Mohamed Nassar, MD, Virginie Fouilloux, MD, Loïc Macé, MD, Bernard Kreitmann, MD, PhD, and Dominique Metras, MD Service of Cardiothoracic Surgery, Children’s Hospital La Timone, Marseille, France
Background. The ideal technique for addressing partial anomalous pulmonary venous drainage into the superior vena cava (SVC), with or without sinus venosus atrial septal defect (ASD), is debated. The risk of sinus node dysfunction, systemic, or pulmonary venous channels obstruction has led to different techniques being developed. We present our experience with 45 patients operated on using a vertical transcaval approach, without atrial or cavoatrial junction incision. Methods. Between 2001 and 2010, 45 patients (28 females, 17 males, with a mean age of 5 years (range, 8 months to 70 years), underwent operations using 1 patch of autologous pericardium, after vertical SVC incision anterior to the anomalous pulmonary veins: 43 had associated sinus venosus ASD, and 6 had associated left SVC. Access was through sternotomy in 19 and right posterior thoracotomy in 26. An additional right atrial incision, without crossing the cavoatrial junction, was used in 2 patients without ASD. Mean cardiopulmonary
bypass time was 76 minutes. Mean cross-clamp time was 44 minutes. Results. No deaths or important morbidities occurred. Mean follow-up was 4.4 years (range, 2 months to 9.3 years). All patients had regular echocardiographic examination, electrocardiogram, and midterm 24-hour Holter electrocardiogram. No new arrhythmias occurred. All patients showed unobstructed caval and pulmonary venous flow. Conclusions. The vertical transcaval approach is a simple, highly reproducible technique for correction of partial anomalous pulmonary venous drainage into the SVC. It yields excellent results, with unobstructed pulmonary and systemic venous flow and without arrhythmia development. It can also be performed through a cosmetic right posterior thoracotomy.
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2. division of the SVC, baffling the PAPV drainage to the left atrium, and insertion of the SVC into the right atrial appendage (known as Warden procedure) [10 –12], and 3. longitudinal or transverse transcaval incisions, with or without terminal SVC augmentation [13–15].
he incidence of partial anomalous pulmonary venous (PAPV) drainage has been reported to be about 0.7% in some autopsy series [1], and the anomalous drainage into the right side of the heart is usually associated with sinus venosus atrial septal defect (ASD), which is present in 87% to 97% of reported cases [1,2]. The PAPV connection to the right side of the heart has always been a controversial topic, not only surgically but also concerning embryology and pathology [3, 4]. Several techniques to complete the surgical correction of the PAPV connection to the superior vena cava (SVC) have been developed. Since the early surgical correction by Neptune and colleagues [5] and then Kirklin and colleagues [6] in 1956, several modifications and different techniques have been proposed. The three basic techniques that are commonly in use nowadays are: 1. a single-patch or double-patch technique through a right atriotomy traversing the cavoatrial junction [6 –9], Accepted for publication Sept 14, 2011. Address correspondence to Dr Metras, Service of Cardiothoracic Surgery, Children’s Hospital La Timone, Boulevard Jean Moulin, 13385 Marseille, France; e-mail:
[email protected].
© 2012 by The Society of Thoracic Surgeons Published by Elsevier Inc
(Ann Thorac Surg 2012;93:193– 6) © 2012 by The Society of Thoracic Surgeons
We have adopted the longitudinal, vertical transcaval approach, as reported by Nicholson and colleagues [15], since 2000 and report our results with that technique. The Ethic Committee of our institution approved the study, and individual consent of the patients was not required.
Material and Methods Since 2000, all patients referred to our center for surgical correction of PAPV drainage into the SVC (with or without sinus venosus ASD) were addressed using the vertical transcaval approach. Operations were initiated through a median sternotomy or a cosmetic posterior right thoracotomy. All were done under normothermic cardiopulmonary bypass (CPB) with direct cannulation of the SVC above the insertion of the highest anomalous pulmonary vein, with the azygos vein well controlled and cannulation of the 0003-4975/$36.00 doi:10.1016/j.athoracsur.2011.09.042
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PEDIATRIC CARDIAC Fig 1. Three anomalous pulmonary veins (PV) enter the terminal part of the superior vena cava (SVC) with the cannulation site high enough above the site of drainage of the pulmonary veins. (IVC ⫽ inferior vena cava; RA ⫽ right atrium.)
inferior vena cava, both cavae with right-angled cannulas (Fig 1). After verification of the anatomy and the start of CPB, application of aortic cross-clamp, and introduction of cold antegrade cardioplegia, the SVC is vertically incised just anterior to the anomalous pulmonary veins (Fig 2). The cavoatrial junction is always respected, and is never crossed. In a patient with a sinus venosus ASD, a patch is used to divide the cavopulmonary channel in such a manner that the SVC is directed to the right atrium (anterior to the patch) and the anomalous veins are directed toward the left atrium (posterior to the patch; Fig 3). Appropriate sizing of the patch with a flat final
Fig 2. After a vertical incision of the superior vena cava, the orifice of the abnormal pulmonary veins (PV) is well seen. (RA ⫽ right atrium; SVD ⫽ sinus venosus defect.)
