全腔静脉肺动脉连接术治疗复杂先天性心脏病

目的总结全腔静脉肺动脉连接术(TCPC)的经验和体会。方法6例复杂先天性心脏病患者均在全麻低温体外循环下手术。上腔静脉与右肺动脉吻合完毕后,2例采用右心房内隧道法、4例采用心房外管道完成TCPC手术(合并左位上腔静脉的2例同时行左侧G lenn手术)。结果1例三尖瓣闭锁患者术后2d死亡,5例治愈者血氧饱和度由术前的0.75±0.03提高至术后的0.93±0.02,生活质量改善。结论TCPC矫治复杂先心病,能明显改善患者的缺氧症状;术中肺动脉直接测压是能否行TCPC的重要依据。     

全腔静脉肺动脉连接术治疗复杂紫绀型先天性心脏病体会

对35例复杂紫绀型先天性心脏病患者施行全腔静脉肺动脉连接术(TCPC),其中在低温体外循环心脏停跳下行右心房内侧隧道法18例、心外管道法3例,常温体外循环心脏跳动下行心外管道法11例,非体外循环下行心外管道法3例.结果 除1例术后第8天死于痰堵窒息外,余患者指端血氧饱和度明显提高,心功能改善.无晚期死亡.提示全TCPC为治疗本类疾病的有效手术方法,但应根据患者年龄、体质量、心内畸形等情况选择具体术式.     

一期或分期全腔静脉-肺动脉连接术治疗功能性单心室

目的总结、比较一期和分期全腔静脉-肺动脉连接术(total cavopulmonary connection,TCPC)治疗复杂先天性心脏病的疗效。方法分析2003年12月至2007年9月在广东省人民医院住院,适合一期生理矫治并施行了一期TCPC术的患儿25例和已先期完成第一阶段姑息手术并施行了二期TCPC术的患儿17例的临床资料。前期手术包括15例双向Glenn手术、1例体-肺分流术、1例三尖瓣下移畸形矫治加双向Glenn手术。一期TCPC组中14例采用外管道,11例肺动脉直接下拉与下腔静脉吻合。二期TCPC组中15例采用外管道,1例采用心房内通道,1例肺动脉直接与下腔静脉吻合。结果早期死亡4例,病死率9.5%(4/42)。一期TCPC组死亡3例,病死率12.0%(3/25);二期TCPC组死亡1例,病死率5.9%(1/17)。二期TCPC组的辅助通气时间、胸管停留时间、住监护室时间、术后住院天数比一期TCPC组短,但差异无统计学意义(P0.05)。随访8个月到4.8年。1例于术后8个月死于严重的房室瓣反流、心力衰竭。结论一期和分期TCPC手术治疗复杂先心病都能达到良好的效果。     

非体外循环下双向Glenn术治疗复杂先天性心脏病

目的探讨双向腔静脉—肺动脉吻合术（双向Glenn术）对复杂先天性心脏病的疗效。方法 2003年12月～2007年12月,对我院24例复杂先天性心脏病患者在非体外循环下行双向Glenn术。结果全组无手术死亡,14例术后出现头部水肿,10例出现胸腔积液,均经保守治疗痊愈。术后血氧饱和度、肺动脉压均较术前提高。结论非体外循环下的双向Glenn术是一种有效治疗复杂性先天性心脏病的方法。     

2岁以上合并重度肺动脉高压先天性心脏病行单心室修复的初步研究

目的：探讨2岁以上合并重度肺动脉高压先天性心脏病（先心病）行单心室修复的手术指征及效果。方法：回顾性分析年龄〉2岁合并重度肺动脉高压仅能行单心室修复的先心病患儿15例,男性9例,女性6例。年龄2.0～10岁,平均（7.8±3.8）岁。体质量11～24kg,平均（15.32±4.24）kg,术前经皮血氧饱和度（SPO2）88%～95%,平均（91.75±2.29）%。肺动脉压（mPAP）52～91mmHg（（1 mm-Hg=0.133 kPa））,平均（54.61±16.8）mmHg。心脏畸形：单心室7例,三尖瓣闭锁3例,室缺远离两大动脉伴左心室发育不良型右心室双出口2例,心室不均衡型完全型房室通道2例,十字交叉心1例。均在静吸复合麻醉下先期行肺动脉环缩术,后期逐步完成双向格林或全腔肺动脉吻合术。结果：全组无住院死亡;PAB术前后mPAP、SPO2明显下降;PAB术后3～6年内完成双向格林（Glenn）4例,全腔肺动脉吻合术（TCPC）2例。结论：PAB术能有效降低肺动脉压力,≥2岁PAB术后可进一步完成Glenn或TCPC术。     

