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.     

复杂先天性心脏病的外科治疗

近15年来，本所手术治疗治疗法乐氏四联症、右室双出口、Ebstein心脏病畸形、单心室和三尖瓣闭锁、矫正型大动脉转位合并心内畸形、完全性肺静异位引流、主动脉狭窄、大动脉转位合并室缺和肺动脉狭窄以及室间隔完整的肺动脉闭锁等1880例，总的手术死亡率为4．05，本文重点介绍上述复杂先天性心脏病的外科治疗经验。     

心脏外科手术与介入性心导管术镶嵌治疗小儿先天性心脏病

目的 报道23例介入性心导管术与心脏外科手术镶嵌治疗小儿先天性心脏病(CHD)的经验。方法外科术后镶嵌治疗：12例动脉导管未闭结扎术后残余分流及2例心脏术后留置室间隔缺损(VSD)的复杂CHD行经导管封堵术。2例法洛四联症(TOF)根治术后分支肺动脉狭窄分别行球囊血管成形术及支架置入术，1例肺动脉闭锁合并VSD根治术后出现体肺侧支血管(APCAs)破裂行介入栓塞止血术。外科术前介入治疗：5例室间隔完整的大血管转位(TCA)病例行大动脉调转术前行球囊房隔造口术(BAS)，1例有巨大APCAs的重症。TOF在根治术前予以侧支血管堵塞术。结果动脉导管未闭术后残余分流及复杂CHD残留VSD病例均封堵成功，分支肺动脉狭窄病例介入治疗后狭窄减轻或治愈；侧支血管破裂病例栓塞止血后出血停止。BAS病例介入术后低氧及酸中毒改善，大动脉调转术后3例治愈，2例死亡。TOF病例APCAs堵塞后成功行根治术。结论心脏外科手术与介入性心导管术镶嵌治疗是治疗小儿CHD术后残余分流及部分复杂疑难CHD的有效方法。     

同种主动脉治疗复杂先天性心脏病

目的　总结用同种带瓣主动脉和同种主动脉治疗 11例复杂先天性心脏病的经验。　方法　对 11例法洛四联症合并肺动脉闭锁和动脉导管未闭 ,矫正型大动脉转位合并室间隔缺损和肺动脉狭窄 ,完全型大动脉转位合并室间隔缺损和肺动脉狭窄 ,右心室双出口合并完全性房室通道 , 型永存动脉干 ,主动脉弓中断合并动脉导管未闭 ,主动脉缩窄合并动脉导管未闭和二尖瓣关闭不全患者施行了手术治疗 ;其中 Rastelli手术 7例 ,全腔静脉与肺动脉连接 (TCPC) 2例 ,升主动脉至降主动脉旁路移植 2例。　结果　手术死亡 1例 ;慢性心力衰竭 1例 ,内科治疗 1年后心功能 级。随访 1个月～ 7.2年 ,无远期死亡。　结论　同种主动脉是矫正复杂先天性心脏病的理想材料     

完全型大动脉转位行大动脉调转术十年(2001～2012年)的变化趋势——单中心报告

目的考察完全型大动脉转位行大动脉调转术术前因素以及住院死亡率10年的变化趋势。方法回顾性分析阜外医院2001~2012年连续473例完全型大动脉转位行大动脉调转术患者的临床资料。按照手术时间先后顺序,将所有患者分为6组(每2年1组),首先使用趋势分析的方法,对这些患者的术前因素及住院死亡率的变化趋势进行了分析;然后通过危险因素调整的方法,再次分析了住院死亡率的变化趋势。结果全组共有29例住院死亡,总体住院死亡率6.1%。所有术前因素包括年龄(P=0.13)、性别(P=0.94)、身高(P=0.29)、体重(P=0.21)、合并肺动脉高压(P=0.59)、行左心室训练(P=0.14)、冠状动脉异常分型(P=0.27)及合并非限制性室间隔缺损(P=0.14)未表现出显著的变化趋势。未经调整的住院死亡率在这10年间明显下降[17.4%(第1组)vs.4.1%(第6组),P0.001]。经过术前因素调整后,住院死亡率亦表现出了明显下降(以第1组为参照,第6组的比值比:0.098,95%可信区间0.018-0.550,P=0.01)。结论在2001~2012年间,在术前因素没有显著变化的情况下,完全型大动脉转位行大动脉调转术的住院死亡率显著下降。     

