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。     

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。     

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.     

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。     

双向腔肺分流术在复杂先心病治疗中的应用

目的探讨运用双向腔静脉-肺动脉分流术（BCPS）治疗复杂先心病的疗效及安全性。方法47例复杂先心病患者,21例在体外循环下,26例在非体外循环下完成BCPS手术。共行单侧BCPS术30例,双侧BCPS术17例。结果术后同术期死亡3例,病死率6．38％。随访35例,有7例术后2年左右进行二期根治手术,2例术后2年死亡,余26例心彩超复查无吻合口狭窄,发绀得到不同程度的缓解,心功能改善,生活质量明显提高。结论双向腔静脉-肺动脉分流术对于不能一期矫治的功能性单心室类复杂先心病患者是一种有效的姑息手术方法,非体外循环下行腔肺吻合术有助于心、肺功能的早期恢复,还可减少患者的住院开支,是安全、可行且有利的。     

心外管道全腔静脉肺动脉连接术疗效分析

目的 :分析心外管道全腔静脉肺动脉连接手术的治疗效果。方法 :1990年 1月至 2 0 0 0年 1月施行心内全腔静脉肺动脉连接术 34例 ,心外管道全腔静脉肺动脉连接术 14例 ,对其围术期多种疗效指标进行分析。结果 :与心内全腔静脉肺动脉连接术相比 ,心外管道全腔静脉肺动脉连接术后多巴胺用量减少 ,中心静脉压降低 ,体外循环时间、胸腔引流管留置时间及监护天数均明显缩短。手术死亡率7 1%。结论 :心外管道全腔静脉肺动脉连接术有利于改善术后心脏功能 ,疗效优于心内全腔静脉肺动脉连接术。     

经导管封堵全腔静脉肺动脉连接术外管道开窗的初步临床应用

目的 探讨经导管封堵全腔静脉肺动脉连接术外管道开窗的方法及疗效.方法 2007年3至7月共行3例外管道开窗封堵术,男2例,女1例,年龄分别为7、14及8岁,术前诊断均为发绀,属复杂先天性心脏病,不能行双心室矫治,而行全腔静脉肺动脉连接术,术中均加行外管道开窗.术后患者持续存在口唇发绀,血氧饱和度降低.3例患者行心导管及造影检查后证实上、下腔静脉与肺动脉吻合口通畅,无狭窄改变,肺动脉平均压均小于15 mm Hg(1 mm Hg=0.133 kPa),分别采用AGA公司Amplatzer动脉导管未闭封堵器及房间隔缺损封堵器对外管道与右房之间的交通(开窗)进行封堵.结果 所有患者均封堵成功,封堵后即刻、15 min、30 min及1 h分别测肺动脉及腔静脉压,与封堵前比较无明显升高,且患者无明确不适症状,血氧饱和度较封堵术前均有提高,发绀减轻,超声心动图及造影证实封堵器形态及位置良好.无术中及术后并发症,术后随访3至6个月,经超声心动图证实封堵器形态及位置良好,无残余分流.结论 经导管外管道开窗封堵术技术成功率高、疗效确切,但远期疗效尚需密切随访.     

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。     

一期全腔静脉-肺动脉连接术治疗功能性单心室患者的中期疗效

目的探讨全腔静脉肺-动脉连接术(total cavopulmonary connection,TCPC)治疗功能性单心室患者的术后临床疗效。方法回顾性总结广东省人民医院2008年1月1日至2016年12月31日一期TCPC治疗功能性单心室患者50例的临床资料。其中女17例(34%),男33例(66%);手术年龄(10.5±7.6)岁,体质量(25.3±13.2)kg。所有患者术前均有活动后发绀、气促等临床表现。术前动脉血气示饱和度为80.1%±7.5%,术前血红蛋白浓度为(176.9±29.3)g/L,术前红细胞压积为0.52%±0.09%,术前肺动脉指数为(345.5±174.0)mm^2/m^2,术前肺动脉压力为(14.4±4.3)mmHg(1 mmHg=0.133 kPa)。随访截至2016年12月31日。结果全组患者住院期间病死率为10%,术后5年、8年生存率均为86%。COX多因素回归分析未发现与术后死亡相关的危险因素。随访期间10例患者需再次干预治疗,主要并发症包括反复胸腔积液、心包积液、房室瓣反流、蛋白丢失性肠病。术后5年、8年未干预率为82%,71%。结论一期TCPC是治疗功能性单心室达到生理性矫治的有效手术方案,但术前应严格把握适应证,具备条件的患者可行一期手术,对高危患者仍需行分期手术降低手术风险,减少死亡及中远期并发症。     

