the sheath and used to guide the balloon across the PV. Sequential dilations were performed until a full opening of the PV with the guidance of epicardial echocardiography. In patients ＜ 3 months PDA ligation was performed followed by modified Blalock-Taussig (B-T) shunt. In patients ＞ 3 months PDA ligation was not performed. A modified B-T shunt was inserted if severe systemic oxygen desaturation occurred after PDA ligation. Bidirectional Glenn shunt was performed for severe hypoplasia. Hybrid procedure was achieved in all patients. The simultaneous procedures included 25 cases of PDA ligation. 6 newborns underwent modified B-T shunt placement (3.5 to 5 mm) after pulmonary valvuloplasty and PDA ligation, and 2 patients ＞ 1 month underwent modified B-T shunt. Another 2 patients were selected for univentricular palliative surgery because of a diminutive monopartite right ventricle and bidirectional Glenn procedure was performed. No pericardial effusion or cardiac tamponade was observed in all patients. Another case without PDA ligation underwent a modified B-T shunt because of hypoxemia three days after hybrid procedure, and the rest patients were discharged without any further surgical intervention.During the follow-up period of 1.5 to 62.0 months, 5 patients died. 25 (83.3%) survived and were all in New York Heart Association functional class 1. Peripheral oxygen saturation increased from 0.73 ± 0.08 to 0.94 ± 0.04 (P ＜ 0.05). One patient remains in a single-ventricle pathway, whereas 24 patients achieved a two-ventricle circulation. Results Conclusion Perventricular balloon pulmonary valvuloplasty using a hybrid approach is a safe and feasible procedure for patients with PAIVS.     

Pulmonary vascular disease in shunted and nonshunted patients with tetralogy of Fallot

Histometric analysis of pulmonary vascular disease was performed in 21 nonshunted patients and in 13 shunted patients with tetralogy of Fallot and in 29 normal controls. There was no significant difference in the medial thickness of the small pulmonary arteries between cases of tetralogy of Fallot and normal controls. However, the media in the shunted cases of tetralogy of Fallot gave the impression of being thicker than these in the nonshunted cases. Intimal fibrosis, regarded as organized thrombi, and thrombi of small pulmonary arteries were observed generally after 4 years of age in shunted and nonshunted cases of tetralogy of Fallot. Intimal proliferation of musculoelastosis which was formed longitudinal smooth muscle bundles and elastic fibers was characteristic in shunted patients, especially after the central palliation procedure, Waterston anastomosis and modified Blalock-Taussig (B-T) anastomosis using a Gore-Tex tube graft. However, musculoelastosis was not usually seen in the B-T anastomosis cases. Unexpected pulmonary hypertension due to shunt operation is thought to be the cause of musculoelastosis, because musculoelastosis was observed even in a patient with pulmonary hypertension 3 weeks following surgery. We, therefore, recommend original B-T anastomosis as the shunt procedure. When considering use of the other shunt operations which entail pressure load on the pulmonary vascular bed, attention must be given to the size of the anastomosis or artificial tube graft.     

姑息性右心室-肺动脉连接术中管道材料及型号的选择与应用

目的 评价不同管道材料（Gore-Tex人工血管、牛颈静脉和自体心包管道）在姑息性右心室-肺动脉连接术中的应用效果,探讨患者适用的管道型号与体重、McGoon比之间的相关性。 方法 回顾性分析 2010年7月至2012年7月北京阜外心血管病医院小儿外科中心24例先天性心脏病患者接受姑息性右心室-肺动脉连接术治疗的临床资料,其中男11例,女13例;年龄60 d至6岁;体重（10.22±7.41） kg。肺动脉闭锁合并室间隔缺损（PAVSD） 22例,法洛四联症（TOF） 1例,右心室双出口（DORV） 1例。使用的外管道材料分别为自体心包17例,Gore-Tex人工血管5例,牛颈静脉2例。对重建管道的直径与患儿体重进行线性回归分析,以McGoon比为参数对公式再作矫正。 结果 无围术期死亡,术后全组患儿血氧饱和度较术前增加20.37%±28.33%。心电图均显示窦性心律,心功能（NYHA） Ⅱ级23例,Ⅲ级1例。采用自体心包手术患儿术后呼吸机使用时间短于其它两种材料（P=0.017）。术后随访16例,随访时间10个月～2年。其中采用自体心包手术患儿完成随访12例,采用Gore-Tex人工血管手术3例,采用牛颈静脉手术1例。随访期间采用自体心包手术患儿McGoon比为1.98±0.46,Gore-Tex人工血管手术为1.83±0.33,牛颈静脉手术为1.68,均较术前有明显增加（P＜0.05）。完成根治6例（其中采用自体心包手术患儿完成根治手术5例,采用Gore-Tex人工血管手术患儿完成根治手术1例）。随访过程中未发现明显与手术相关的并发症发生。通过线性回归分析,得出合适的管道型号与患者体重关系的公式：管道直径（mm） =0.327×体重（kg）＋4.599。通过McGoon比、管道型号及公式计算值的对比分析发现：McGoon比＜0.8,可取大于计算值的第一个整数;McGoon比＞1.2,则可取小于计算值的第一个整数。0.8＜McGoon比＜1.2,则前后两个整数均可。病例分组分析发现,术后恢复良好者更符合经公式计算及McGoon矫正后的值。 结论 三种材料均可作为重建右心室-肺动脉连接管道的常规材料;右心室-肺动脉连接术中可以根据患者的体重及McGoon比选择合适的外管道型号。     

