Ann Thorac Surg 2006;82:978-982
© 2006 The Society of Thoracic Surgeons
Original article: Cardiovascular
Partial Anomalous Pulmonary Venous Connection to the Superior Vena Cava
Atsushi Nakahira, MDa,
Toshikatsu Yagihara, MDa,*,
Koji Kagisaki, MDa,
Ikuo Hagino, MDa,
Toru Ishizaka, MDa,
Masahiro Koh, MDa,
Hideki Uemura, MDb,
Soichiro Kitamura, MDa
a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Department of Cardio-Thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
Accepted for publication February 4, 2006.
* Address correspondence to Dr Yagihara, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1, Fujishirodai, Suita, Osaka, 565-8565, Japan (Email: yagihara{at}hsp.ncvc.go.jp).
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Abstract
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BACKGROUND: Repair of partial anomalous pulmonary venous connection (PAPVC) to the high portion of the superior vena cava (SVC) may be complicated by atrial arrhythmia and obstruction of the pulmonary veins or SVC. We reviewed our experience with the modified Warden technique, in which the SVC was transected and anastomosed to the right atrial appendage with anterior augmentation of pedicled autologous pericardial flap, and the atrial septum was directly displaced to the SVC orifice.
METHODS: Twenty of 51 patients with PAPVC underwent this technique. Mean age was 11.9 years. Follow-up averaged 6.5 years. To quantify the height of insertion of anomalous pulmonary veins, the distance between the highest anomalous pulmonary venous orifice and SVC-right atrial junction was indexed by thoracic vertebral body height (height index).
RESULTS: All patients are alive in sinus rhythm. No patients exhibited pulmonary venous obstruction, and mean flow was 0.61 mL. Mean flow of SVC return was 0.79 mL. The SVC occlusion occurred in 2 patients who had persistent left SVC with a good communicating vein. Three patients whose height index exceeded 2.5 successfully underwent catheter intervention at the SVC channel.
CONCLUSIONS: Midterm results with the modified Warden technique were satisfactory. Patients with particularly high insertion of anomalous pulmonary veins should be treated and followed with specific caution for preserving an unobstructed caval pathway.
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Introduction
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Partial anomalous pulmonary venous connection (PAPVC) is a condition in which some, but not all, pulmonary veins connect to the right atrium or its tributaries, rather than to the left atrium. The PAPVC may occur as an isolated anomaly or may be combined with atrial septal defect (ASD). The PAPVC to the superior vena cava (SVC) occurs in about 10% to 15% of all patients with ASD. Most common, one or more pulmonary veins from the right lung connect to the SVC or right atrium, or both (Fig 1A). There have been several reports on operative techniques for PAPVC and findings of follow-up. Repair of PAPVC to the high portion of the SVC may be complicated by atrial arrhythmia and obstruction of the pulmonary veins or SVC. In 1984, Warden and colleagues [1] reported a technique in which the SVC was divided, the cephalic SVC was anastomosed to the right atrial appendage, and the caudal SVC served as a conduit for pulmonary venous drainage to the left atrium (Fig 1B). We adopted and modified the Warden technique with anterior wall augmentation of the SVC drainage channel using pedicled autologous pericardial flap and atrial septal displacement for the pulmonary venous drainage channel without use of artificial products (Fig 1C). We reviewed the midterm results of use of our technique. The height of insertion of the anomalous pulmonary veins was evaluated by preoperative angiography.
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Fig 1. Schemata of partial anomalous pulmonary venous connections to the high portion of the superior vena cava (A), repair using the Warden method (B), and modified Warden method with pedicled autologous pericardial flap as an anterior augmentation of the SVC channel (C). (ASD = atrial septal defect; LA = left atrium; RA = right atrium; SVC = superior vena cava.)
