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     

Outcome of single ventricle and total anomalous pulmonary venous connection

Management of patients with single-ventricle physiology is significantly affected by anomalies of pulmonary venous return at all stages, whether primary palliation, bidirectional Glenn shunt, or completion of Fontan circulation. We treated 25 patients with pulmonary venous anomalies and single ventricle by staged palliation, from June 1996 to May 2005. Visceral heterotaxy with atrial isomerism was present in 19 of them. Primary palliation with a systemic-to-pulmonary artery shunt was undertaken in 15 patients. There were 5 early deaths, of which 4 were due to obstruction of pulmonary venous return. A bidirectional Glenn shunt was constructed in 17 patients including 10 who had it as a primary palliative procedure. There were 7 early deaths after the bidirectional Glenn procedure; only one was due to pulmonary venous obstruction. Five patients attained completion of the Fontan procedure. There was one early death after the Fontan operation. Anomalous pulmonary venous return can significantly complicate the management of patients with single ventricle, with an impact on survival in early infancy. Palliation with the aim of instituting extracardiac conduit Fontan circulation allows greater latitude and more streamlined management.     

Catheter closure of accessory pulmonary blood flow after bidirectional Glenn anastomosis using Amplatzer duct occluder.

Additional source of pulmonary blood flow in patients with bidirectional cavopulmonary anastomosis (Glenn shunt) may cause elevation of the pulmonary artery pressure precluding safe completion of the Fontan operation. A case is presented with single-ventricle bidirectional cavopulmonary anastomosis and additional flow from the ventricle to the pulmonary artery resulting in elevated Glenn pressure. The communication was successfully occluded using Amplatzer duct occluder with satisfactory reduction in the Glenn pressure.     

Transcatheter creation of an atriopulmonary communication in the Hemi-Fontan or Glenn circulation

There have been several modifications of the Fontan procedure, which has been used for palliation of the functionally univentricular circulation since its conception in the early 1970s. The development of intrahepatic veno-venous fistulas, pulmonary arteriovenous fistulas, and communications from the hepatic vein to the pulmonary venous atrium, are some of the complications encountered by patients with cavo-pulmonary communications. We describe transcatheter creation of an atriopulmonary communication, with simultaneous implantation of a stent, as a method of reducing or preventing pulmonary arteriovenous fistulas and associated right-to-left shunting in this setting. The intervention has been undertaken in two patients following a Hemi-Fontan procedure, itself created subsequent to a Glenn anastomosis. The combination of these procedures creates communicating atriopulmonary and cavopulmonary circulations.     

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.     

Bidirectional cavopulmonary anastomosis in congenital heart disease. Functional and clinical outcome.

The bidirectional cavopulmonary anastomosis is a surgical procedure suitable for patients with cyanotic congenital heart disease and univentricular physiology. This operation is able to increase the effective pulmonary blood flow without any additional load on the cardiac work and without any further distortion on the pulmonary artery branches. The cavopulmonary anastomosis can represent the first stage for patients destined for Fontan repair or a definitive palliative operation in high risk Fontan candidates. In order to test the hypothesis of a definitive palliation by cavopulmonary anastomosis in this kind of patients, we evaluated the hemodynamic data before and after this surgical approach and compared these data with their clinical and functional outcome. We evaluated 74 patients submitted to bidirectional cavopulmonary anastomosis by either hemodynamic or functional evaluation. End-diastolic and end-systolic ventricular volumes were significantly reduced by bidirectional cavopulmonary anastomosis (p less than 0.0005). Despite these data and a normal ambulatory ECG, spirometry and echocardiographic analysis, the stress test showed discouraging results. In fact, mean work time and peak heart rate were significantly different from normal values showing an impaired functional capacity of these children. In conclusion we think that bidirectional cavopulmonary anastomosis can not be considered an adequate definitive palliation but it represents a very good stage to preserve the pulmonary arteries and to prepare the systemic ventricle towards the Fontan repair.     

