Asian Cardiovasc Thorac Ann 2006;14:367-370
© 2006 Asia Publishing EXchange Ltd
Outcome of Single Ventricle and Total Anomalous Pulmonary Venous Connection
Manvinder S Sachdev, MD,
Pradyumna K Jena,
Renu P Kurup, DNB,
Roy Varghese, MCh,
R Suresh Kumar, DM,
Robert Coelho, MCh
Division of Pediatric Cardiology and Cardiac Surgery, Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India
For reprint information contact: Manvinder S Sachdev, MD Tel: 91 93 1341 9494 Fax: 91 11 2692 5858 Email: msachdev40{at}yahoo.com, E-86, Sarita vihar, New Delhi 110044, India.
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ABSTRACT
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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.
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INTRODUCTION
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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. The majority of patients with complex single-ventricle physiology and pulmonary venous anomalies have heterotaxy syndrome with atrial isomerism, which may contribute to an adverse outcome.1 We describe our experience over the past decade with management of anomalous pulmonary venous return in patients undergoing single-ventricle palliation. It has been the policy at our institute to manage patients with single ventricle by staged Fontan repair; the primary palliation and construction of a bidirectional Glenn shunt is performed with this in mind.2 Our strategy is to carry out an extracardiac conduit Fontan operation that apart from its other advantages can be applied in patients with the full range of pulmonary venous anomalies, with little or no modification.
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PATIENTS AND METHODS
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Twenty-five patients with pulmonary venous anomalies and single ventricle underwent staged palliation at our institution from June 1996 to May 2005. Visceral heterotaxy with atrial isomerism was present in 19 patients (76%); 15 had asplenia with right atrial isomerism, and 4 had polysplenia with left atrial isomerism. Other primary diagnoses were hypoplastic left heart syndrome and its variants in 2 patients, tricuspid atresia in 2, and other forms of complex single ventricle in 2. Patients with anomalous pulmonary venous return were further categorized as: anomalous but not requiring special intervention at any stage; and anomalous but requiring intervention at some stage of management. Our approach is an extracardiac Fontan operation in all patients with single ventricle, thus anomalies of pulmonary venous return not requiring intervention include all forms of cardiac anomalous drainage, such as drainage to the middle or systemic side of the common atrium. Most forms of high supracardiac and infracardiac drainage and all cases of obstructed anomalous pulmonary venous drainage require intervention. The types of total anomalous pulmonary venous connection (TAPVC) are shown in Table 1
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RESULTS
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Fifteen patients had primary palliation with a systemic-to-pulmonary artery shunt. Ten patients underwent repair of TAPVC at the primary palliation, all of them had been diagnosed echocardiographically with pulmonary venous obstruction. In the 6 patients with obstructed pulmonary venous return to the systemic vein, standard techniques of redirecting pulmonary venous return to the left atrium were performed. In 2 of these patients, TAPVC correction was performed first, followed by placement of a systemic-to-pulmonary artery shunt 4 months later. In patients with hypoplastic left heart syndrome, a stage 1 Norwood procedure was undertaken; both of these patients had supracardiac TAPVC via the vertical vein, which was ligated at the time of the stage 1 Norwood operation.
There were 5 early deaths after primary palliation, 4 of them occurred in patients with obstruction of pulmonary venous return. Two of the early deaths were in patients with hypoplastic left heart syndrome, 2 were in patients with right atrial isomerism, and the other was in a patient with left isomerism. Three of the patients who had primary palliation have not undergone further stages of palliation.
A bidirectional Glenn shunt was constructed in 17 patients, including 10 in whom it was performed as a primary palliative procedure. In 5 patients with unobstructed anomalous pulmonary return to the middle or systemic side of the common atrium, nothing was done to modify the TAPVC. In 3 patients with anomalous pulmonary return to the right superior caval vein, the superior caval vein was divided above the drainage site of the pulmonary venous return for a Glenn shunt, and the stump was oversewn. In 2 of these patients, we encountered pulmonary venous obstruction: one immediately postoperatively necessitating revision of the anastomosis and redirection of the pulmonary venous return to the left atrium directly; the other patient was asymptomatic postoperatively and was diagnosed at postoperative echocardiography. This patient underwent redirection of pulmonary venous return to the left atrium one month after the initial surgery, but subsequently died due to low cardiac output. In 3 patients with supracardiac or infracardiac drainage to the systemic vein, standard techniques of directing pulmonary venous return to the left atrium were performed. There was no mortality in this group. In 2 patients with drainage via the vertical vein, the vertical vein was ligated.
There were 7 early deaths and no late death after the bidirectional Glenn procedure: in 6 patients with heterotaxy and atrial isomerism, and in 1 patient with a complex form of single ventricle. Only one death was caused by pulmonary venous obstruction, the others were due to septicemia with gram-negative bacilli.
Five patients have undergone completion of the Fontan procedure, 4 of them had an extracardiac Fontan conduit and one had a lateral tunnel. There was only one early death after the Fontan operation in a patient with right isomerism. This patient did not have any intervention for anomalous pulmonary venous return to the common atrium, but later developed signs of pulmonary venous obstruction. She underwent re-operation for relief of pulmonary venous obstruction, but subsequently died on the 7th postoperative day due to low cardiac output.
