心外管道全腔静脉肺动脉吻合术2例护理体会

全腔静脉肺动脉吻合通过减轻心脏容量负荷改善慢性缺氧,使三尖瓣闭锁、右心室发育不良、功能性单心室等不能经心室矫治的复杂先心病得到有效治疗。2007年9月-2009年1月,我们共为2例患者行心外管道全腔静脉肺动脉吻合术,效果满意。现将护理体会介绍如下。     

心室间隔缺损心电图心室肥厚与肺动脉压力、缺损大小和分流量的对比分析

目的：分析８３例心室间隔缺损（ＶＳＤ）患者术前心电图改变与血流动力学及缺损大小之间的关系，为ＶＳＤ术前提供初步预测性意见。方法：依据心电图分为正常、左心室肥厚、右心室肥厚和双心室肥厚４组，与右心导管检查所测肺动脉压力和阻力、左向右分流量和术中所见缺损大小、心室壁厚度进行对比。结果：心电图为左心室肥厚、心室间隔为中小缺损且呈中大分流量者，肺动脉压力多为轻中度增高；心电图为右心室肥厚和双心室肥厚，心室间隔多为中大缺损且分流量大者多有重度肺动脉高压，右心室肥厚，肺总阻力增高较双心室肥厚者严重。与术中所见心室壁肥厚比较，心电图诊断左、右心室肥厚符合率较高，分别为１００．０％及８５．７％，但心电图正常者中有７８．６％术中所见为左心室肥厚。结论：ＶＳＤ心电图改变可以间接评估其血液动力学改变及缺损大小。     

骨骼肌心室代替右心室功能的动物实验研究

为探索骨骼肌心室代替右心室功能的可行性，用犬进行下列实验研究：①骨骼肌心室经同种带瓣主动脉连接于腔静脉与肺动脉间的模型；②骨骼肌心室连接于右心房与肺动脉间的模型。结果显示，在骨骼肌心室与犬心脏同步收缩时可明显提高主动脉、肺动脉收缩压及肺动脉血流量，且肺动脉血流出现搏动性，但中心静脉压仅轻度降低。     

双向上腔静脉肺动脉吻合术的临床应用

双向上腔静脉吻合术（BCPA），又称双向格林手术，是将上腔静脉和（或）左上腔静脉的体静脉回流血分流到两肺的一种手术。其主要步骤是将上腔静脉在近右心房交界处上方离断近心端缝闭远心端与右肺动脉端侧蜘合（如是左上腔静脉,则与左肺动脉吻合）。这种分流手术使上半身约相当1/3的体循环静脉血在低压下,直接进入肺动脉进行氧合，使原来右向左分流的血液量减少，通过提高有效肺血流量而增加体循环动脉血氧饱和度，同时改善肺血管发育，碱轻功能单心室的负荷，改善心室的几何构型。     

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。     

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。     

合并肺动脉高压的右心室双出口肺动脉环缩术后Ⅱ期手术治疗效果分析

目的:总结合并肺动脉高压的右心室双出口(DORV)患者Ⅰ期行肺动脉环缩术后,Ⅱ期手术的结果。方法:入选既往在本中心接受肺动脉环缩术的合并肺动脉高压的DORV患者共15例,男性12例,女性3例,年龄(5.3±2.1)岁,体质量(17.2±4.0)kg。术前经皮血氧饱和度(78.3±3.2)%。11例(73%)在体外循环下行双心室矫治术,其中2例行心室内隧道补片,9例行心室内隧道+右心室流出道扩大补片术。术中均拆除原肺动脉环缩带。同期矫治合并畸形。4例(27%)在非体外循环下行右侧双向Glenn术,术中均结扎奇静脉。结果:11例双心室矫治患者中,死亡1例,原因为术后左室流出道狭窄、低心排综合征。室间隔缺损残余分流1例。余无严重围术期并发症发生。4例行单心室类手术(Glenn)患者均未出现围术期死亡或严重并发症。结论:对于无法接受Ⅰ期根治的合并肺动脉高压的DORV患者,可采用Ⅰ期先行肺动脉环缩术,择期行Ⅱ期手术的策略。     

小儿双向腔静脉肺动脉吻合术围术期处理(摘要)

小儿双向腔静脉肺动脉吻合术（双向Ｇｌｅｎｎ术）是近年来提出的复杂先天性心脏病（先心病）手术疗效较佳的一种半生理矫治术，这类患儿的围术期处理具有特殊性，本文报告１２例小儿复杂先心病双向Ｇｌｅｎｎ术病例，重点探讨围术期治疗。１临床资料阜外心血管病医院小儿...     

