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.     

The bidirectional Glenn operation in 100 cases with complex congenital heat diseases: factors influencing surgical results]

OBJECTIVE: The bidirectional Glenn shunt is a well established surgical technique in children with complex congenital heart disease. The present study is a retrospective analysis of patients undergoing this operation in order to assess the influence of different variables in the outcome. PATIENTS AND METHOD: From December 1990 to June 2000, 100 patients received a bidirectional Glenn shunt.Two groups were defined, based on the outcome. Group A (n = 15, unsuccessfully result) including death and need to reoperate, and Group B (n = 85, patients with good outcome). RESULTS: The mortality was 8%. Eight patients were reoperated at follow-up. The survivors were followed for a mean of 3.5 years. Mean pulmonary artery pressure 7 mmHg was a factor associated with poor clinical progress. Other variables (age less than 1 year, excessive pulmonary blood flow, double Glenn operation, significant anatomic anomalies, and arrythmias), were also associated with outcome. Significant variations were observed in the time of by-pass or the need for aortic clamp in cases with simultaneous operative repair of pulmonary branch stenosis. Actuarial survival rate, most more than 1 year was 92%, and freedom from reoperation at 3 years was 90%. CONCLUSIONS: The bidirectional Glenn shunt is an excellent palliation in patients with functionally single ventricle. Mean pulmonary artery pressure was the most important variable related with the outcome. We are encouraged to continue with tendency to perform bidirectional Glenn shunt preferably early, avoiding, whenever possible, previous palliative surgery.     

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.     

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.     

Treatment of elevated pulmonary artery pressure in a child after Glenn procedure: transcatheter closure of pulmonary artery banding with subsequent sildenafil therapy

An additional source of pulmonary blood flow in a patient with bidirectional Glenn procedure (BGD) may cause elevation of mean pulmonary artery pressure (MPAP), precluding safe completion of the Fontan operation. We present a case of single ventricle physiology after pulmonary artery banding (PAB) and Glenn procedure. At the age of six years, cardiac catheterisation revealed in the patient elevated MPAP (22 mm Hg). The PAB was closed through the right internal jugular vein with an Amplatzer Atrial Septal Occluder. After the procedure, MPAP remained at a similar level. Sildenafil oral therapy was applied for six months. Subsequent heart catheterisation confirmed complete closure of PAB and decrease of MPAP to 10 mm Hg. The abovementioned complex treatment of elevated MPAP pressure in a child after Glenn therapy allowed safe completion of the Fontan operation.     

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.     

Amplatzer occlusion of accessory ventriculopulmonary connections

Background: Children with complex congenital heart disease often require staged palliation to regulate systemic and pulmonary blood flow. Accessory sources of pulmonary blood flow including aortopulmonary collaterals, aortopulmonary shunts, and ventriculopulmonary connections following Glenn or Fontan palliation can be associated with elevated central venous pressures and persistent pleural drainage. Occlusion of accessory ventriculopulmonary connections in this setting has traditionally been accomplished surgically. Objective: To review the efficacy of Amplatzer devices in transcatheter occlusion of accessory ventriculopulmonary connections in children with complex congenital heart disease. Methods: Patients were identified and their records retrospectively reviewed for indication, procedural details, and clinical efficacy and outcome. Results: Between December 2004 and March 2008, seven patients underwent occlusion of accessory ventriculopulmonary connections using an Amplatzer Septal Occluder (3), an Amplatzer Duct Occluder (3), or an Amplatzer Vascular Plug (1). Underlying single ventricle physiology was present in six of these patients. The site of occlusion was the right ventricle to pulmonary artery (Sano) conduit in two patients, the native main pulmonary artery in three patients, the pulmonary valve in one patient, and a left ventricle to pulmonary artery homograft in one patient with biventricular physiology. There were no complications associated with these procedures. Conclusions: Amplatzer occlusion devices provide a safe and effective means of eliminating accessory ventriculopulmonary connections in children who have undergone surgical palliation of congenital heart disease. © 2008 Wiley‐Liss, Inc.     