改良Fontan术治疗内脏异位综合征的术后护理

目的总结对15例Fontan手术治疗内脏异位综合征患者的术后护理经验。方法 Fontan手术是内脏异位综合征主要的手术方式,术中均采用心外管道建立下腔静脉一肺动脉连接。术后患者易出现肺动脉高压、心律失常、蛋白丢失性肠病、持续性胸腔积液等并发症,手术风险大。通过体位护理、呼吸机气道管理和并发症护理,为患者提供良好的康复条件。结果 15例患者经精心的治疗与护理,术后早、中期结果良好,无住院死亡,1年随访死亡1例。结论有针对性的、个性化的术后护理有利于Fontan手术患者顺利康复。     

非体外循环下复杂先天性心脏病的姑息手术

目的:探讨非体外循环下复杂先天性心脏病姑息手术适应证及处理要点。方法:2008-01-2010-06对26例复杂先天性心脏心病患儿实行非体外循环下姑息手术,其中肺动脉环缩术5例,改良体肺分流术6例,双向腔肺分流术14例,双向腔肺分流+改良体肺分流术1例;其中功能单心室9例,法洛四联症肺动脉闭锁6例,右室双出口4例,三尖瓣闭锁3例,完全性大动脉转位2例完全心内膜垫缺损并肺动脉闭锁1例,Ebstein畸形1例。结果:死亡4例,22例患者顺利出院,死亡率15.4%;死亡原因包括低氧血症及心功能衰竭、肺部感染合并心功能衰竭;肺动脉环缩术、改良体肺分流术、双向腔肺分流术后平均血氧饱和度分别为(81.8±2.4)%、(90.0±3.2)%、(86.4±4.1)%;术后并发症包括肺部感染3例,胸腔积液7例,上腔静脉综合征1例。术后随访2个月～1年,无远期死亡,心功能NYHAⅠ级7例,NYHAⅡ级15例。结论:非体外循环下姑息治疗是复杂先天性心脏病的有效治疗手段,特别适用于有体外循环高危因素患者,手术适应证的正确把握、术中术后合理的处理是降低死亡率的关键。     

一期Fontan手术治疗复杂紫绀型先天性心脏病21例

目的：总结一期房坦（ Fontan）手术治疗复杂紫绀型先天性心脏病的临床经验和疗效。方法2007-06～2012-12该院心血管外科对21例复杂紫绀型先天性心脏病患者施行了一期Fontan单心室手术,同期行房室瓣整形术8例。12例采取心内管道或自体心包内隧道连接,8例采取心外管道连接,1例采取不用管道材料肺动脉直接下拉吻合法。5例保留房间隔缺损或行心房板障开窗。结果20例手术获得成功,1例术后当天因低心排出量综合征死亡。1例房室瓣成形术后随访至25个月出现房室瓣重度反流,行机械瓣置换。术前经皮血氧饱和度为67％～91％,术后经皮血氧饱和度为88％～95％。术后随访（7～52个月）所有患者心功能Ⅰ～Ⅱ级。结论一期Fontan手术治疗复杂紫绀型先天性心脏病安全有效,可以避免多次手术创伤,节约医疗资源,但应注意把握手术适应证。     

心外管道全腔静脉与肺动脉连接术治疗复杂先天性心脏病(附3例报告)

对3例无法进行双心空矫治的复杂先天性心脏病患者，采用心外管道全腔静脉与肺动脉连接术行单心室矫治，近期手术效果满意。认为该术式是治疗复杂先天性心脏病的良好方法之一。     