可调式房间隔造口术在全腔静脉肺动脉连接术中的临床应用

目的介绍可调式房间隔造口技术在全腔静脉肺动脉连接术中临床应用的经验。方法3例紫绀型复杂性先天性心脏病患儿，在施行全腔静脉肺动脉连接术中采用了可调式房间隔造口技术。1例术后中心静脉压＜15mmHg，予以关闭房间隔造口；2例术后中心静脉压＞15mmHg，予以永久性开放房间隔造口（分别为5mm，4mm）。结果3例患儿术后均无严重并发症；2例开放房间隔造口者，术后动脉血氧饱和度维持在90％以上；均于手术12～18天痊愈出院。结论在全腔静脉肺动脉连接术中，采用可调式房间隔造口技术比采用房间隔开窗术具有更大和更多的优点。     

Pulmonary and caval flow dynamics after total cavopulmonary connection

OBJECTIVE: To assess flow dynamics after total cavopulmonary connection (TCPC). DESIGN: Cross-sectional study. SETTING: Aarhus University Hospital. PATIENTS: Seven patients (mean age 9 (4-18) years) who had previously undergone a lateral tunnel TCPC mean 2 (0. 3-5) years earlier. INTERVENTIONS: Pressure recordings (cardiac catheterisation), flow volume, and temporal changes of flow in the lateral tunnel, superior vena cava, and right and left pulmonary arteries (magnetic resonance velocity mapping). RESULTS: Superior vena cava flow was similar to lateral tunnel flow (1.7 (0.6-1.9) v 1. 3 (0.9-2.4) l/min*m2) (NS), and right pulmonary artery flow was higher than left pulmonary artery flow (1.7 (0.6-4.3) v 1.1 (0.8-2. 5) l/min*m2, p < 0.05). The flow pulsatility index was highest in the lateral tunnel (2.0 (1.1-8.5)), lowest in the superior vena cava (0.8 (0.5-2.4)), and intermediate in the left and right pulmonary arteries (1.6 (0.9-2.0) and 1.2 (0.4-1.9), respectively). Flow and pressure waveforms were biphasic with maxima in atrial systole and late ventricular systole. CONCLUSIONS: Following a standard lateral tunnel TCPC, flow returning via the superior vena cava is not lower than flow returning via the inferior vena cava as otherwise seen in healthy subjects; flow distribution to the pulmonary arteries is optimal; and some pulsatility is preserved primarily in the lateral tunnel and the corresponding pulmonary artery. This study provides in vivo data for future in vitro and computer model studies.     

Esophageal varices in congenital heart disease with total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) is an uncommon congenital anomaly in which the anatomical presentations vary widely among patients. We hereby present two newborns with TAPVC associated with asplenia syndrome; both had severe esophageal varices due to infradiaphragmatic pulmonary venous drainage. Ultrafast computed tomography (CT) scanning was superior to color Doppler echocardiography and cardiac catheterization as it provided a detailed portrait of the pulmonary drainage. The remarkable radiographic manifestations are presented.     

Feasibility and safety of immediate perioperative implantation of intravascular stents in cavopulmonary pathways.

Fontan-type procedures are widely used for palliation of patients with many forms of a functional univentricular heart. An ideal Fontan circuit should be free of any distortion or stenosis. The use of stents for the treatment of stenotic and hypoplastic vessels is well established in pediatric cardiac practice. We report the successful use of endovascular stents during the immediate postoperative period in patients undergoing cavopulmonary anastomoses.     

Changes in pulmonary artery size before and after total cavopulmonary connection

Objective—To assess changes in size of the central pulmonary arteries following a total cavopulmonary connection (TCPC).
Design—A retrospective analysis of the angiographic diameters of the central pulmonary arteries, expressed as z scores, in infancy before the TCPC and 3.5 (0.9) years (mean (SD)) later. Analysis of the relation between the pulmonary arteriolar resistance and the z scores at follow up.
Setting—Tertiary referral centre.
Patients—32 patients who had TCPC from February 1990 to July 1993.
Results—The patients were divided into two groups (n = 16) depending on their preoperative flow ratio: group I, Qp/Qs  1; group II, Qp/Qs > 1. At the initial study in infancy the mean z scores in group I were −6.0 for the right pulmonary artery (RPA) and −9.6 for the left pulmonary artery (LPA); in group II the respective values were −2.7 and −3.0. Before the TCPC the values increased to 0.5 (RPA) and −0.5 (LPA) in group I, and to 8.8 (RPA) and 8.2 (LPA) in group II. At follow up the z scores decreased to −2.4 (RPA) and −4.9 (LPA) in group I, and to 2.2 (RPA) and −0.7 (LPA) in group II. The changes in pulmonary artery diameters were significant for both groups (p < 0.02). Following the TCPC, no significant difference in pulmonary arteriolar resistance index was found between patients with relatively small pulmonary arteries (z score RPA+LPA  0) and those with relatively large pulmonary arteries (z score RPA+LPA > 0).
Conclusions—Creation of a TCPC results in a significant reduction in size of the central pulmonary arteries. At a mean interval of 3.5 years following the TCPC, however, there was no significant difference in pulmonary arteriolar resistance index between patients with smaller and larger central pulmonary arteries.

Keywords: total cavopulmonary connection;  congenital heart disease;  pulmonary artery size;  pulmonary arteriolar resistance