Rastelli手术治疗大动脉转位伴室间隔缺损

目的介绍Rastelli手术治疗大动脉错位伴室间隔缺损的经验.方法全组49例中男29例,女20例.平均年龄5.6岁;平均体重17.2kg.完全性大动脉转位31例,纠正性大动脉转位18例;伴肺动脉狭窄45例,伴肺动脉高压4例.均在低温体外循环下行Rastelli手术.二期根治5例.体外循环灌注(178.5±52.5)min;主动脉阻断(109.2±38.3) min.结果手术早期死亡6例,死亡率12.2%.死因为肺动脉高压危象、肾衰、心律紊乱和严重低心输出量综合征.术后并发症有心律紊乱、肺动脉高压危象、蛛网膜下腔出血、脑功能紊乱、肾衰及多脏器功能衰竭、心包或胸腔积液、感染等.CICU平均监护7.3 d.随访中因同种带瓣大动脉(VHC)感染死亡1例.结论完全性大动脉转位手术中,右室流出道直切口有利于心内隧道修补室间隔缺损;纠正性大动脉转位手术中,解剖右室径路显露缺损较好且易避开传导系统,但不利于术后心功能恢复.而解剖左室径路修补室间隔缺损的房室传导阻滞发生率高;大于4岁者手术宜选择大号VHC可减少再次手术几率;对无长段左室流出道狭窄的完全性大动脉转位病婴,可在动脉换位术基础上行肺动脉瓣叶交界切开或Konno术以解除左室流出道梗阻.为防止术后功能性二尖瓣反流,对伴肺动脉狭窄的纠正性大动脉转位病儿,提倡心房-大动脉双换位手术.     

169例动脉调转手术治疗大动脉转位的早、中期结果

目的 探讨大动脉调转手术(ASO)治疗完全性大动脉转位(TGA)的早、中期结果,分析其随访死亡、术后主动脉瓣反流及肺动脉狭窄的危险因素.方法 2004年1月至2007年12月,169例行动脉调转术病儿入选,其中男129例,女40例,平均年龄(11.7±26.3)个月.病儿分两组:Ⅰ组为室间隔完整组(56例),Ⅱ组为室间隔缺损组(113例).所有术后生存病儿均进行超声随访,平均随访时间(27.7±14.6)个月.危险因素采用Logistic回归模型分析.结果 全组住院死亡19例(11.24%),两组间差异无统计学意义.随着整体治疗水平的提高,住院病死率由2004年的16.67%下降到2007年的3.92%.1、3及5年生存率分别为94.00%、91.33%及91.33%,两组间差异无统计学意义.Logistic回归分析发现,ASO术后随访死亡的危险因素为手术年龄大于6个月;术后主动脉瓣反流的危险因素为合并室间隔缺损、年龄大于6个月、术后新主动脉瓣Z值>1;术后肺动脉狭窄的危险因素为手术年龄小于1个月和肺动脉采用补片成形.结论 ASO手术具有良好的早、中期结果,是治疗完全性大动脉转位的理想术式.TGA病儿应该尽早手术治疗,手术年龄大于6个月是随访死亡及主动脉瓣反流的重要危险因素;新主动脉根部与主动脉远端不匹配是术后主动脉瓣反流的危险因素;病儿的生长发育与肺动脉成形材料无生长特性的矛盾是导致术后肺动脉狭窄的危险因素.     