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.     

单心室合并完全性肺静脉异位引流的外科治疗

目的:探讨单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:2009-05至2011-09,我科共收治单心室合并TAPVC患儿11例,年龄(30.1±21.4)个月,体重(11.0±3.2)kg。例2行改良B-T分流术,例1、3～6、8、9、11行一侧或双侧双向腔肺分流术,例7、10行一期改良Fontan术。例1、6～9、11同期行TAPVC矫治术。结果:例6、10围术期死亡(18.2%,2/11),死亡原因分别为中枢神经系统感染和多器官功能衰竭。存活的患儿随访1～29个月,例4死亡,余患儿氧饱和度改善,未发现残余肺静脉梗阻,例5行二期改良Fontan术并顺利出院。结论:单心室合并TAPVC患儿仍具有较高早期死亡率,术前明确诊断TAPVC并制定合理的个体化治疗方案是提高手术疗效的关键。     

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.     

A case of total left anomalous pulmonary venous connection with intact atrial septum diagnosed by two-dimensional and Doppler echocardiography

Unilateral total anomalous pulmonary venous connection from a unilateral lung is extremely rare. A 6-year-old patient with anomalous pulmonary venous connection from the entire left lung to the left innominate vein, with an intact atrial septum, diagnosed by two-dimensional and Doppler echocardiography is reported. The combination of two-dimensional and Doppler echocardiography is very useful not only for anatomical diagnosis, but also for evaluation of its hemodynamics, despite the wide anatomical variability of the pulmonary venous connection. This is the first report of a case of left total anomalous pulmonary venous connection diagnosed noninvasively.     

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.     

Total Anomalous Pulmonary Venous Connection in an Adult: Comprehensive Multimodality Evaluation

An unusual case of total anomalous pulmonary venous connection surviving to adulthood without surgical correction is presented. Transthoracic echocardiography first led to this diagnosis and magnetic resonance imaging refined the anatomic diagnosis leading to successful surgical correction.     

De Vega tricuspid annuloplasty for systemic tricuspid regurgitation in children with univentricular physiology

BACKGROUND AND AIM OF THE STUDY: Significant tricuspid valve regurgitation (TR) is problematic in children with univentricular physiology and a systemic tricuspid valve occasionally requiring tricuspid (systemic atrioventricular) valve replacement. Since 1998, the De Vega tricuspid annuloplasty technique has been applied for TR in these children. METHODS: Twelve children (median age 2.2 years; range: 6 months to 17 years) with moderate or severe systemic TR underwent a De Vega tricuspid annuloplasty during a bidirectional Glenn anastomosis (n = 3), Fontan procedure (n = 8) or aortic valve replacement late after a Fontan procedure (n = 1). Nine patients (75%) had prior Norwood palliation for hypoplastic left heart syndrome. Four patients had simultaneous repair of an abnormal tricuspid valve in addition to the De Vega procedure. RESULTS: There were no deaths during a mean follow up of 2.0 +/- 1.4 years (range: 6 months to 5.1 years). One child required pacemaker implantation early after operation, and one child with a Glenn anastomosis underwent cardiac transplantation 21 months postoperatively. In the remaining 11 patients, the most recent echocardiogram showed mild or no TR in eight children, mild-to-moderate TR in one child, and moderate TR in two children. No child had symptomatic TR (including the two with moderate TR), significant tricuspid stenosis, or late pacemaker implantation. CONCLUSION: The De Vega tricuspid annuloplasty safely provides excellent relief of systemic TR in children with univentricular physiology, with a majority of patients (73%) having mild or less residual TR at follow up examination. This simple technique is preferred to tricuspid (systemic) valve replacement in these children.     