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DISCUSSION
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Staged palliation culminating in Fontan physiology is becoming the procedure of choice in patients with a wide variety of complex single-ventricle anatomy, including those with pulmonary venous anomalies. Although some reports have addressed the issue of managing TAPVC at primary palliation, bidirectional Glenn shunt, and the Fontan operation, there are few regarding the overall influence of pulmonary venous anomalies on outcomes of staged palliation.1–7 Typically, single-ventricle palliation involves placing a systemic-to-pulmonary artery shunt at the first stage, followed by a bidirectional Glenn shunt in mid-infancy, and finally, Fontan completion after several years. The aim of any management strategy is to optimize the circulation in preparation for the ultimate Fontan operation. This is especially the case when pulmonary venous anomalies are present because first- and second-stage procedures facilitate straightforward techniques for managing TAPVC before the Fontan operation.
Most of the anomalous pulmonary venous return in patients with single ventricle is unobstructed, nevertheless pulmonary venous return should be evaluated completely with preoperative echocardiography which is adequate to diagnose almost all cases of TAPVC. In the setting of reduced pulmonary blood flow, diagnosis of obstruction may be difficult; but with increasing use of prostaglandins in the neonatal period, such obstruction can be easily picked up. Also, some forms of TAPVC, such as infradiaphragmatic return, are always obstructed and must be assumed so when diagnosed. When obstruction is present, it is imperative to correct the anomalous pulmonary venous return on an emergency basis. Early mortality after neonatal palliation in heterotaxy syndrome with TAPVC was 26% in a 10-year series at Boston Children’s Hospital, and substantially higher in other reports.1,3,8,9 In most studies, mortality was higher in patients with obstruction. In the present study also, pulmonary venous obstruction significantly complicated the procedure, and all early deaths were related to obstruction. This is true across the complete spectrum of anatomic diagnoses, irrespective of visceral heterotaxy. In patients with reduced pulmonary blood flow, increasing flow to the lungs by shunt placement might unmask the obstruction to pulmonary venous return.3,7,10 Thus, it is of the utmost importance to evaluate pulmonary venous anomalies after procedures to increase pulmonary blood flow. In several series, the presence of pulmonary outflow obstruction in patients with heterotaxy and anomalous pulmonary venous return was associated with higher mortality after primary palliation.1,3
A bidirectional Glenn shunt is most often instituted as the second stage of palliation, but it can be employed as a primary palliative procedure.11 This operation also provides a convenient opportunity to manage TAPVC. In this study, an increased risk of death was found in patients with single ventricle and TAPVC undergoing a bidirectional Glenn shunt, in contrast to the findings of McElhinney and colleagues.12 When a bidirectional Glenn shunt is undertaken in patients with pulmonary venous drainage just above the superior cavoatrial junction, the superior caval vein is transected more cranially than usual, ensuring sufficient room above the entrance of the pulmonary veins to avoid obstruction. Alternatively, the pulmonary venous confluence can be directly connected to the left atrium. In 2 of the 3 patients undergoing repair by the former method, we found evidence of postoperative obstruction, which could have resulted from retraction of the transected end of the superior vena cava. This might be avoided if the pulmonary venous confluence was directly anastomosed to the left atrium. In view of the small number of patients undergoing this type of repair, it is difficult to generalize these results. With other forms of extracardiac anomalous pulmonary venous drainage, various techniques can be used to achieve pulmonary venoatrial connection, including anastomosis of the vertical vein to the cardiac stump of the transected superior vena cava or to either atrial appendage. It is important to remember that when patients with mildly reduced pulmonary blood flow undergo a bidirectional Glenn procedure as primary palliation, this will further reduce the pulmonary blood flow, and potential mild pulmonary venous obstruction may remain masked until Fontan completion.
For the Fontan procedure, it is our institutional protocol to create an extracardiac conduit whenever possible, so almost all cases of primary palliation and establishment of a bidirectional Glenn shunt are undertaken with the ultimate goal of an extracardiac Fontan. The extracardiac Fontan operation is better suited to hearts with complex single ventricle with TAPVC. As the right atrium is excluded from the circuit in an extracardiac Fontan, the common atrium receives all the pulmonary venous blood flow. If required, atrial septectomy can be performed at primary palliation or during the bidirectional Glenn procedure.
It was concluded from this experience that anomalous pulmonary venous return can significantly complicate the management of patients with single ventricle. Some form of pulmonary venous anomaly occurs in more than 60% of patients with right isomerism and half of the patients with left isomerism.13–15 Anomalous pulmonary venous return is present in 6% of patients with hypoplastic left heart syndrome17, including those in whom a levoatriocardinal vein provides egress of pulmonary venous blood to the systemic veins.5,16 Thus, TAPVC remains a substantial problem with a major impact on survival in early infancy. Palliation with the aim of performing an extracardiac conduit Fontan allows greater latitude and more streamlined management of this subgroup of patients.
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ABSTRACT
INTRODUCTION