功能性单心室全腔静脉-肺动脉连接术后管理

目的:探讨全腔静脉-肺动脉连接术(total cavopulmonany correction,TCPC)治疗功能性单心室的术后管理经验。方法:2012年1月~2013年6月我院共行TCPC手术60(男43,女17)例;年龄(5.5±1.7)岁,体质量(18±4)kg。术前脉搏血氧饱和度(80±7)%,血色素(181±17)g/L。术前射血分数(EF)(64±7)%,平均肺动脉指数(371±234)mm2/m2,Mcgoon比(2.2±0.7),术前平均肺动脉压(12±4)mm Hg。60例患者均在全身麻醉、体外循环并行辅助下手术,5例因同期行心内畸形矫治需阻断主动脉。心外管道为直径18~22 mm Gore-tex血管,术毕开窗25例(42%)。为保证患者术后顺利恢复,治疗方面必须注意以下几点:1降低肺血管阻力。2保证足够的容量负荷。3增加心肌收缩力。4控制心律失常。5妥善处理胸腔积液。6术后常规抗凝。结果:患者住院期间有2例(3%)死亡。55例并行循环手术患者体外循环时间(112±52)min。5例患者需要停循环修补心内畸形,主动脉阻断时间30~52 min。呼吸机使用时间(19±6)h。术后住ICU时间(5.1±2.1)d。术后胸液引流时间(15±12)d。术后住院天数(24±12)d。本组患者术后10例出现低心排综合征。5例出现急性肾功能衰竭,行腹膜透析或血液滤过治疗,其中2例死亡,余治愈出院。20例出现顽固性胸腔积液(胸引时间大于2周),25例乳糜试验阳性,1例患儿出现蛋白丢失性肠病,最终并发肺部感染死亡。4例术后出现心律失常,心律表现为短阵房性心动过速,交界性心动过速等,其中1例难以矫治的室上性心动过速导致低心排、多脏器衰竭、死亡。10例并发术后肺部感染。所有存活患儿术后腔静脉压力下降明显[(12±4)mm Hg vs.(9±3)mm Hg,P0.05],氧饱和度改善明显[(80±7)%vs.(97±4)%,P0.01],血色素恢复正常[(181±17)g/L vs.(125±29)g/L,P0.01]。结论:根据TCPC术后病理生理的改变,制定合理的治疗方案,可提高TCPC术后成功率和减少术后并发症。     

Protein‐losing enteropathy resolved by percutaneous intervention

The extracardiac total cavopulmonary connection is the final stage of palliation of hearts with single‐ventricle physiology. Protein‐losing enteropathy (PLE) and plastic bronchitis are catastrophic sequale that may occur in patients with the total cavopulmonary connection and may complicate the early and long‐term follow‐up. Here we report on the successful treatment of a 16‐year boy affected by PLE by percutaneous closure of a persistent anterograde pulmonary blood flow by using an 8‐mm Amplatzer VSD Occluder. © 2011 Wiley‐Liss, Inc.     

Total cavopulmonary connection with extraatrial tunnel

Between April 1997 and February 2000, total cavopulmonary connection with an extraatrial tunnel was used to treat 9 cases of complicated congenital heart disease: single ventricle (4), double-outlet right ventricle (3), mitral atresia (1), and tricuspid atresia (1). There was no mortality. One patient developed bacterial endocarditis and required reoperation after 52 days to replace the tunnel. At follow-up ranging from 11 months to 3 years, 3 patients were in New York Heart Association functional class I, and 6 were in class II. One patient with single ventricle had refractory supraventricular tachycardia after a modified Fontan operation 4 years earlier, which was cured by the total cavopulmonary connection procedure. The essential factors for a good outcome include appropriate surgical indication, avoidance of aortic crossclamping and cardiac arrest, and unobstructed anastomosis between the superior and inferior venae cavae and the pulmonary artery.     

Total cavo-pulmonary bypass. Short-term results]

In order to reduce the complications of the Fontan procedure, 2 techniques of total cavopulmonary connection using a right intra-atrial baffle (Pugas' technique, 19 patients), and a modified de Kreutzer (19 patients) were assessed in 38 consecutive patients operated between November 1980 and November 1991. All patients had complex cyanotic congenital heart disease: tricuspid atresia (13), single ventricle (12), hypoplasia of one of the two ventricles (8), pulmonary atresia with an intact septum (5). The average age was 5.8 +/- 5.6 years (range 1 to 30 years), the weight was 18 +/- 11.7 kg (range 8 to 59.7 kg). Six patients (15.8%) died, 4 of whom (10.5%) in the operative period. Five of these deaths were related to extremely high right heart pressures without any technical fault, and the other to thrombosis of the conduit in a patient not taking anticoagulants. Two immediate and 10 secondary reoperations were necessary. All 3 patients who had disconnection of the anastomosis because of high right heart pressures died. The postoperative course was often difficult: right heart failure (63%), pleural effusion (57%), and pericardium effusion (13%). Of the risk factors analysed, age had no influence on postoperative outcome. The two poor prognostic factors were: presence of atresia or hypoplasia of the systemic atrioventricular valve (mortality 42.8% versus 6.5%, p = 0.05 and prolongation of the hospital period: 39.5 +/- 32 days versus 21 +/- 11 days, p less than 0.05) and previous banding (reoperation rate 62.5% versus 16.6%, p less than 0.05, and longer stay in the intensive care unit and hospital period).(ABSTRACT TRUNCATED AT 250 WORDS)     