Surgical outcome of staged univentricular-type repairs for patients with univentricular physiology and pulmonary hypertension

BACKGROUND: There is no consensus about the most appropriate limits of pulmonary artery pressure and vascular resistance in case of patients undergoing univentricular or one and one-half ventricular repair. This study was conducted to analyze the mortality and morbidity of a heterogenous group of patients with a functionally univentricular heart and pulmonary artery hypertension, undergoing pulmonary artery banding followed by univentricular-type repairs. METHODS AND RESULTS: Out of 254 patients undergoing pulmonary artery banding for a functionally univentricular heart with increased pulmonary blood flow, 148 patients underwent definitive second stage surgery. Post-band hemodynamic evaluation revealed persistently high pulmonary artery pressure (> 18 mmHg), and pulmonary vascular resistance (>2.0 Woods units/m2) in 78.3% patients. Sixteen patients with moderate right ventricular hypoplasia were given a one and one-half ventricle repair (Group I), 82 patients a bidirectional Glenn connection (Group II), and 50 patients a fenestrated total cavopulmonary connection (Group III). The overall mortality following second stage surgery for the high pulmonary artery pressure group (n=116) was 30.17%, while none of the low pulmonary artery pressure group died (p=0.0009). Pulmonary hypertensive crises and/or systemic desaturation were the main causes of death at second stage repair. All mortality occurred in patients with mean pulmonary artery pressure > 18 mmHg and pulmonary vascular resistance > 3.5 Woods units/m2. Survivors from this group had persistent morbidity in the form of superior vena caval syndrome and suboptimal oxygen saturation (70-75%). CONCLUSIONS: It is advisable not to proceed with definitive second stage repair if post-pulmonary artery banding mean pulmonary artery pressure is over 25 mmHg and pulmonary vascular resistance exceeds 4.0 Woods units/m2. These patients may possibly be deemed to have undergone definitive palliation during their pulmonary artery banding.     

Catheter conversion of classic glenn to bidirectional glenn with closure of left central shunt years after surgical attempts

Bidirectional Glenn shunt is usually performed in patients with single ventricle in preparation for a total cavo‐pulmonary connection. We present a patient born with complex congenital heart disease consisting of single ventricle, pulmonary atresia, non confluent pulmonary arteries, and anomalous pulmonary venous return in whom surgical bidirectional Glenn was attempted. After multiple surgical attempts she was converted to a Classic Glenn and a central ascending aorta to left pulmonary artery shunt. Several years later by the aid of radiofrequency wire the occluded pulmonary artery segment was canalized establishing continuity between the two pulmonary artery branches with stenting of the intervening segment. The central shunt to the left pulmonary artery was subsequently embolized. Thus this patient was converted in the catheterization laboratory from the physiology of a classic Glenn to the more preferred bidirectional Glenn physiology. © 2011 Wiley Periodicals, Inc.     

Is the "perfect Fontan" operation routinely achievable in the modern era?

OBJECTIVE: In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called "Perfect Fontan" operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a "perfect" outcome is routinely achievable in the current era when using a standardized surgical procedure. METHODS: Between 1 January, 1988, and 12 December, 2005, 112 patients underwent the Fontan procedure using an internal or external polytetraflouroethylene tube plus a bidirectional cava-pulmonary connection, the latter usually having been constructed as a previous procedure. This constituted 45% of our overall experience in constructing the Fontan circulation between 1988 and 1996, and 96% of the experience between 1996 and 2005. Among all surviving patients, the median follow-up was 7.3 years. We calculated the expected survival for an optimal candidate, given from the initial equations, and compared this to our entire experience in constructing the Fontan circulation. RESULTS: An internal tube was utilized in 61 patients, 97% of whom were operated prior to 1998, and an external tube in 51 patients, the latter accounting for 95% of all operations since 1999. At 1, 5, 10 and 15 years, survival of the entire cohort receiving polytetraflouroethylene tubes is superimposable on the curve calculated for a "perfect" outcome. Freedom from replacement or revision of the tube was 97% at 10 years.CONCLUSION: Using a standardized operative procedure, combining a bidirectional cavopulmonary connection with a polytetraflouroethylene tube placed from the inferior caval vein to the pulmonary arteries for nearly all patients with functionally univentricular hearts, early and late survival within the "perfect" outcome as predicted by the initial equations of Fontan and Kirklin is routinely achievable in the current era. The need for late revision or replacement of the tube is rare.     