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Patients and Methods
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Patient Population
Between 1993 and 2004, 51 patients with PAPVC underwent repair at the National Cardiovascular Center, Japan. Twenty of these patients (39%) had high insertion of anomalous pulmonary veins to the SVC and were treated with the modified Warden technique, including SVC translocation and atrial septal displacement. The 10 male and 10 female patients ranged in age from 4 to 26 years (mean, 11.8 ± 7.0 years [standard deviation]). Weight ranged from 15.0 to 44.7 kg (mean, 34.2 ± 15.5 kg). No patients had isomerism heart and none had documented arrhythmia preoperatively. This retrospective review was approved by the Ethics Committee in our institution. Informed consent was obtained from all 20 patients and their legal guardians as required prior to undertaking this retrospective analysis.
Preoperative Angiographic Data
Cardiac catheterization was performed in all patients before operation. Pulmonary blood flow was increased in all children, with a mean pulmonary-systemic blood flow ratio (Qp/Qs ratio) of 2.70 ± 0.70. The mean pulmonary vascular resistance was 1.06 ± 0.26 units x M2, and the mean pulmonary arterial pressure was 15.5 ± 3.2 mm Hg. The mean number of anomalous pulmonary veins was 2.25 (1 to 4), and the number of veins from the upper lobe was a mean of 2.1 (1 to 4). Persistent left SVC drained into the coronary sinus in 2 patients (10%), in both of whom the left SVC was larger than the right SVC, with a good communicating vein present between them (Fig 2A). Left pulmonary venous drainage was normal in all patients.
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Fig 2. Schemata of preoperative (A) and postoperative (B) findings for the 2 patients with SVC obstruction who had persistent left SVC, which was larger than the right SVC with a good communicating vein. (LA = left atrium; PLSVC = persistent left superior vena cava; RA = right atrium.)
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Follow-Up
Follow-up of the patients has extended from 1.2 to 12 years (mean, 6.5 ± 3.0 years). Postoperative cardiac catheterization was performed in all children about 1 year postoperatively, and the patients have been followed consecutively with echocardiography.
Definition of Height Index
For quantitative evaluation of the height of insertion of the anomalous pulmonary veins, we defined and measured a height index, equal to the length between the cephalic end of the highest anomalous pulmonary venous orifice and SVC-right atrium junction divided by the height of the thoracic vertebral body at the same level as the junction (Fig 3). Preoperative angiographic images included all information needed to calculate the index.
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Fig 3. Height index is defined as the length (X) between the cephalic end of the highest anomalous pulmonary venous orifice and the SVC-RA junction divided by the height (Y) of the vertebra at the same level as the junction, and can be determined using the preoperative catheterization images. (SVC = superior vena cava; RA = right atrium.)
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Surgical Technique
Median sternotomy
A standard median sternotomy was performed. The pericardial cavity was opened on the left side to preserve the pericardium for augmentation of cavoatrial anastomosis, and the heart was exposed. The SVC was dissected out to the bridging vein, exposing the azygos vein and anomalous pulmonary veins. The SVC, brachiocephalic vein, and the jugular-subclavian junction were largely mobilized.
Establishment of cardiopulmonary bypass
Cardiopulmonary bypass was established with cannulation to the ascending aorta, innominate vein, or high portion of SVC into the innominate vein, and the inferior vena cava (IVC) through the right atrium. In 2 patients with persistent left SVC, the left SVC was also directly cannulated. The venous cannula for the innominate vein was an L-shaped catheter with an additional hand-made hole at the heel. The IVC cannula was a simple flexible straight catheter. Initially, the SVC was cross-clamped and divided above the insertion of the highest anomalous pulmonary vein, and its caudal stump was oversewn using 6-0 polypropylene, with division of the azygos vein. In one patient, there was no azygos vein. A vent tube was inserted through the caudal tip of the divided SVC or left atrial appendage. After cross-clamping of the ascending aorta, cardiac arrest was obtained using St Thomas solution through the root cannula under moderate hypothermia. A tourniquet was applied to the IVC and the anomaly was approached by a longitudinal right atriotomy parallel to the atrioventricular sulcus. A sinus venosus ASD was present in 15 patients (75%), 4 of whom had an additional patent foramen ovale. Four patients (20%) had a secundum ASD and one had an intact atrial septum.