Radiofrequency recanalization of an occluded long segment left pulmonary artery in bidirectional cavopulmonary anastomosis

In patients with hypoplastic left heart syndrome who experience the Norwood surgical palliative program, development of the pulmonary artery branches is crucial. Balloon dilation and stenting may relieve potential stenosis but complete occlusion remains difficult to address with transcatheter therapy. We report the use of radiofrequency followed by balloon dilation and stenting to treat a long segment left pulmonary artery occlusion in a 15‐month‐old boy bidirectional Glenn. The left pulmonary artery was successfully recanalized with an excellent short‐term outcome. This further enhances the interest of radiofrquency for recanalization of occluded pulmonary artery branches, as an alternative to surgical reconstruction. © 2009 Wiley‐Liss, Inc.     

Surgical Improvement of Hepatic Venous Mixing to Resolve Systemic Arterial Hypoxemia Associated with Post-Fontan Pulmonary Arteriovenous Fistulae

Pulmonary arteriovenous fistulae are known to develop in patients who have functional single-ventricle heart disease and interruption of the inferior vena cava with direct hepatic drainage to the heart, in which a bidirectional Glenn shunt is the only source of pulmonary blood flow. The progressive systemic arterial hypoxemia that is associated with pulmonary arteriovenous fistulae can have important clinical consequences. Baffling the hepatic venous return to the pulmonary circulation can alleviate pulmonary arteriovenous fistulae.Herein, we present the case of a 13-year-old patient with modified Fontan anatomy and pulmonary arteriovenous fistulae, in whom redirection of a previously placed hepatic venous-to-right pulmonary artery conduit was required in order to increase systemic arterial oxygen saturation. Revision of the conduit improved mixing of hepatic venous effluent with blood flow from the bidirectional Glenn shunt. Three years after this revision, the patient''s oxygen saturation remained stable at 90%, and his physical activity was markedly improved. We present our rationale for selected redirection of the conduit and discuss other surgical options that can improve hypoxemia that is associated with pulmonary arteriovenous fistulae.Key words: Arteriovenous fistula/physiopathology/surgery, arteriovenous malformations/etiology/surgery, Fontan procedure/adverse effects/methods, heart defects, congenital/surgery, hepatic veins/physiology/surgery, postoperative complications/etiology/physiopathology/surgery, vena cava, inferior/abnormalities/surgery, pulmonary artery/surgery, regional blood flow/physiology, reoperationPulmonary arteriovenous fistula (PAVF) can develop in patients who have undergone placement of a bidirectional Glenn shunt for single-ventricle heart disease that is associated with interruption of the inferior vena cava (IVC) and direct hepatic venous drainage to the heart.^1,2 The progressive systemic arterial hypoxemia that is associated with PAVF can have important clinical manifestations. It has been reported that baffling hepatic venous return to the pulmonary circulation can alleviate PAVF. Here, we present and discuss the case of a 13-year-old modified-Fontan patient with PAVF, in whom redirection of a previously placed hepatic venous-to-right pulmonary artery (PA) conduit was required in order to overcome unfavorable streaming and to increase systemic arterial oxygen saturation levels.     

A novel use of the Amplatzer muscular ventricular septal defect occluder.

We report a case of a 12-month-old-infant with double outlet right ventricle and pulmonary stenosis who presented with signs of superior vena cava syndrome secondary to a dysfunctioning bidirectional Glenn shunt. The patient was successfully treated with transcatheter obstruction of an accessory pulmonary blood flow using the Amplatzer muscular ventricular septal defect occluder.     

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.     

Novel method for delivering the Amplatzer® muscular VSD occluder in a patient with double outlet right ventricle after bidirectional Glenn procedure and pulmonary artery band

We report the first use of bilateral femoral venovenous rail creation for the delivery of an Amplatzer® Muscular Ventricular Septal Defect Occluder in a patient with a large mid‐to‐apical muscular ventricular septal defect before Rastelli operation. The presence of a right‐sided bidirectional Glenn shunt, a banded main pulmonary artery, and double outlet right ventricle anatomy precluded the use of standard delivery techniques. The patient underwent successful transcatheter device placement followed by Rastelli operation on the following day. © 2009 Wiley‐Liss, Inc.     