改良Fontan手术后脑部并发症相关因素分析

我们对 1984年至 1997年间 ,12 0例行改良Ｆｏｎｔａｎ手术后病人进行多因素分析 ,并重点分析了其中 19例术后发生脑部并发症的相关因素。现报告如下。资料与方法　本组 12 0例中男 82例 ,女 38例 ;年龄 2～34岁 ,平均 (10± 6 )岁 ;体重 10～ 6 9ｋｇ ,平均 (2 7± 14)ｋｇ。单心室 6 0例 ,三尖瓣闭锁 31例 ,右心室双出口 12例 ,二尖瓣闭锁 7例 ,完全性大动脉转位 6例 ,矫正性大动脉转位、孤立性右心室发育不全、Ｅｂｓｔｅｉｎ心脏畸形、室间隔完整的肺动脉闭锁各 1例。术前均作心血管造影检查 ,测量ＭｃＧｏｏｎ比值及肺动脉指数 (ＰＡＩ…     

肺动脉融合术治疗肺动脉闭锁合并大主肺侧支动脉

目的 总结肺动脉融合术治疗肺动脉闭锁、室间隔缺损合并大主肺侧支动脉的初步经验.方法 1999年12月至2007年6月完成肺动脉闭锁,室间隔缺损合并大主肺侧支动脉(PA/VSD/NAPCAs)的肺动脉融合术17例,其中男7例,女10例;年龄0.8～18岁,平均(6.0±5.6)岁;体重6.5～55.0 kg,平均(20.0±14.9)kg.术前固有肺动脉指数(PAJ)为42.0～366.1mm2/m2,平均(133.7±87.8)mm2/m2.手术入路包括正中剖胸10例,正中+左后外侧剖胸5例,正中+右后外侧剖胸2例.一期肺动脉融合及心内畸形矫治术12例,肺动脉融合及姑息性体肺分流术4例,肺动脉融合及姑息性右室流出道扩大1例.全组病儿术前造影发现大主肺侧支动脉共44支;术中融合29支,结扎5支.肺动脉闭锁矫治术及肺动脉融合术前介入栓堵3个侧支1例;肺动脉融合及体肺分流术后介入栓堵2个残留侧支1例.结果 全组术中转流174.3min,主动脉阻断88.7min.术后呼吸机辅助7.4d,术后ICU 11.8d.生存11例,平均新建肺动脉指数(TNPAI)249mm2/m2.6例死亡,其中2例TNPAI<200mm2/m2,死于肺血管发育不良,术后发生低心排出量综合征(低心排)和突发室性心律失常;1例因低心排无法脱离体外循环,尸检发现肺小动脉明显肌型化,管腔高度狭窄,证实术前局部肺段已有严重的肺高压;3例>450 mm2/m2分别死于严重肺部感染、渗出、急性肾功能衰竭,反复气道大量出血和顽固性室性心律失常.其他主要并发症为大脑皮层盲、膈肌麻痹和阴沟杆菌性肺炎肺出血各1例.结论 完全矫治+肺动脉融合是治疗肺动脉闭锁/室间隔缺损合并大主肺侧支动脉最根本的治疗手段,但手术操作复杂.由于同一病儿肺动脉病变的多样性,常见MAPCAs高压及狭窄并存.适应证选择和手术技巧的提高仍需不断的探索.     

Double Switch术治疗先天性矫正性大动脉转位二例

目的 介绍解剖矫治先天性矫正性大动脉转位的手术特点。方法 2例矫正性大动脉转位患者,1例9个月,合并室间隔缺损、肺动脉高压,行Senning加动脉调转术;1例23岁,合并室间隔缺损、肺动脉狭窄、三尖瓣关闭不全,行Rastelli加Senning术。结果 2例患者全部成活,其中1例术前出现Ⅲ度房室传导阻滞,安装永久起搏器,术后超声检查心室功能正常,心内隧道和心外管道通畅。无三尖瓣反流。结论 Double Switch手术是一种理想的治疗先天性矫正性大动脉转位的方法。     

