非体外循环下双向格林术的麻醉处理

目的探讨非体外循环下双向格林术围术期的麻醉处理。方法48例施行非体外循环下双向格林手术患者,年龄5个月至29岁,体重7～51kg,气管插管全麻,采用中小剂量芬太尼(15～20μg/kg)维持麻醉,必要时辅以低浓度的异氟醚吸入,术中通过及时补充血容量、静注血管活性药物和行上腔静脉引流等方法预见性处理循环变化。结果全组死亡1例,余均顺利完成手术。SpO2由术前(75.67±11.08)%升到(89.53±6.56)%,均于术后24h内脱离呼吸机,ICU停留时间(3.2±1.6)d,所有患者呼吸支持时间、输血浆量、术后引流量、ICU停留时间均明显少于同期CPB组(P<0.01)。结论术前正确评估患者病情,术中维护心血管功能稳定和维持低状态肺血管阻力是手术能否顺利进行的关键,同时加强血液和脑保护是术后迅速恢复的重要保证。     

Use of aortic homograft conduit in bidirectional Glenn shunt

We present our experience with a patient in whom a homograft conduit was used to connect the superior vena cava (SVC) to the right pulmonary artery (RPA) in a functionally univentricular heart where corrective repair was impossible.     

Factors influencing survival in patients after bidirectional Glenn shunt

Clinical characteristics, echocardiographic values, and catheterization data of 45 patients with a functional univentricular heart who had a bidirectional Glenn shunt instituted between November 1994 and October 2006 were retrospectively reviewed. Median age at operation was 20 months (range, 9 months to 19 years). Median follow-up time after the bidirectional Glenn operation was 4 years (range, 1 day to 11 years). The early mortality rate was 4/45 (8.9%); overall mortality was 24.4%. Actuarial survival after a bidirectional Glenn shunt was 73% +/- 8% at 5 years and 55% +/- 17% at 10 years. In multivariate Cox proportional hazards analysis, heterotaxy syndrome and systemic right ventricle were independent predictors of mortality after the bidirectional Glenn shunt. Age at operation, oxygen saturation, previous surgery, a pulsatile Glenn shunt, cardiopulmonary bypass, postoperative pulmonary artery pressure, bilateral superior venae cavae, and Nakata index were not predictive of mortality. The presence of heterotaxy syndrome and systemic right ventricle in patients with a functional univentricular heart should lead to aggressive investigation and management strategies.     

经皮介入栓堵双向Glenn术后扩张的奇静脉

目的:总结经皮介入栓堵双向Glenn术后扩张奇静脉的结果。方法:8例复杂性先天性心脏病患儿,双向Glenn术后发现奇静脉和/或半奇静脉扩张,平均年龄(7.7±3.3)岁,平均体质量(24±6)kg,距双向Glenn手术1.0～4.7年,平均2.7年。入院时氧饱和度(SO2)平均(0.80±0.08)。心血管造影显示扩张的奇静脉和/或半奇静脉,平均内径(8±2)mm,与上腔静脉内径比值平均0.66±0.14。肺动脉的Mcgoon比值平均2.3±0.3,Nakata指数平均(248±75)mm2/m2,平均肺动脉压(PAP)和上腔静脉压(SCVP)均为(13.4±3.3)mmHg(1 mmHg=0.133 kPa)。1例合并左肺动脉(LPA)起始部中度狭窄。于基础麻醉下经皮导管栓堵奇和/或半奇静脉,1例同时行LPA狭窄部位球囊扩张。结果:栓堵术后SO2升高至平均〔(0.86±0.04),P<0.0001〕,PAP〔(12.9±3.4)mmHg,P=0.53〕和SVCP〔(12.7±3.2)mmHg,P=0.09〕无变化。4例于栓堵术后1 w至1年行全腔静脉-肺动脉连接术(TCPC)。4例随诊1～2年SO2无变化。结论:双向Glenn术后合并奇静脉和/或半奇静脉扩张、SO2降低,但无SCVP或PAP升高者,可经皮介入栓堵奇静脉和/或半奇静脉,以增加肺血流量,提高体循环SO2。     

The bidirectional cavopulmonary (Glenn) shunt without cardiopulmonary bypass: a safe and advisable technique

The bidirectional cavopulmonary (Glenn) shunt is almost a routine first step procedure for total cavopulmonary connection in children with single-ventricle cardiac anomalies. It is usually performed with cardiopulmonary bypass, of which adverse effects can be especially deleterious in these cardiac conditions. To avoid these adverse effects, we performed the cavopulmonary shunt in 5 children through sternotomy without cardiopulmonary bypass. There was no mortality nor morbidity. We think that this technique is safe, reproducible, and even advisable in children with single-ventricle anomalies.     