右心旁路及右心室肺动脉旁路手术后补液标准探讨

1　对象与方法　　自 1996年 6月至 2 0 0 1年 11月 ,采用右心旁路及右心室肺动脉旁路手术治疗肺血减少型重症复杂性先天性心脏病 62例 ,年龄 0 5～ 7 0岁 ,平均 (3 0 4± 1 5 8)岁 ,体重 6～ 2 3kg ,平均 (12 66± 3 5 8)kg。采用的手术方法分别是 :右心室肺动脉旁路手术 19例 (经典Rastelli手术 11例 ,右心室肺动脉外通道手术 8例 ) ;右心旁路术 43例 ,包括 :改良Fontan手术 12例 ,全腔静脉肺动脉连接 (TCPC) 4例 ,一个半心室矫治术 2例 ,双向Glenn手术 2 5例 (其中体外循环下 8例 ,非体外循环17例 )。手术后早期死亡 5例 (8 0 6% …     

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。     

A better approach for left ventricular training in transposition of the great arteries and intact interventricular septum: Bidirectional cavopulmonary anastomosis and pulmonary artery banding

Objective: Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients.
Methods: Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage‐1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re‐anastomosis of the superior vena cava to right atrium after an interstage period of 21‐30 months (stage‐2: anatomical repair).
Results: Any mortality was not encountered. The left ventricular mass indices increased from 18‐32 to 44‐74 g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow‐up period was 20 months (9‐32 months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function.
Conclusions: Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic‐to‐pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.     

Does hypoplasia of one pulmonary artery preclude a definitive repair in pulmonary atresia,intact ventricular septum,and hypoplastic right ventricle?

Summary Twins with pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle associated with underdeveloped and discontinuous left pulmonary artery are described. Operations to connect the left pulmonary artery to the main pulmonary trunk, with subsequent biventricular repair assisted by bidirectional cavopulmonary anastomosis and atrial fenestration were performed. Follow-up catheterization proved spontaneous closure of the fenestration in one patient. Both patients have been in a satisfactory condition for 3 years since operation.     

Systemic venous collateral development after the bidirectional cavopulmonary anastomosis: Prevalence and predictors

Objectives. To determine the prevalence of systemic venous collaterals after the bidirectional cavopulmonary anastomosis and the factors associated with their development.Background. Systemic venous collaterals have been found after cavopulmonary anastomosis.Methods. Cardiac catheterization was performed in 103 patients before and after a bidirectional cavopulmonary anastomosis.Results. After surgery, 51 venous collaterals were identified in 32 patients (31%). Collateral development was associated with an abnormal superior vena caval connection (56% incidence vs. 26% with a single right superior vena cava, p = 0.01) and postoperative factors including pulmonary artery distortion (53% incidence vs. 22% without distortion, p = 0.002); increased superior vena caval mean pressure (14 ± 5 mm Hg versus 11 ± 4 mm Hg with no collaterals, p = 0.0002); increased pulmonary artery mean pressure (13 ± 4 mm Hg vs. 11 ± 4 mm Hg with no collaterals, p = 0.02); lower right atrial mean pressure (5 ± 2 mm Hg vs. 6 ± 3 mm Hg with no collaterals, p = 0.04); and increased mean gradient between superior vena cava and right atrium (8 ± 3 mm Hg vs. 5 ± 4 mm Hg with no collaterals, p = 0.0002). Using multiple logistic regression, only this last factor was independently associated with collateral development with an odds ratio per 1 mm Hg of 1.33 (95% CI 1.12–1.58, p = 0.001) for their presence.Conclusions. Systemic venous collaterals occur frequently after a bidirectional cavopulmonary anastomosis and are found postoperatively when a significant pressure gradient occurs between cava and right atrium.     

Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedure but (re-)opened after surgery, leading to cyanosis either by reducing effective pulmonary blood flow (bidirectional cavopulmonary connection) or by an obligatory right to left shunt (Fontan). These observations suggest that, embryologically, the lumen of the left SVC obliterates rather than disappears. Balloon occlusion angiography of the innominate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.