Atrial septal displacement
To make a pulmonary venous drainage channel, we performed intraatrial rerouting from the native SVC orifice to the ASD, to drain the anomalous pulmonary veins into the left atrial cavity, using a patch made of a Gore-Tex surgical membrane (W L Gore & Assoc, Flagstaff, AZ) in 4 patients and a free autologous patch in 2 patients with 5-0 polypropylene. The suture line of the patch was placed away from the sinus node and the pulmonary veins. In the other 14 patients, rerouting was performed by displacement of the free edge of the ASD crest onto the venous component of the right atrium using 6-0 polypropylene just above the intracardiac orifice, with careful attention to avoid injury of the sinus node. Enlargement of the ASD is not required when the defect is large enough to provide a nonrestrictive pulmonary venous drainage channel and the defect is not far away from the orifice of the SVC. However, it was necessary to enlarge the sinus venosus defect in 2 patients, to enlarge the secundum ASD in 3 patients, and to create a high atrial defect in one patient with an intact atrial septum.
Weaning from cardiopulmonary bypass and cavoatrial anastomosis
The aortic clamp was released and, in all patients, sinus rhythm was recovered, although cardioversion was necessary in 5 patients. We then reconstructed the cavoatrial channel with the heart beating.
In 17 patients (85%), right atriotomy was extended to the appendage to make an atrial flap, which was to be the posterior wall of the cavoatrial channel. The anterior wall of the channel was augmented by a pedicled autologous pericardial flap. We used this technique to avoid excessive tension on the anastomosis, especially in patients with anomalous pulmonary veins draining into the SVC far above the SVC-right atrial junction. In 3 other patients early in our series, we performed repair without the pedicled autologous pericardial flap. In 2 patients (10%), the tip of the atrial appendage was amputated and, after excision of obstructing muscular trabeculae, the cephalic end of the divided SVC was anastomosed directly to the amputated right atrial appendage. In another patient (5%), cavoatrial continuity was established by the creation of a tube of the right atrial appendage and lateral wall. A large pedicle was cut from the appendage and lateral right atrial wall and a roll-shaped conduit was prepared from the pedicled flap. The pedicle was sutured circumferentially to the divided end of the cephalic SVC. In all cases, the anterior wall of the cephalic end of the divided SVC was vertically cut to make the orifice for the anastomosis larger, and the cavoatrial anastomosis was made by continuous suturing of the posterior wall and intermittent suturing of the anterior wall. Finally, in all cases, the inner diameter of the cavoatrial anastomosis was checked to ensure a channel sufficient for SVC drainage.
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Results
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Intraoperative Data
Mean operative time was 358 ± 65 minutes. Mean bypass time and ischemic time were 148 ± 37 minutes and 56 ± 23 minutes, respectively. None of the patients received heterologous blood transfusion.
Mortality
All children survived operation and hospitalization. There were no early or late deaths with the mean follow-up of 6.5 ± 3.0 years.
Morbidity
Cavoatrial channel
Mean flow through the cavoatrial channel was 0.79 mL (range, 0.23 to 1.2) on echocardiography. On follow-up catheterization, the pressure gradient between the SVC and right atrium averaged 4.1 mm Hg (range, 0 to 16). The flow rate on echo and the pressure gradient on catheterization were proportional to the height index on preoperative angiographic images (Fig 4). Cavoatrial occlusion was incidentally found in 2 asymptomatic patients on postoperative SVC angiography. Both had persistent left SVC, which was larger than the right SVC, with a good communicating vein between them (Figs 2A, 2B). Three patients required catheter intervention in the cavoatrial anastomosis, and all three had significantly higher height index than the other patients (Fig 5).
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Fig 4. Relationship between height index and postoperative SVC flow on echocardiography and the pressure gradient in the SVC channel on catheterization. Excluding two patients with no blood flow in the cavoatrial channel due to cavoatrial occlusion, the data for eighteen patients have been plotted. R is the coefficient correlation between the height index and SVC flow, and between the index and pressure gradient through cavoatrial anastomosis, respectively, in each figure. ( = intervention group; SVC = superior vena cava; PG = pressure gradient; RA = right atrium.)