有搏动性双向Glenn术治疗小婴儿复杂先心病的临床分析

目的 回顾性总结3岁以内复杂先心病患儿行有搏动性双向Glenn术的治疗效果,探讨双向Glenn分流术的手术时机.方法 收集2008年1月至2013年7月间3岁内接受双向Glenn术的77例患儿资料,根据年龄分为A组(小婴儿组,＜6个月,23例)和B组(婴幼儿组,6-36个月,54例),比较两组患儿围手术期及术后随访资料.结果 A、B两组患儿的手术时间[(148.20±21.21)min、(154.87±52.37)min]、呼吸机辅助时间[(30.63±23.86)h、(24.61±18.83)h]、ICU滞留时间[(6.35±4.16)d、(4.90±2.57)d]、术后住院时间[(12.11±3.68)d、(10.03±4.82)d]、血制品使用量[(401.21±276.79)ml、(435.76±226.95)ml]及术后引流量[(15.68±7.32)ml/kg、(17.78±8.43)ml/kg]比较差异均无统计学意义(P＞0.05).术后两组患儿肺动脉均较术前明显发育(P＜0.05),两组间发育程度比较差异无统计学意义(P＞0.05).18例患儿(A组6例,B组12例)后期接受了TCPC治疗.结论 对于无法行一期根治的复杂发绀型先心病患儿,即使是＜6个月的小婴儿,有搏动性双向Glenn术都是安全、有效的治疗方法.     

Off-pump bidirectional Glenn shunt by active decompression of the superior vena cava

We report 2 cases of infants in whom off-pump bidirectional Glenn shunts were performed. A technique of decompressing the superior vena cava by active manual aspiration has been described. The challenges of maintaning the hemodynamic status, and cerebral protection maneuvers in association with mild hypothermia and a high transcranial pressure have been highlighted.     

Risk factors in the Glenn bidirectional shunt as an intermediate procedure before Fontan correction

OBJECTIVE: The bidirectional Glenn shunt is the most common palliation before the Fontan repair, especially in high-risk patients. We studied the influence of certain risk factors in bidirectional Glenn results, with and without an additional source of pulmonary blood flow. METHODS: Between 1993 and 1998 twenty patients (6-53 months of age) underwent a bidirectional Glenn shunt as the intermediate repair for the Fontan procedure. Diagnoses were: 7 cases of double inlet single ventricle, 4 of tricuspid atresia, 3 of unbalanced AV septal defect, 4 of mitral atresia, 1 hypoplastic left heart syndrome and 1 TGA with hypoplasia of the right ventricle. 17 patients had undergone previous operations. Mean preoperative arterial oxygen saturation was 78.5%. In 6 patients an auxiliary source of pulmonary blood flow was added. RESULTS: Hospital mortality was 15%. In 4 patients the bidirectional Glenn failed. By univariate analysis low weight, preoperative functional status and high pulmonary pressure were factors associated with early death. In bidirectional Glenn failure only the duration of ventilatory support was significant. By multivariate analysis, preoperative functional status and pulmonary pressure were significant. Mean postoperative arterial oxygen saturation at a mean follow-up of 10 months was 84%. CONCLUSIONS: Bidirectional Glenn shunt is an effective and low-risk palliation for patients with univentricular hearts. Only low weight and high pulmonary pressure were significant in hospital mortality, and we advise the association of an additional source of pulmonary blood flow in these patients at the start. Early extubation provides correct performance of the shunt.     

Pulmonary venous wedge pressure provides an accurate assessment of pulmonary artery pressure in children with a bidirectional Glenn shunt

PURPOSE: In circulations with pulsatile pulmonary artery flow the pulmonary venous wedge pressure (PVWp) has been validated as a good estimate of pulmonary artery pressure (PAp), when PAp is low. The purpose of this study was to validate PVWp estimates of PAp in the less-pulsatile pulmonary circulation of children after bidirectional Glenn shunts. METHODS: A retrospective study was performed of 22 simultaneous measurements of PVWp and PAp made during 20 catheterizations in 19 children who had undergone bidirectional Glenn procedures. The PAp was measured directly from the branch PA ipsilateral to the side of the PVWp, or in the SVC. Pulmonary resistance (Rp) was calculated with both PAp and PVWp, to assess the impact of PAp estimates on Rp determinations. RESULTS: Patients ranged in age from 5 months to 10.7 years. There were a variety of univentricular cardiac malformations in the study group. Two children had antegrade pulmonary blood flow in addition to a bidirectional Glenn shunt. The mean PAp ranged from 4 to 14 mmHg, while mean PVWp ranged from 3 to 15 mmHg. Mean PVWp never differed from mean PAp by more than 3 mmHg. There was a significant linear relation between mean PAp and PVWp: PAp = 0.86 (PVWp) + 2.0 (R2 = 0.89; P < 0.0001). PVWp provided a good approximation of PAp regardless of the presence (n = 2) or absence (n = 19) of antegrade pulmonary flow. There was a good linear correlation between the Rp calculated by both methods (RpPAp = 0.9 (RpVWp) + 0.5; R2 = 0.74; P < 0.0001). CONCLUSION: The mean PVWp provides a close approximation of mean PAp in children with a bidirectional Glenn shunt and provides valuable hemodynamic information in cases where direct PAp measurements are unavailable.     