Surgical management of critical pulmonary stenosis in the neonate

Experience with 36 consecutive neonates less than 1 month of age with critical pulmonary stenosis (PS) with intact ventricular septum was analyzed to define the role of pulmonary valvotomy and of a concomitant systemic-pulmonary shunt as well as the impact of prostaglandin E1 (PGE1) therapy in the management of this disorder. Operative procedures included pulmonary valvotomy as an isolated procedure (Group 1, N = 22), pulmonary valvotomy plus a systemic-pulmonary shunt (Group 2, N = 8), pulmonary valvotomy with PGE1 therapy (Group 3, N = 5), and one miscellaneous procedure. The hospital mortality (+/- 70% confidence limits [CL]) by treatment group was as follows: Group 1, 54% (CL, 41-67%) (12/22); Group 2, 25% (CL, 9-50%) (2/8); and Group 3, 0 (CL, 0-32%); Group 1 versus Group 2 plus Group 3 (p less than or equal to 0.05). Patients managed with a shunt or perioperative administration of PGE1 experienced a significant improvement in early survival. Late postoperative angiography demonstrated exemplary right ventricular growth in the majority of patients, although important residual abnormalities of the outflow tract necessitating operative repair were frequently present (5-year and 10-year actuarial freedom from reoperation, 73 +/- 10% and 42 +/- 16%, respectively [+/- standard error of the mean]). This review illustrates the limitations of pulmonary valvotomy as an isolated therapeutic method in neonates with critical PS. Provision of a systemic extracardiac source of pulmonary blood flow, accomplished by a systemic-pulmonary shunt or PGE1 infusion continued postoperatively, is the most important determinant of early survival in this disorder.     

Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome

Objective. Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy. Methods. Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status. Results. Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 ± 2.6 mmHg, cardiac index of 3.6 ± 0.8 l/kg/min, and PA index of 194.0 ± 58.4 mm²/m²; there was no difference between the groups. Conclusion. The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity. Presented at the 59th Annual Scientific Meeting of the Japanese Association for Thoracic Surgery, held in Tokyo, Japan, October 1–4, 2006     

Surgical intervention in neonates with critical pulmonary stenosis.

The surgical experience with 18 consecutive neonates with critical pulmonary stenosis (PS) and intact ventricular septum was reviewed. All patients had cardiac catheterization with calculation of right ventricular volume. Group A patients (N = 8) had a small right ventricular end-diastolic volume (RVEDV less than 72% of predicted). Group B patients (N = 10) had a normal or enlarged RVEDV. All patients had a closed pulmonary valvotomy. Five Group A patients required a systemic-pulmonary shunt or prostaglandin (PGE1) after operation; one patient died. Nine Group B patients did well after valvotomy; one moribund patient died after valvotomy and shunt. Six of 16 survivors required reoperation: valvectomy in four patients and shunt takedown in two patients. Four of the six patients who had reoperation also had a transannular patch. There was one unrelated late death. All long-term survivors are asymptomatic. Recatheterization in four patients with a small right ventricle (RV) documented significant RV growth. In conclusion, most neonates with critical PS can be managed with closed valvotomy. Patients with a small RV may require PGE1 or a shunt after operation for persistent hypoxemia. Some patients with a small RV will have significant RV growth after valvotomy.     

室间隔完整型肺动脉闭锁的右心室减压策略

目的 总结室间隔完整型肺动脉闭锁(PA/IVS)右心室减压的经验与教训,反思右心室减压策略.方法 回顾性分析2015年3月至2019年12月于我院行右心室减压手术的12例PA/IVS患儿的临床资料,其中男10例、女2例,手术时中位年龄5(1～627)d.对减压后肺动脉瓣跨瓣压差与三尖瓣Z值变化进行相关性分析.结果 术后...     

Palliative surgery for cyanotic congenital heart disease

Recently, there has been an increasing emphasis on definitive correction of congenital heart defects at an early age. Nevertheless, in the presence of severe cyanotic congenital heart disease, survival may depend on palliative techniques designed either (1) to promote increased pulmonary blood flow by means of a systemic-pulmonary artery shunt or (2) to promote mixing of blood by means of a left-to-right shunt. For some patients with other cases, palliative techniques serve to "buy time" until the patient attains a more desirable age and body size. Whether subsequent definitive repair is performed, the above-described procedures allow the salvage of numerous patients whose cyanotic anomalies would otherwise be incompatible with life.     