肺动脉闭锁伴室间隔缺损患者姑息手术后血气与血流动力学指标的改变

目的比较不伴主、肺动脉侧枝（MAPCAs）的肺动脉闭锁伴室间隔缺损（PA／VSD）患者接受右心室至肺动脉（RV—PA）管道连接或改良体-肺动脉分流术（mBT）术后血气及血流动力学指标改变,以明确两种术式对氧供需平衡的影响。方法2006年7月至2007年10月,对38例不伴MAPCAs的PA／VSD患者根据手术方式不同分为两组：RV—PA组（n=25）和mBT组（n=13）。比较围术期死亡率,术后48h内的血气及血流动力学指标,包括心率、血压、动脉血氧饱和度、混合静脉血氧饱和度、氧剩余参数和正性肌力药物评分等的改变。结果术后RV—PA组死亡率（4．0％）与mBT组（7．7％）比较差异无统计学意义（P〉0．05）。随访33例,随访时间6～18个月。11例患者（其中mBT组4例、RVPA组7例）在术后9～18个月接受了根治手术治疗,术后死亡1例,死于肺血管阻力过高,右心衰竭。术后24h、48h时RV—PA组和mBT组混合静脉血氧饱和度均分别高于本组术后6h（P〈0．01）,术后6h、24h和48hRV—PA组收缩压均低于mBT组（P=0．048,0．043,0．045）;平均动脉压均高于mBT组（P=0．048,0．046,0．049）;舒张压均高于mBT组（P=0．038,0．034,0．040）;正性肌力药物评分均低于mBT组（P=0．035,0．032,0．047）。结论RV—PA管道连接与mBT两种姑息手术,虽然术后血压和正性肌力药物评分有显著差异,但体循环氧输送基本相当,术后6h两组患者血流动力学状态达最低水平。     

Hemodynamic status after the Norwood procedure: a comparison of right ventricle-to-pulmonary artery connection versus modified Blalock-Taussig shunt

Background

The aim of this study is to compare hemodynamic status, in particular systemic oxygen delivery, in patients undergoing a Norwood procedure with a right ventricle-to-pulmonary artery (RV-PA) versus a modified Blalock-Taussig (mBT) shunt.

Methods

From June 2000 to November 2003, 44 consecutive neonates with hypoplastic left heart syndrome underwent a Norwood procedure. The first 25 patients received an mBT shunt; the subsequent 19 an RV-PA shunt. Hemodynamic data, including mixed venous oxygen saturation, was determined during the first 48 hours after surgery.

Results

The mBT and RV-PA shunt patients had no significant differences in systemic oxygen saturation, mixed venous oxygen saturation, arteriovenous oxygen saturation difference, or oxygen excess factor during the first 48 hours. Mixed venous saturation declined to a nadir in both groups at 6 to 12 hours. The RV-PA patients had significantly higher diastolic and mean blood pressures, and lower systolic blood pressure. Mean heart rate, common atrial pressure, and inotrope score did not differ between the two groups. The RV-PA patients received higher fraction of inspired oxygen and minute ventilation to achieve partial pressures of arterial oxygen and carbon dioxide, and pH, similar to mBT patients. Durations of mechanical ventilation, intensive care unit stay, and hospital stay did not differ between mBT and RV-PA patients. Operative survival in the mBT versus RV-PA group was 20 of 25 (80%) versus 17 of 19 (89%; p = 0.7).

Conclusions

Indicators of postoperative systemic oxygen delivery are equivalent in neonates who have undergone a Norwood procedure with an mBT or RV-PA shunt. Both mBT and RV-PA patients undergo similar declines in hemodynamic status 6 to 12 hours after surgery. Any advantages of one approach over the other lie in areas other than systemic oxygen delivery, such as resistance to physiologic insults, or preservation of ventricular function.     