Keywords: persistent left superior vena cava; cavopulmonary connection; Fontan procedure; congenital heart disease     

The history of right heart bypass before Fontan.

Numerous clinical and experimental studies have suggested that adequate pulmonary blood flow and normal central venous pressure may be maintained without significant contribution of right ventricular function. These data induced experimental and clinical research in the quest for complete bypass of the right heart. More than 40 different operations have been designed to bypass the right ventricle. Partial right ventricular bypass was achieved in 1949 by anastomosing the superior vena cava to the pulmonary artery in animal experiments. In 1950 such as operation was successfully performed clinically for the first time. Complete bypass of the right heart was accomplished for the first time experimentally in 1954 by performing a superior vena cava-to-right pulmonary artery anastomosis and implanting the inferior vena cava into the left atrium. Successful clinical bypass of the right heart was carried out later by implanting both vena cavae into the pulmonary circulation. Until the cavopulmonary shunt conclusively proved the validity of the concept that compensated circulation is possible without participation of the right ventricle, surgeons did not fully understand its possible relevance in clinical situations. The development of partial and complete right heart bypass procedures, both experimental and clinical, not only presented us with viable alternatives to aortopulmonary shunts like the Blalock-Taussig or Potts anastomosis, but it also opened the way to development of new operations such as the Fontan procedure which are based on the principle of low pressure pulmonary flow and right ventricular bypass.     

Congenital Portosystemic Venous Connections and Other Abdominal Venous Abnormalities in Patients with Polysplenia and Functionally Univentricular Heart Disease: A Case Series and Literature Review

Objective. Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemic venous anomalies in patients with polysplenia and inferior vena cava (IVC) interruption who underwent a cavopulmonary anastomosis procedure at our center, and to review the published literature on this topic and the potential clinical importance of such anomalies. Design. Retrospective cohort study and literature review were used. Results. Among 77 patients with heterotaxy, univentricular heart disease, and IVC interruption who underwent a bidirectional Glenn and/or modified Fontan procedure, pulmonary arteriovenous malformations were diagnosed in 33 (43%). Bilateral superior vena cavas were present in 42 patients (55%). Despite inadequate imaging in many patients, a partial PSVC, dual IVCs, and/or renal vein anomalies were detected in 15 patients (19%). A PSVC formed by a tortuous vessel running from the systemic venous system to the extrahepatic portal vein was found in six patients (8%). Abdominal venous anomalies other than PSVC were documented in 13 patients (16%), including nine (12%) with some form of duplicated IVC system, with a large azygous vein continuing to the superior vena cava and a parallel, contralateral IVC of similar or smaller size, and seven with renal vein anomalies. In patients with a partial PSVC or a duplicate IVC that connected to the atrium, the abnormal connection allowed right‐to‐left shunting. Conclusions. PSVC and other abdominal venous anomalies may be clinically important but under‐recognized in patients with IVC interruption and univentricular heart disease. In such patients, preoperative evaluation of the abdominal systemic venous system may be valuable. More data are necessary to determine whether there is a pathophysiologic connection between the polysplenia variant of heterotaxy, PSVC, and cavopulmonary anastomosis‐associated pulmonary arteriovenous malformations.     

Cardiac catheterization through the internal jugular vein in pediatric patients. An alternative to the usual femoral vein access.

The percutaneous femoral vein approach is used routinely for cardiac catheterization in the pediatric age but in some children, it may be impossible as in the case of iliac vein or inferior vena cava thrombosis due to previous cardiac catheterization, or inconvenient as for right ventricular endomyocardial biopsies. In the period between 1982 and 1990, 160 cardiac catheterizations or right ventricular endomyocardial biopsies were performed in 102 children. Patients ranged in age between 2 months and 17 years (mean, 3.8 years) and in weight from 3.2 to 57.3 kg (mean, 14.4 kg). Indications for the internal jugular vein approach were as follows: (1) thrombosis of the inferior vena cava due to previous cardiac catheterization in 42 patients (41 percent); (2) right ventricular endomyocardial biopsy after cardiac transplant in 19 patients (19 percent); (3) control catheterization of the pulmonary arteries following classic or bidirectional cavopulmonary anastomosis in 16 patients (16 percent); (4) superior vena cava obstruction following Mustard's procedure in 14 patients (14 percent); (5) failed percutaneous femoral venous approach in six patients (6 percent); and (6) absence of the hepatic segment of the inferior vena cava in four patients (4 percent). The right or left internal jugular vein could be entered in all but three procedures (98 percent). Seventeen patients had more than one procedure through the same internal jugular vein and the vein was found patent in all. A complete right heart cardiac catheterization was performed using this route. Right ventricular endomyocardial biopsy and interventional procedure were performed through this route. Two major complications occurred. A patient developed a central transient ischemic attack and another patient developed a persistent Horner syndrome. Accidental carotid puncture occurred in five patients without consequences. Our data indicate that cardiac catheterization in infants and children can be performed safely through the internal jugular vein, with a high success rate and a low incidence of major complications.     