自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣狭窄

目的探讨解剖纠治完全性大血管错位（TGA）伴肺动脉瓣狭窄的手术方法。方法1例8月龄、体重6．5kg的TGA伴肺动脉瓣狭窄男病婴，术中将原主动脉瓣移植至肺动脉瓣处形成新的主动脉；再行Switch术，同种带瓣管道连接右心室与肺动脉。另1例10月龄、体重9．8kg的该病男病婴，术中将整个主动脉瓣取下，保留左、右冠状动脉，向后移植，后半部分直接与原肺动脉瓣环连续缝合，前半部分与室间隔缺损之间采用dacron补片连续缝合关闭；肺动脉与右心室切口直接连接。结果2例手术均取得成功。出院时超声检查各吻合口通畅，无残余分流．无主动脉瓣反流。术后随访12个月和6个月，生长发育良好，心胸比率0．75。超声示左心功能良好，前例示主动脉瓣轻度反流．后例左、右心室流出道无残余梗阻．结论采用自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣和瓣下狭窄，从解剖上得到彻底纠治．效果良好。由于病例少，随访时间短．还需进一步观察。     

Pulmonary artery configuration after palliative operations for hypoplastic left heart syndrome

Pulmonary artery architecture and symmetry after palliative operations for hypoplastic left heart syndrome may affect subsequent suitability for a modified Fontan operation. Two-dimensional echocardiography was used to measure pulmonary artery diameter and assess symmetry after two types of systemic-pulmonary artery shunts: modified right Blalock-Taussig shunt (14 patients) and central shunt (from underside of aortic arch gusset to pulmonary artery confluence) (14 patients). Age, weight, preoperative diameter of right and left pulmonary arteries (proximal, middle, and distal segments), and mean interval between preoperative and postoperative echocardiographic studies (20.2 +/- 4.4 days in the Blalock shunt group; 19.1 +/- 6.8 days in the central shunt group) were similar. Early postoperatively, patients with a Blalock shunt showed a significant decrease in the diameter of all pulmonary artery segments except the distal right pulmonary artery. The diameters tapered from distal right to distal left pulmonary artery in this group. Patients with the central shunt had a significant decrease in the diameter of all pulmonary artery segments. There were no significant differences when cross comparisons were made of the various pulmonary arterial segments in patients after a central shunt. Similar findings persisted in 19 patients from both groups who had a late postoperative echocardiogram (mean interval between studies = 271 days in the group of 10 patients with Blalock shunt and 167 days in the group of nine patients with a central shunt). In conclusion, the central shunt preserves pulmonary artery symmetry, which may be important in candidates for the Fontan operation in infancy.     

Novel surgical approach 'intrapulmonary-artery septation' for Fontan candidates with unilateral pulmonary arterial hypoplasia or pulmonary venous obstruction

It is difficult to manage patients with single ventricular physiology and unbalanced pulmonary arteries. Since 1998, we started a novel approach [Intrapulmonary-artery Septation (IPAS)] to improve the result for those with severe unbalanced pulmonary arteries consisting of a well-grown pulmonary artery and an inadequately-grown pulmonary artery. The inadequately-grown pulmonary artery includes severe pulmonary arterial hypoplasia and pulmonary venous obstruction. This approach is based on the following concepts: (1) A reliable blood source should be secured to recover the inadequately-grown pulmonary artery; (2) Wasteful volume-load should be prevented for the heart; (3) Long stenosis or non-confluence of pulmonary artery should be avoided. IPAS primarily consists of (A) a Glenn shunt; (B) a systemic-pulmonary artery shunt; and (C) a septation-patch. Both (A) and (B) are adjoined on a well-grown pulmonary artery, and (C) is placed between (A) and (B). PAS brings two separate blood flows of a Glenn shunt to the well-grown side and SPS on the inadequately-grown side. IPAS was performed in 20 patients. Seventeen reached the Fontan operation. Eleven underwent postoperative catheterization and seven had acceptable-balanced pulmonary blood flow distribution. IPAS can pilot more complicated cases having severe unbalanced pulmonary arteries to the Fontan circulation.     