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。     

Longevity of Neonatal Ductal Stenting for Congenital Heart Diseases with Duct‐dependent Pulmonary Circulation

Introduction. Ductal stent (DS) in duct‐dependent pulmonary circulation is less morbid than neonatal Blalock–Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair. Methods. This is a retrospective review of clinical follow‐up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion. Results. Among 22 infants, four Group A patients followed for 26–54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short‐term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5–14 months at a body weight of 5–7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6–8.5 kg after 8–15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow‐up for hypoxia. Four patients had sudden death. Conclusions. The short‐term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%.     

Transcatheter left ventricular outflow tract stent placement in a low birth weight child with hypoplastic left ventricle,mitral atresia,transposition of the great arteries,ventricular septal defect and severe pulmonary stenosis

A baby boy, the first of triplets born at 33 weeks gestation weighing 1.88 kg, presented with neonatal cyanosis and was diagnosed with mitral atresia, hypoplastic left ventricle, ventricular septal defect, d‐transposition of the great arteries, severe pulmonary stenosis, and hypoplastic branch pulmonary arteries. He was treated with prostaglandin until seven weeks of age. The patent ductus arteriosus was curly Q and not suitable for stent placement. Cardiac catheterization was undertaken and using an antegrade approach, a Multi‐Link Ultra stent was implanted across the left ventricular outflow tract (LVOT) and inflated to 5 mm to improve antegrade pulmonary blood flow. This allowed reasonable pulmonary arterial growth to allow the patient undergo bidirectional Glenn shunt at five months of age. This first report of LVOT stenting in this setting may provide an alternative to placement of a systemic to pulmonary arterial shunt when ductal stenting is not possible. © 2013 Wiley Periodicals, Inc.     

An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

The differential diagnosis for irritability in children is broad. In patients with congenital heart disease, one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure (CVP). In patients with single-ventricle physiology, the second stage of palliation includes bidirectional Glenn, which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle. Typically, early in the post-operative period, patients may experience a headache due to the acute increase in CVP, and symptoms improve over time. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a rare neurologic disorder in children, characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesions or cerebrospinal fluid (CSF) abnormalities. While the pathogenesis of IIH is unknown, early recognition and treatment of IIH are important to prevent permanent vision loss. There are only rare reports of IIH in patients with Fontan circulation. To our knowledge, we report the first case of IIH in a 2-year-old female after bidirectional Glenn.     

Single-stage versus two-stage modified Fontan procedure

We compared surgical outcomes of the single-stage and two-stage modified Fontan procedures to clarify clinical superiority. Of 28 children undergoing a modified Fontan procedure from October 1995 to October 2005, 15 had a 1-stage and 13 had a 2-stage operation. In the 2-stage group, pulmonary artery growth was evaluated before and after the first stage. Operative mortality was 26.6% in the 1-stage group and 0% in the 2-stage group. The benefits of a previous bidirectional Glenn shunt were decreased cyanosis and volume overload, but there was no significant difference in pulmonary artery growth reflected in pulmonary artery indices before and after the bidirectional Glenn procedure. Older children underwent a 2-stage modified Fontan procedure and had better outcomes in terms of lower mortality, improved oxygen saturation, decreased volume load, and less deterioration of atrioventricular valve regurgitation.     

Successful surgical repair of Uhl's anomaly

We report a case of Uhl's anomaly in a 5-month-old cyanotic infant who presented with thromboembolic stroke and acute hemiparesis. The patient underwent successfully an initial surgical repair, which included exclusion of the right ventricle by patch closure of the tricuspid valve, atrial septectomy and construction of a bidirectional Glenn shunt. This was followed by successful construction of a total cavopulmonary connection.