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Fig 5. Height indices in the three groups of patients: (a) two asymptomatic patients, both of whom were incidentally found to have SVC occlusion on postoperative SVC angiography and had persistent left SVC, which was larger than the right SVC, with a good communicating vein between them; (b) patients who did not have any symptom nor need any additional intervention; and (c) patients who required catheter intervention in the cavoatrial anastomosis. (SVC = superior vena cava.)
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Pulmonary venous channel
No patients exhibited pulmonary venous obstruction, and the mean flow of corrected pulmonary venous return was 0.61 mL (range, 0.23 to 0.94) on follow-up echocardiography.
Arrhythmia
All patients remain in sinus rhythm, although 2 patients had a short postoperative episode of dysrhythmia (junctional bradycardia and coronary sinus rhythm in one each) and required temporary pacing for less than 5 days postoperatively. Both of the rhythm disturbances resolved and medications and pacing were discontinued prior to discharge. Twenty-four-hour continuous Holter electrocardiographic monitoring was performed in 6 children, including the 2 patients noted above, but revealed no sinus dysfunction.
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Comment
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Surgical repair for PAPVC to SVC ideally includes complete closure of the septal defect and redirection of the anomalous pulmonary veins into the left atrium without pulmonary venous or SVC obstruction or injury of the sinus node or its blood supply. When anomalous pulmonary veins enter the right atrium opposite the ASD, the surgical technique consists of closing the ASD while including the orifice of the pulmonary veins, which then drain into left atrium through the ASD [2]. However, when the veins enter further up the SVC, surgical treatment can be more complex. In 1984, Warden and colleagues [1] reported a new technique for high PAPVC, in which caval continuity was established by direct cavoatrial anastomosis. With it, the SVC was divided above the orifices of the anomalous pulmonary veins, and the cephalic end of the divided SVC was then anastomosed directly to the right atrial appendage after amputation of the tip. A patch within the right atrium diverted the pulmonary blood flow from the orifice of the SVC through the associated sinus venosus ASD. The caudal end of the divided SVC was closed by sutures (Fig 1B). Williams and colleagues [3] subsequently used the technique and reported 6 cases of it. Warden's group [4, 5] has also subsequently reported further clinical experience with this technique. The main advantage of this technique is decreased manipulation of the cavoatrial junction while avoiding the creation of conduits inside the SVC.
To avoid cavoatrial stenosis, the cavoatrial anastomosis must be accomplished without tension. This requires, first, very careful and extensive dissection of the brachiocephalic vein and SVC, so that tension on the cavoatrial anastomosis is not excessive. Second, particular care is needed to excise completely all of the intraatrial trabeculated muscle. In recent cases, we have used a pedicled autologous pericardial flap for anterior augmentation of cavoatrial anastomosis, with the expectation of a sufficient channel and tension-free anastomosis with growth potential (Fig 1C). Pedicled autologous pericardium has been widely used in various repairs of the right heart in patients with congenital cardiac anomalies [6–9]. The use of pedicle autologous pericardium is also supported by an experimental animal study by Guyton and colleagues [10], which revealed the ability of pedicled pericardium to grow. In our institution, we have employed the Fontan operation with an extracardiac conduit, constructed using pedicled autologous pericardial roll, in selected patients with the expectation of the possibility of growth and less thrombogenicity of the conduit [11].