Evaluation of the complications of different types of right ventricular bypass by contrast echocardiography]

We evaluated 54 patients with different right heart by-pass by means of contrast echocardiography with rapid venous injection of shaken saline (3 cc, < 20 k; 6cc, > 20 k), in order to detect venous collateral circulation in partial by-pass, pulmonary arteriovenous fistulae in partial and total by-pass, and right-to-left shunt in total by-pass. Forty three patients had partial by-pass: 29 had a bidirectional cavopulmonary anastomosis with additional pulmonary flow (systemic-pulmonary anastomosis and/or restrictive anterograde ventricular flow); 10 had a partial ventricular correction (bidirectional cavopulmonary anastomosis and non restrictive anterograde ventricular flow; two had classical Glenn procedures; two had Kawashima operations (bidirectional cavopulmonary anastomosis and non restrictive anterograde ventricular flow; two had classical Glenn procedures; two had kawashima operations (bidirectional cavopulmonary anastomosis with inferior vena cava interruption), and 11 with total by-pass (10 atriopulmonary anastomosis and 1 with total cavopulmonary anastomosis). The age ranged from 2.5 to 33 years (x = 12.2 years), and the mean postoperative period was 4.3 years. Venous collateral circulation: 32/43 patients (74%) with partial by-pass, specially in those without partial biventricular correction: 29/33 patients (88%) compared to those with partial biventricular correction: 3/10 (30%) p < 0.01. Pulmonary arteriovenous fistulae: 6/43 patients (14%) with partial by-pass; 6/33 (18%) with partial biventricular correction, 0/10 patients with partial biventricular correction, and 0/11 with total by-pass. Right-to-left shunt: 2/11 patients (18%) with total by-pass, all of them with atriopulmonary anastomosis. Contrast echocardiography is an excellent noninvasive method for the initial evaluation of specific dysfunctions of different right heart by-pass. We emphasize the higher frequency of collateral venous circulation in cavopulmonary anastomosis, that would explain the dysfunction with progressive hypoxia in the evolution of these patients. Pulmonary arteriovenous fistulae were detected only in partial by-pass, without partial biventricular correction (p < 0.01). The minor frequency of these fistulae in bidirectional cavopulmonary anastomosis would be due to additional pulmonary flow.     

Peripheral and intracardiac levels of atrial natriuretic factor during cardiothoracic surgery.

Immunoreactive atrial natriuretic factor (ANF) levels were measured in blood taken from superior vena cava, right atrium, pulmonary artery, pulmonary vein, left atrium, and radial arteries in patients undergoing cardiac surgery. Significant (p less than 0.05) differences were seen between sites. Levels of atrial natriuretic factor were seen to rise from superior vena cava (27.5 pmol/L) to right atrium (54.3 pmol/L) and then fall in pulmonary artery (38.5 pmol/L). A further significant fall was seen in pulmonary vein (32.7 pmol/L) samples. There was no significant change in levels in left atrium (32.1 pmol/L) or radial artery (30 pmol/L). The fall between radial artery levels and superior vena caval levels was also significant. The rise from superior vena cava to right atrium is to be expected as this represents the major site of addition of atrial natriuretic factor to the circulation. We propose that the fall in levels from right atrium to pulmonary artery and from pulmonary artery to pulmonary vein suggests uptake and therefore possible local hormonal action on both right ventricle and pulmonary vasculature. The fall from radial artery to vena cava would be in keeping with atrial natriuretic factor's known systemic uptake and action.