Total anomalous pulmonary venous connection beyond infancy

Most patients with total anomalous pulmonary venous connection are operated on in infancy, with low mortality and morbidity, but in developing countries, we still encounter patients beyond infancy. We describe our experience in 26 patients aged 1-16 years (mean, 5.01 years), with total anomalous pulmonary venous connection, who underwent correction between June 2007 and December 2009. Eleven patients were >5-years old. Transthoracic echocardiography was diagnostic in all cases. Mean intensive care unit stay was 2.3 ± 0.87 days, and hospital stay was 9.23 ± 2.34 days. There were no early deaths and no major postoperative complications. Follow-up ranged from 10-40 months. Pulmonary artery pressure, as judged by echocardiography, reduced significantly in all patients. Repair of total anomalous pulmonary venous connection beyond infancy can be carried out safely with acceptable results.     

Univentricular heart and pulmonary stenosis with a right-to-left shunt between the innominate and pulmonary veins after Glenn operation: a case report

A 12-year-old girl with a univentricular heart and pulmonary stenosis, who had undergone Glenn operation at two years of age and Fontan operation at 11 years of age, had a right-to-left shunt via collaterals between the innominate and pulmonary veins. This right-to-left shunt was detected by two-dimensional contrast echocardiography. The contrast material was injected into the left antecubital vein appeared in the left atrium, and this was confirmed by innominate venography. The contrast material injected into the left innominate vein passed via the collaterals into the pulmonary veins bilaterally. No such cases have so far been reported. In our experience, in four of six cases after Glenn operation, including the present case, the abnormal collateral circulation from the superior vena cava to the atrium was detected by two-dimensional contrast echocardiography. This case is interesting embryologically and suggests that the connection between the pulmonary and systemic veins which is present in early fetal life, and subsequently disappears, was present after birth.     

Total anomalous pulmonary venous connection to azygos vein: echocardiographic recognition of a rare variant

Total anomalous pulmonary venous connection to the azygos vein is a rare congenital malformation in which all the pulmonary veins drain anomalously to the azygous vein. Among 55 consecutive patients diagnosed as isolated total anomalous pulmonary venous connection by echocardiography at our institute between 1995 and January 2005, this anomaly was diagnosed in two patients. The malformation was diagnosed correctly in both the patients by echocardiography and the findings were confirmed on surgery. The importance of differentiating this condition from total anomalous pulmonary venous connection to superior vena cava by echocardiography and the differential diagnosis are discussed here.     

心外管道全腔肺动脉连接术在复杂先心病中的应用

目的：总结心外管道全腔肺动脉连接（total cavopulmonary connection,TCPC）术治疗复杂先天性心脏病的临床经验。方法：2002年1月～2011年5月,采用外管道TCPC术矫治复杂先天性心脏病47例（病种包括三尖瓣闭锁、单心室、肺动脉闭锁、右心室发育不良、右室双出口）患者,手术年龄（8±4）岁。一期手术25例,分期手术22例。结果：术后早期（术后1月）死亡4例（8%）。死亡原因：多脏器功能衰竭、心脏骤停、感染、蛋白丢失性肠病和低心排。术后早期并发症发生率为17%,主要为肺水肿3例、胸腔积液2例、心律失常1例、心包积液1例、蛋白丢失性肠病和低心排1例。术后均随访,随访时间9～87月。远期再住院率为9%（4/43）,再次手术7%（3/43）。2例再次出现胸腔积液,2例因自行停服肠溶阿司匹林后出现外管道堵塞再次手术,1例于术后出现低心排死亡。其余患者恢复良好。结论：对于适应证明确的复杂先天性心脏病患者行心外管道TCPC手术治疗,近期临床效果满意。