The pulmonary artery closure may be a thromboembolic risk factor in patients with bidirectional cavo-pulmonary (Glenn) shunt

Postoperative thrombosis after the Fontan procedure has been well noted in the literature, and its risk factors are also well known. In contrast, thrombosis after the bilateral cavo-pulmonary shunt (Glenn) has been rarely reported and almost always occurs around the anastomosis itself or near it, mainly causing pulmonary embolism. We present 2 cases with cerebral embolism 2-7 months after pulmonary artery closure and Glenn procedure, due to dislodgement of a thrombus in the proximal pulmonary artery stump. Based on these two cases and a few others reported in the literature, we want to call the attention to this new cause of thromboembolism after Glenn and stimulate discussion about its incidence, risk factors and preventive measures.     

Cardiac catheterization and comprehensive clinical evaluation after bidirectional Glenn shunt surgery in 60 patients with complex congenital heart disease

Background The bidirectional Glenn shunt surgery is a palliative procedure for patients with complex congenital heart disease(CHD) who are not suitable for biventricular repair in early life. There is limited evidence of successful strategies for long-term hemodynamic stabilization. Furthermore, there have been no data on optimal hemodynamics that could be used as a reference for patients' follow-on management. Methods Sixty CHD patients, 44 male and 16 female, with bidirectional Glenn shunt surgery and cardiac catheterization were enrolled at our hospital between January 2014 and December 2016. Pre-and post Glenn shunt percutaneous oxygen saturation(SpO_2), 6-minute walk test(6 MWT), superior vena cava pressure(SVCP), pulmonary arterial pressure(PAP), pulmonary capillary wedge pressure(PCWP), pulmonary vascular resistance(PVR), small pulmonary vascular resistance(s PVR) were measured. Pre-and post-total cavopulmonary connection(TCPC) SpO_2, and in-hospital complications were monitored. The optimal hemodynamic cutoff values for TCPC patient selection were estimated by receive operating characteristic(ROC) curve analysis. Results SpO_2 was significantly increased by bidirectional Glenn shunt surgery(75.42 ± 9.62% to 86.98 ± 7.63%, P 0.001) from 82.70 ± 5.99% to 95.00 ±4.07% in the 47 patients with TCPC. Forty-two patients completed the 6 MWT with a mean distance of 362.7 ±75.0 m and a SpO_2 decrease from 81.80 ± 7.84% to 67.59 ± 1.82%(P 0.001). The △SpO_2 and 6-minute walk distance(6 MWD) in the 32 who underwent TCPC and ten of them did not reach statistical significance(17.22 ±13.82% vs. 13.87 ± 8.74%, P = 0.08 and 358.88 ± 78.97 m vs. 374.80 ± 62.55 m, P = 0.564]. After cardiac catheterization, 47 patients were selected for TCPC. The right pulmonary artery systolic pressure(s RPAP), mean right pulmonary artery pressure(m RPAP), mean left pulmonary artery pressure(m LPAP), PVR, and s PVR were significantly lower in the TCPC group than in the non-TCPC group. The differences in superior vena cava systolic blood pressure(s SVCP), mean superior vena cava pressure(m SVCP), and left pulmonary artery systolic pressure(s LPAP) were not significant. The optimal cutoff values for TCPC were s SVCP ≤ 20 mm Hg(P = 0.025),s RPAP ≤ 22 mm Hg(P = 0.0001, mRPAP ≤ 13 mm Hg(P =0.003), s LPAP ≤ 27 mm Hg(P =0.03), m LPAP ≤ 11 mm Hg(P = 0.01), PVR ≤ 4.3 Wood U/m~2(P 0.0001) and were significantly associated with TCPC selection,except for m SVCP ≤ 19 mm Hg(P = 0.06) and s PVR ≤ 2.0 wood U/m~2(P = 0.0531). One patient died because of low cardiac output after TCPC. In-hospital mortality was 2.1%. Conclusion The SpO_2 can be significantly improved after bidirectional Glenn shunt and TCPC surgery. The 6 MWT is an index of activity tolerance prior toTCPC. Hemodynamic values of s SVCP ≤ 20 mm Hg, s RPAP ≤ 22 mm Hg, m RPAP ≤ 13 mm Hg, s LPAP ≤ 27 mm Hg, m LPAP ≤ 11 mm Hg, and PVR ≤ 4.3 Wood U/m~2 can help identify post Glenn-shunt patients indicated for TCPC.     