肺动脉闭锁合并室间隔缺损及大的体肺动脉侧支患者肺循环的解剖分析和临床意义

目的 分析肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者肺循环的病理解剖特点,并探讨其临床意义.方法 回顾性分析2002年4月至2010年6月33例肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者,男性21例,女性12例,年龄11个月～29岁.根据1999年国际儿童心脏外科数据和命名会议的分型标准,B型22例,C型11例.29例(87.9%)存在自身肺动脉,其中发育较好的6例(18.2%),发育不良或左右肺动脉无汇合的23例(69.7%).4例(12.1%)自身肺动脉完全缺如.其中31例进行了33次手术治疗,包括主动脉-肺动脉分流手术8例,一期单源化手术2例,矫治手术23例.结果 分流手术和一期单源化手术的患者术后动脉氧饱和度83%～90%.矫治手术早期死亡1例,死亡原因为多脏器功能衰竭;低心排血量综合征4例,低氧血症(氧合指数<150 mmHg,1 mmHg=0.133 kPa)3例.16例矫治手术患者随访超过1年,右心室压41～99 mmHg;肺动脉瓣中度反流2例;射血分数>50%者14例,<50%者2例.结论 肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支应根据肺血管的解剖特点采取个体化治疗的方案,存在中央肺动脉、左右肺动脉有汇合、体肺动脉侧支与肺动脉之间存在肺内交通是决定一期单源化和心内矫治手术的关键.

Abstract：

Objectives To analyze the anatomy features of the pulmonary circuits in the patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCA), and discuss the clinical significance. Methods From April 2002 to June 2010, the anatomy features of pulmonary circuits in 33 patients with PA/VSD/MAPCA were examined and analyzed. There were 21 male and 12 female patients. The age ranged from 11 months to 29 years. The anatomic types of PA/VSD included group B for 22 cases, group C for 11 cases. Thirty-one patients of them underwent 33 operative procedures. The operations included aorta-pulmonary shunt in 8 cases, one stage unifocalization with VSD open in 2 cases, complete repair in 23 cases. Results Twenty-nine (87.9%) patients had native pulmonary arteries, 6 of them were normal size and 23 were hypoplastic size. Four patients (12. 1%) had no native pulmonary arteries. The postoperative oxygen saturation of the patients undergone shunt and one stage unifocalization was increased to 83% to 90%. There was one early death after complete repair because of multiorgan function failure. There were 4 cases of severe low cardiac output and 3 cases of respiratory function failure. Sixteen patients after complete repair were followed up more than one year. The postoperative right ventricular pressure was 41 to 99 mmHg (1 mmHg =0. 133 kPa). The ejection fraction value was more than 50% in 14 patients and less than 50% in 2 patients. Two patients had medium pulmonary insufficiency. Conclusions An individualized approach based on the anatomy of the pulmonary circuits permits achievement in the patients with PA/VSD/MAPCA. The surgical strategy for PA/VSD/MAPCA mainly depends on the anatomy features of native pulmonary arteries, confluent pulmonary arteries and MAPCA.     

完全性大动脉转位合并重度肺动脉高压增加调转术后病死率

目的 探讨完全性大动脉转位(TGA)术前肺动脉高压与术后近、中期结果的关系.方法 将101例行动脉调转术的病儿根据术前平均肺动脉压力(mPAP)分为3组,肺动脉压力正常(对照)组,轻度肺动脉高压组和重度肺动脉高压组,比较分析不同组间的术后近中期结果.结果 手术后肺动脉高压组病儿肺动脉压力均有明显下降.术后各组间并发症和手术死亡比例差异无统计学意义.但是重度肺高压组中期病死率较高.结论 mPAP＜50 mm Hg(1 mm Hg=0.133 kPa)的TGA合并肺动脉高压病儿可以进行大动脉调转术,并可取得较好的术后结果,但mPAP≥50 mm Hg者虽然行大动脉调转术后肺动脉压力明显下降,且术后早期结果良好,但随访中期病死率较高.