In the Warden technique of SVC translocation, when the highest anomalous pulmonary vein enters the higher portion of the SVC, it can be assumed that the cephalic end of the divided SVC becomes more distant from the right atrial appendage and the cavoatrial anastomosis has more tension and likelihood of stenosis. That is why we believe that the Warden technique with SVC translocation has anatomic limitations in a small number of cases. In order to solve this limitation, what we can do first is enough dissection of the SVC and the brachiocephalic vein to mobilize them. There are other possible ways, including sacrificing high and small anomalous pulmonary veins and dividing the SVC in the lower portion, making a pedicled flap with the right atrial wall and mobilizing the anastomosis more cranially, and interposing a graft between the divided SVC and right atrial appendage, and so on. However, we have found no previous clinical study that quantitatively determined the height of anomalous pulmonary vein insertion or discussed the indications for use of and results obtained with this technique. In our study, the length between the cephalic end of the highest anomalous pulmonary venous orifice and the SVC-right atrial junction was indexed to the height of a thoracic vertebral body, and the height of anomalous pulmonary vein insertion was thereby determined quantitatively (Fig 3). This method of evaluation has the advantage that the index can be calculated using only preoperative angiographic images, and is not influenced by the patient's age or body size. In our series, patients whose height index was over 2.5 required catheter intervention for release of cavoatrial channel stenosis, excluding 2 patients with persistent left SVC (Fig 5), who had difficulty maintaining patency of the repaired cavoatrial channel because blood flow is easily stolen to the coronary sinus through the larger left SVC. The patient with a height index of 2.5 underwent the original Warden technique without anterior augmentation of the pedicled autologous pericardial flap. Evaluating only cases for which the modified Warden technique was used, it is clear that both patients with a height index above 3.2 required this intervention. Care is thus needed to ensure a tension-free cavoatrial channel in patients who have extremely high insertions of anomalous pulmonary veins into the SVC, especially those with a height index above 2.5.
Another strategy recently adopted in our institution is the lack of use of foreign material to create channels to redirect anomalous pulmonary venous flow into the left atrium. To accomplish this, the intracardiac orifice of the SVC is simply coapted to that of the sinus venosus defect by suture approximation of their margins, with the expectation of future growth of the route of the pulmonary venous pathway and low incidence of atrial arrhythmia. When any patch augmentation for the anomalous pulmonary venous flow to the left atrium other than atrial septal displacement is necessary because of low location of the ASD, a free autologous pericardial patch should be placed well away from the sinoatrial node and the caval orifice. At the same time, the surgeon should not hesitate to enlarge the ASD when it is considered restrictive intraoperatively.
Study Limitations
The first limitation of our study is its retrospective, observational nature, and that it was performed in a nonrandomized fashion. We did not evaluate the height index preoperatively or use it to determine indications for use of the modified Warden technique with pedicled autologous pericardial flap. In our study we retrospectively evaluated preoperative angiographic images and surgical outcome. The second limitation is related to the small number of our patients and bias possibly resulting from performance of the study at a single institution. Although all patients in our series routinely underwent catheterization preoperatively for precise diagnosis and to determine indications for surgical treatment and strategy, preoperative catheterization could be replaced by other radiologic examinations, such as MRI with three-dimensional reconstruction. In cases in which only preoperative MRI images are performed, we believe that an index similar to the height index could be defined to evaluate the height of anomalous pulmonary veins quantitatively.
In summary, we retrospectively reviewed a consecutive series of patients who underwent treatment with the modified Warden technique. Our study revealed favorable midterm outcomes with preservation of sinus node function and patent corrected pulmonary venous return. With use of the height index, it is possible to evaluate quantitatively the height of anomalous pulmonary vein insertion and to determine which patients require more care in the creation of a cavoatrial channel. Particularly for patients with persistent left SVC, which is larger than the right SVC with a good communicating vein between them, atrial septal displacement and ligation of the right SVC above the orifices of the anomalous pulmonary veins can be a choice for repair. Patients with especially high insertion of anomalous pulmonary veins should be treated and followed, with care taken to preserve an unobstructed caval pathway.
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Acknowledgments
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We gratefully acknowledge the excellent technical assistance of Akiko Kada (General Clinical Research Unit, National Cardiovascular Center, Japan) in the statistical analysis.
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References
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- Hvass U, Pansard Y, Bohm G, Depoix JP, Enguerrand D, Worms AM. Bicaval pulmonary connection in tricuspid atresia using an extracardiac tube of autologous pediculated pericardium to bridge inferior vena cava Eur J Cardiothorac Surg 1992;6:49-51.[Abstract]
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Abstract
Introduction