Transcatheter closure of native pulmonary artery for the elimination of accessory pulmonary blood flow after bidirectional Glenn shunt

We report a case where excessive accessory pulmonary blood flow via the native pulmonary valve after cavopulmonary anastomosis resulted in pulmonary hypertension and heart failure. This flow was successfully eliminated in the cardiac catheterization laboratory using an Amplazter duct occluder that was placed across the native pulmonary valve.     

双向格林术后心血管造影特征分析

目的 分析双向格林术后并发症及心血管造影特征,为临床治疗提供依据.方法 38例先天性心脏病双向格林术后患者(男性24例,女性14例)接受心血管造影检查.年龄(7.8±5.6)岁,双向格林术至造影间隔时间为(4.1±1.9)年.所有患者均行上腔静脉及肺动脉造影,并测量肺动脉-上腔静脉连续压力.结果 38例患者肺动脉平均压力为(14.8±4.5)mm Hg(1 mm Hg=0.133kPa),上腔静脉平均压力为(15.4±5.4)mm Hg;肺动脉到上腔静脉连续测压均无压力阶差.其中9例出现肺动脉压力升高,年龄为(6.6±4.7)岁,其上腔静脉平均压力为(22.9±5.1)mm Hg,肺动脉平均压力为(21.5±2.9)mm Hg.心血管造影提示14例患者[年龄(7.9±4.6)岁]出现静脉侧支血管,其中2例成功行经导管封堵术.侧支血管组上腔静脉压力[(17.8±7.2)mmHg比(14±3.6)mm Hg,P＜0.05]及肺动脉压力[(16.7±5.7)mm Hg比(13.7±3.4)mm Hg,P＜0.05]均高于无侧支血管组(n=24).14例静脉侧支血管分布:后纵隔侧支12例,其中合并前纵隔侧支3例,合并中纵隔侧支1例;单独中纵隔侧支血管2例.2例患者肺动静脉瘘形成,其中单发囊状瘘和多发动静脉瘘各1例.2例患者存在体肺动脉侧支血管,均成功行经导管栓塞术.结论 双向格林术可引起多种并发症,心血管造影检查能够早期发现并指导治疗.     

超声评价肺血减少型复杂先天性心脏病双向Glenn术后肺血管发育的临床价值

目的:探讨超声心动图评价肺血减少型复杂先天性心脏病(先心病)双向Glenn分流术后肺血管发育的临床应用价值。方法:应用二维经胸超声心动图测量101例复杂发绀型先心病患儿双向Glenn分流术前后左、右肺动脉内径,同时测量术前、术后经皮血氧饱和度,评价其术后肺血管发育。根据有无肺动脉前向血流将患儿分为肺动脉闭锁组(30例)和肺动脉狭窄组(71例);另分为婴幼儿组(年龄≤3岁,51例)和儿童组(年龄>3岁,50例),比较各组内及组间手术前后肺动脉分支内径及血氧饱和度变化。结果 :术后应用超声心动图随访12～36个月,左、右肺动脉内径及血氧饱和度较术前有不同程度改善。肺动脉狭窄组较肺动脉闭锁组术后肺动脉生长发育及血氧饱和度的改善更明显;婴幼儿组肺动脉发育及血氧饱和度改善情况优于儿童组(P<0.01)。应用超声定量的肺动脉分支发育与临床血氧相关性回归分析结果满意(P<0.001)。结论:肺血减少型复杂先心病患儿施行双向Glenn分流术后,可在不增加右心室前负荷情况下增加肺血流,促进患者肺动脉血管发育,提高血氧饱和度。超声心动图对术后定量随访评估肺血管发育,评估预后有重要的临床应用价值。     

Bidirectional Glenn shunt in an infant with prune-belly syndrome.

The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.     

双向格林术后心内膜永久起搏器入径的研究现况

双向格林(Glenn)术是先天性心脏病的一种手术治疗方式,部分患者在术后需要植入起搏器,由于此时上腔静脉经右心房的传统入径缺失,植入起搏器需要其他不同的入径。本文介绍了目前已知的几种相关入径,它们有各自的特点,分别适用于不同的临床情况,但均未成为操作常规。因此,双向Glenn术后的起搏器入径目前仍需要根据实际情况进行个体化选择。