Fig 3. The patch is sutured to the inferior and medial edge of the sinus venosus atrial septal defect and in the terminal superior vena cava anterior to the pulmonary vein (PV), dividing an anterior caval channel and a posterior pulmonary channel. (RA ⫽ right atrium; SVD ⫽ sinus venosus defect.)
aspect is crucial to avoid obstruction of either channel. The patch (autologous pericardium treated with glutaraldehyde) is sewn using a running polypropylene 5– 0 suture that fixes the patch to the inferior and medial edges of the defect, then to the inner aspect of the SVC, rejoining the highest pulmonary vein orifice. The lateral edge (the incision site) is closed in sandwich between the two edges of the caval incision (Fig 4). The sinus venosus ASD was absent in 2 patients, and so a small right atriotomy was initiated in addition to the caval incision to create an ASD by removal of the floor of the fossa ovalis. A larger patch was inserted to baffle the anomalous veins to the left atrium using a running suture, taking advantage of both incisions.
Fig 4. After finishing the suture of the patch at the site of incision (*) in the sandwich technique, the suture stands well above the cavoatrial junction.
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Air was removed and aortic clamp release was done in the standard manner, followed by weaning of the CPB. Regular follow-up of all patients included a physical examination, chest roentgenogram, electrocardiography, and echocardiography. A midterm or long-term 24-hour Holter electrocardiogram was obtained in most patients.
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Results Between 2000 and 2009, 45 patients (17 males and 28 females), with a mean age of 5 years (range, 8 months to 70 years), were operated on using the transcaval approach. Of these, 22 patients were addressed through a median sternotomy and 23 through a cosmetic right posterior thoracotomy. There were 2 anomalous veins in 20 patients, 3 in 24 patients, and 1 patient had 4 anomalous veins draining into the SVC. Persistent left SVC was found in 6 patients. PAPV drainage without a sinus venosus ASD was found in 2 patients who had a small patent foramen ovale (PFO). For those with a sinus venosus ASD, the defect was large enough so that only 1 patient required enlargement of the defect to avoid obstruction of either channel. Four patients had PFO in addition to the sinus venosus ASD, and in all cases, closure of the PFO was accessible through the caval incision. Only 1 patient required augmentation of the site of incision of the SVC to avoid SVC obstruction because the terminal SVC, associated with the presence of a left SVC, was estimated to be too small. The mean aortic cross-clamp time was 44 minutes, with a mean CPB time of 76 minutes. The mean stay in the intensive care unit was 1.6 days. No deaths were recorded. Morbidity included pneumothorax in 2 patients, pericardial effusion in 4, postcardiotomy syndrome in 1, and a transient dysfunction of the right phrenic nerve in 1 that returned to normal 1 month later. The follow-up period was a mean 5.4 years (range, 4 months to 10.4 years) and was complete. All patients were regularly followed up and were asymptomatic through the follow-up period. Echocardiography confirmed the absence of any residual ASD. No obstruction of the pulmonary venous drainage was noted. No echocardiographic signs of caval flow obstruction were detected, except for 1 patient who had a gradient of 6 mm Hg across the cavoatrial junction in the immediate postoperative period that completely disappeared during follow-up. All patients, excluding a 70-year-old woman in atrial fibrillation preoperatively, were in sinus rhythm at the last report by their cardiologist. No incidence of new arrhythmia was detected. A 24-hour Holter echocardiography in 20 patients confirmed the absence of any arrhythmia. Systematic Holter monitoring is planned for all others.
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Since the early reported techniques for PAPV drainage and sinus venosus ASD repair, the risk of complication has always been the motive for innovation. The most fearful complications are arrhythmia and obstruction of the caval or pulmonary venous channel. It is the complexity of the anatomic site of the anomaly that makes such a repair challenging. Injury to the sinus node can result in a serious arrhythmia and subsequently the need for permanent pacing. The arterial supply to the sinus node is highly variable [16], and injury to the feeding artery can have the same results. Theoretically, procedures trying to avoid crossing the cavoatrial junction, such as the Warden procedure and the transcaval techniques, are believed to have a minimal incidence of arrhythmia. That is true for the transcaval techniques, with no incidence of new arrhythmia in all reviewed literature [13–15]. Although the Warden procedure is supposed to yield the same result, the first report by Warden and colleagues [10] reported a 10% incidence of new arrhythmia that decreased on follow-up to 2.5%. Other reports of the same procedure have a variable incidence of arrhythmia from 0% to 23% [17]. Of interest were the results of two reports: ●
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the atrial flap technique by Takahashi and colleagues [18], who reported an 93% incidence of arrhythmia in the immediate postoperative period that decreased to 57% at the time of discharge, although according to their technique, the cavoatrial junction was always respected; and the Toronto series of 171 patients who received a lateral right atriotomy crossing the cavoatrial junction, after which Alsoufi and colleagues [1] reported a paradoxic 0% incidence of new arrhythmia.