肺动脉发育不良型法洛四联症根治术前姑息手术和介入技术的应用

目的 总结姑息手术和介入技术治疗合并肺动脉发育不良的重症法洛四联症的临床经验.方法 2002年12月至2009年12月,1586例患儿行法洛四联症根治术中18例(男12例、女6例)在根治手术前进行了姑息手术和介入技术相结合的复合治疗.合并心血管畸形包括:房间隔缺损3例,左肺动脉缺如2例,左肺动脉起自主动脉1例,永存左上腔静脉1例,合并粗大体肺侧支血管7例.根治手术前行一次姑息手术者13例,两次者4例,三次者1例.手术术式包括改良Blalock-Taussig分流术14例次,Waterston分流术4例次,右室流出道重建术3例次,肺动脉瓣球囊扩张术3例次,肺动脉环缩1例,行侧支血管结扎6例(16支),侧支血管融合1例(2支),侧支血管介入封堵2例(6支).结果 全组无死亡,1例因人工血管堵塞在术后第1天再次行体肺分流术,患儿根治手术前Nakata指数和McGoon比值[(200±81)和(1.77±0.51)]均较姑息手术前[(84±40)和(1.14±0.33)]有明显增加(P＜0.001),末梢血氧饱和度和血红蛋白浓度[(0.71±0.09)和(175±46) g/L]均显著改善[(0.86±0.05)和(149±15) g/L,P＜0.05].所有18例患儿均完成了最终的根治手术.结论 采用姑息手术和介入技术相结合的复合治疗措施能有效改善肺动脉发育,为合并肺动脉发育不良的重症法洛四联症根治手术创造条件.     

Postoperative course after first-stage palliation of hypoplastic left heart syndrome with right ventricle-to-pulmonary artery shunt; compared with classic Norwood procedure

BACKGROUND: A recent modification of the Norwood procedure involves the use of a right ventricle-to-pulmonary artery (RV-PA) shunt to provide pulmonary blood flow for patients with hypoplastic left heart syndrome (HLHS). We investigated the hemodynamics after first-stage palliation of HLHS with RV-PA shunt compared with classic Norwood procedure with subclavian-to-pulmonary artery (BT) shunt. METHODS: The postoperative course of 12 infants who had undergone first-stage palliation for HLHS using BT shunt (group BT: n=6) and RV-PA shunt (group RV-PA: n=6) were retrospectively reviewed and we obtained the following data: blood pressure, heart rate, inotropic support, atrial pressure, lactate, base excess, PaO2, FIO2. RESULTS: The RV-PA shunt using a non-valved conduit provided higher diastolic blood pressure than the BT shunt, but no significant difference in heart rate, systemic blood pressure, inotropic support and atrial pressure was observed between the two groups. Although the infants in the group RV-PA required significantly more myocardial ischemic time for operative procedure than those in the group BT, the serum lactate level in the group RV-PA was significantly lower than those in the group BT. CONCLUSIONS: These results show that the RV-PA shunt provides a stable systemic circulation and abundant tissue oxygen supply. Excellent hemodynamics provided by RV-PA shunt is beneficial for infants undergoing stage I palliation for HLHS.     

Right ventricular aneurysm following modified Norwood-Sano operation for hypoplastic left heart syndrome

Hypoplastic left heart syndrome is a rare congenital heart defect characterized by underdevelopment of left-sided heart structures, including the aortic arch. The contemporary surgical management of this anomaly includes the Norwood procedure and provision of pulmonary blood flow by either a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery (RV-PA) conduit, commonly referred to as the Sano shunt. We report on an unusual complication of the Sano shunt, that of a giant right ventricular pseudoaneurysm occurring at the shunt insertion site.     

Impact of right ventricle to pulmonary artery conduit on outcome of the modified Norwood procedure

BACKGROUND: To determine and compare outcome of the modified Norwood procedure using either a systemic to pulmonary artery (SPA) shunt or right ventricle to pulmonary artery (RV-PA) conduit in a consecutive series of neonates at a single institution. METHODS: The medical records were retrospectively examined for preoperative demographic and echocardiographic data, operative variables, and postoperative clinical and hemodynamic data. From November 2001 to March 2003, 21 neonates had a modified Norwood procedure (SPA shunt, n = 8; RV-PA conduit, n = 13) at a median age of 5 days (range 1 to 18 days) and a median weight of 2.9 kg (range 1.7 to 4.1 kg). Of the 21 infants, 12 were considered high risk due to presence of low birth weight (n = 4), extracardiac or genetic anomalies (n = 5) or obstruction to pulmonary venous return (n = 5). Nine "high risk" infants were in the RV-PA conduit group. RESULTS: Overall Norwood operation survival was 90% (19/21) and did not differ between groups. There were 2/19 interstage deaths and Kaplan-Meier survival at 1 year is 79%. Neonates in the RV-PA conduit group had significantly higher diastolic blood pressures at 1, 6, and 24 hours postoperatively (p < 0.05). Neonates in the SPA shunt group had significantly higher heart rates at 1 hour postoperatively (p < 0.05) than those in the RV-PA group. There was a trend to higher number of ventilatory interventions to balance Qp:Qs in the SPA shunt group (p = 0.06). CONCLUSIONS: In a relatively high-risk group, neonates having an RV-PA conduit as part of the Norwood procedure have favorable postoperative hemodynamics and a good likelihood of stage I survival.