Is it necessary to routinely fenestrate an extracardiac fontan?

OBJECTIVES: This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan. BACKGROUND: Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined. METHODS: Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients. RESULTS: Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy. CONCLUSIONS: Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.     

Fenestrated Fontan surgery in high risk patients]

A retrospective analysis is presented of all patients who had fenestrated Fontan procedure between january 1990 and may 1996. Surgery was indicated in the presence of anyone of the following risk factors: mean pulmonary pressure higher than 20 mmHg; pulmonary vascular resistance higher than 2 UW; ejection fraction less than 60%; systemic ventricular end diastolic pressure higher than 8 mmHg; Nakata index less than 200 mm2/m2, McGoon index less than 2. The diagnosis were: Absent right atrio-ventricular connection with concordance ventriculo-arterial connection 10 patients; pulmonary atresia with intact septum, 1 patient; Ebstein's malformation, 1 patient and absent left A-V connection with discordance VA connection, 1 patient. The mean of age was 6.7 years (range 2.5-11 years). Overall mortality was 23%. No significant difference in risk factors was found between survivals and no survivals. Nonsurvivors had between two an four risks factors. Postoperative complications were 1 patient with protein losing enteropathy and stroke (1 patient). The mean duration of pleural effusion was 16 days (range 4-45 days). We consider fenestrated Fontan procedure useful for patients with congenital heart disease with a one hypoplastic ventricle and one o more risks factors.     

Total cavopulmonary connection with off-pump technique

The aim of this study was to evaluate an off-pump technique for total cavopulmonary connection. Between May 2000 and June 2004, 16 patients underwent total cavopulmonary connection without cardiopulmonary bypass. There were 8 males and 8 females, with a mean age of 9.8 +/- 6.2 years and a mean weight of 27.7 +/- 14.9 kg. Preoperative percutaneous oxygen saturation was 75% +/- 6%, and pulmonary arterial pressure was 12.6 +/- 2.7 mm Hg. Postoperative outcomes were retrospectively compared with a group of 17 patients who underwent total cavopulmonary connection with an extracardiac conduit under cardiopulmonary bypass. Two patients died early postoperatively. Chylothorax occurred in 2 patients and 3 underwent reoperation for fenestration between the extracardiac conduit and the right atrium in the early postoperative period. All survivors were acyanotic. On discharge from hospital, the percutaneous oxygen saturation breathing air was 95% +/- 3%. Patients in the off-pump group demonstrated significantly shorter extubation time and intensive care unit stay. This connection technique is easy to perform and helpful in the early management of patients with a functional univentricular heart. However, much remains to be learned about this unique physiologic system.     

PiCCO技术对复杂先天性心脏病患儿围手术期治疗的意义

目的：探讨脉搏指数连续心排出量（pulse indicated continuous cardiac output，PiCCO）监测在复杂性先天性心脏病患儿围术期治疗的指导意义。方法：选取复杂性先天性心脏病矫治术后的患儿104例，随机分为两组，PiCCO组（51例），均放置PiCCO导管监测平均动脉压（MAP）、心输出量（CO）、连续心输出量（CC0）、外周血管阻力（SVR）、胸腔内血容量（ITBV）、全心舒张末期容量（GEDV）、血管外肺水（EVLw）等，并指导临床治疗。对照组（53例），不放置PiCCO导管，凭经验及其他常规指标指导治疗。结果：PiCC0组机械通气时间、ICU滞留时间、血管活性药物使用时间显著短于对照组，但两组患儿左室射血分数（LVEF）、脑钠尿肽（BNP）、胸片渗出情况、感染率、病死率均无统计学差异。结论：PiCCO技术在复杂性先天性心脏病患儿术后治疗中有一定的实用价值。