The risk of SVC obstruction after surgical correction is inherent in all techniques, and that was the reason for the incorporation of another patch to enlarge the terminal SVC. A literature review revealed a highly variable incidence of SVC obstruction with different techniques. Iyer and colleagues [19] compared the single-patch and double-patch technique, both through atriotomy crossing the cavoatrial junction, and found a statistically significant difference in favor of the double-patch technique. Results of the Warden procedure and its modifications have shown a low incidence of SVC obstruction despite a more complex technique that sometimes needs a patch when the appendage is small and the SVC distant, and they all emphasized the importance of a large caval-auricular anastomosis with resection of any trabeculation that may cause future obstruction [10 –12]. With the transcaval approach, no incidence of obstruction was reported whether or not the terminal SVC was augmented [13–15]. Although we felt it was necessary to augment the terminal SVC of a patient who had a SVC of an abnormally small caliber and an associated left SVC, we believe that the terminal SVC receiving caval flow and PAPV drainage flow with this anomaly is usually
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large enough to allow an adequate division in two parts, provided the patch is adequately sized and inserted, and we therefore think that this approach is always possible. Obstruction of the pulmonary venous return after surgical correction is uncommon, with an incidence of about 0% in most of the reviewed techniques [6 –15]. However, some experiences have reported an incidence as high as 50% with the single-patch technique [18]. It is of some interest that the approach through a right posterior thoracotomy (for cosmetic purposes) [20] allows an excellent view of the area, as was done in 26 of our patients. The SVC and the cavoatrial junction are the closest structures using this approach in children. In conclusion, the vertical transcaval technique reported by Nicholson and colleagues [15] has by far the least incidence of complications reported when compared with all other procedures. For us it has led to excellent and reproducible results, and we consider it as our routine and basic approach. In addition, this technique is simple, highly reproducible, and can also be completed through a cosmetic right posterior thoracotomy.
7. Ohmi M, Mohri H. A single pericardial patch technique for repair of partial anomalous pulmonary venous drainage associated with sinus venosus atrial septal defect. Ann Thorac Surg 1988;46:360 –1. 8. Kouchoukos N, Blackstone EH, Doty DB, Hanley FL, Karp RB. Atrial septal defects and partial anomalous pulmonary venous connection. In: Kouchoukos N, Blackstone E, Doty D, Hanley F, Karp R, eds. Kirklin/Barratt-Boyes Cardiac Surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003:715– 52. 9. Backer CL, Mavroudis C. Paediatric cardiac surgery. 3rd ed. Philadelphia: Mosby; 2003:283–97. 10. Trusler GA, Kazenelson G, Freedom RM, et al. Late results following repair of partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. Journal of Thoracic and Cardiovascular Surgery 1980;79:776 – 81. 11. Warden HE, Gustafson RA, Tarnay TJ, et al. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984;38:601–5. 12. Atsushi N, Toshikatsu Y, Koji K, et al. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 2006;82:978 – 82. 13. Gaynor JW, Burch M, Dollery C, et al. Repair of anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;59:1471–5. 14. Victor S, Nayak VM. Transcaval repair of sinus venosus defect. Using a butterfly-shaped patch. Tex Heart Inst J 1995;22:304 –7. 15. Nicholson IA, Chard RB, Nunn GR, et al. Transcaval repair of the sinus venosus syndrome. J Thorac Cardiovasc Surg 2000;119:741– 4. 16. Baskett R, Ross DB. Superior vena cava approach to repair of sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 2000;119:178 – 80. 17. Futami C, Tanuma K, Tanuma Y, et al. The arterial blood supply of the conducting system in normal human hearts. Surg Radiol Anat 2003;25:42–9. 18. Takahashi H, Oshima Y, Yoshido M, et al. Sinus node dysfunction after repair of partial anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 2008;136: 329 –34. 19. Iyer AP, Somanrema K, Pathak S, et al. Comparative study of single- and double-patch techniques for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 2007;133:656 –9. 20. Metras D, Kreitmann B. Correction of cardiac defects through a right thoracotomy in children. J Thorac Cardiovasc Surg 1999;117:1040 –2.
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