Ann Thorac Surg 2001;71:723-725
© 2001 The Society of Thoracic Surgeons
Case report
Modified repair of mixed total anomalous pulmonary venous connection
Pascal A. Berdat, MDa,
Jean-Pierre Pfammatter, MDa,
Igor Genyk, MDa,
Thierry P. Carrel, MDa
a Department of Cardiovascular Surgery and Division of Pediatric Cardiology, Swiss Cardiovascular Center, University Hospital, Bern, Switzerland
Accepted for publication April 19, 2000.
Address reprint requests to Dr Berdat, Department of Cardiovascular Surgery, Swiss Cardiovascular Center, University Hospital, CH-3010 Bern, Switzerland
e-mail: pascal.berdat{at}insel.ch
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Abstract
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Mixed total anomalous pulmonary venous connection (TAPVC) is a rare condition, accounting for only 5% of diagnosed TAPVC. It is associated with a poor prognosis unless surgically corrected by connecting the pulmonary venous sinus to the left atrium and optionally dividing the abnormal connection to the systemic venous system. We report a modified technique of complete intracardiac repair of mixed supracardiac and cardiac TAPVC in a 3-year-old child without pulmonary venous sinus-to-left atrium anastomosis.
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Introduction
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Total anomalous pulmonary venous connection (TAPVC) is rare with a poor prognosis unless treated surgically by connecting the pulmonary venous sinus (PVS) to the left atrium behind the heart. This technique, however, may be tricky because the direct side-to-side anastomosis has to be performed while the heart is luxated. Alternatively, the procedure may be realized from inside the heart using a transatrial, transseptal approach, which may be demanding, because of the usually small left atrium. Both techniques contain a risk of late anastomotic stenosis, usually occurring within 6 to 12 months of the repair, remains substantial [1]. We used a different technique in a 3-year-old child with mixed TAPVC of the supracardiac and cardiac type, allowing complete intracardiac repair.
A 3-year-old child was presented to the pediatric cardiologist because of increasing cyanosis, poor general development, and exertional dyspnea. Unobstructed TAPVC of the supracardiac and cardiac type with mild pulmonary hypertension was suspected by echocardiography and confirmed by cardiac catheterization.
Through a median sternotomy cannulation of the aorta and right atrium with a two-stage cannula was performed for cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). The right atrium was opened and a large connection of the PVS—crossing the left atrium horizontally behind the heart—at the superior cavoatrial junction and an atrial septal defect (ASD) type II were found (Fig 1).
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Fig 1. Intraoperative view of the anatomy through the opened right atrium. (1 = connection of the right upper and middle pulmonary vein directly to the superior vena cava; 2 = connection of the pulmonary venous sinus to the right atrium; 3 = ostium secundum atrial septal defect.)
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The pulmonary veins from the right upper and middle lobe were found to drain directly to the superior vena cava (SVC), approximately 15 and 20 mm above the superior cavoatrial junction. Instead of opening the dorsal wall or roof of the left atrium to perform an anastomosis with the PVS, a much simpler technique was used.
First, the ridge between the PVS junction and the ASD was resected and the caudal rim of the PVS junction was sutured directly to the cranial rim of the ASD to create a continuous dorsal wall, thus considerably enlarging the ASD. A 0.4 mm thin Gore-Tex patch (ePTFE Cardiovascular patch, Impra Inc, Tempe, AZ) was fashioned to form an intraatrial tunnel, rerouting the blood from the PVS and the two pulmonary veins connecting to the SVC across the enlarged ASD to the mitral valve.
The patch was sutured using a running 6-0 polypropylene suture (Prolene, Ethicon, Somerville, NJ) (Fig 2). Care was taken to create a tunnel large enough to drain the whole pulmonary venous blood across the enlarged ASD and not to compromise the superior cavoatrial junction. Cardiopulmonary bypass was resumed after 28 minutes and after rewarming the child was successfully weaned for CPB with normal cardiac function, no residual shunt, and with only mild pulmonary hypertension. Pulmonary artery pressure was monitored by a surgically introduced catheter during the first 24 hours. Postoperatively, no complications occurred and the child was discharged after an uneventful recovery. No pulmonary or intracardiac tunnel stenoses were found during follow-up control by transthoracic echocardiography after 18 months and the child is doing well clinically without rhythm disturbances.
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Comment
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Standard repair of TAPVC consists of creating an anastomosis between the PVS and the left atrium, either behind the luxated heart or using a transatrial, transseptal approach [1]. These methods can be technically demanding and portend the risk of development of anastomotic stenosis, necessitating reoperations with a high mortality [1, 2]. Because of the wide variety of TAPVC, various techniques and modifications have been published in recent years [3, 4]. The modified technique presented here has two distinct advantages over the standard method. First, it avoids a somewhat hazardous construction of a PVS-to-left atrium anastomosis, being technically much easier and probably avoiding the problem of postoperative anastomotic stenosis. Second, in this case of mixed TAPVC it allowed us to correct the pulmonary venous return of the right middle and upper lobe with one single intraatrial tunnel patch. As an alternative to Gore-Tex a pericardial patch may be used but was avoided in this case because of the size of the tunnel and the possible postoperative shrinkage of this material. Care must be taken not to narrow the distal SVC with the patch, and especially in small children we recommend enlargement of the distal SVC with a separate triangular patch, with the method reported by Warden and associates [5] for repair of partial anomalous pulmonary venous connection to the SVC, or with an atriocavoplasty [6]. Single cannulation of the right atrium avoids selective cannulation of the SVC or innominate vein with its risk of incomplete venous drainage and late stenosis. Deep hypothermic circulatory arrest allows for single cannulation and removal of all of the venous cannula for thorough inspection of the situs. Besides the modification of the surgical technique, this case was very unusual because of the late presentation with cyanosis and dyspnea at the age of 3 years.
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References
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Kirklin J.W., Barratt-Boyes B.G. Total anomalous pulmonary venous connection. In: Kirklin J.W., Barratt-Boyes B.G., eds. Cardiac surgery. New York: Churchill Livingstone, 1993:645-681.
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Ninet J., Gordillo M., Vigneron M., et al. Total anomalous pulmonary venous connection. Results of repair in 50 infants. Arch Mal Coeur Vaiss 1990;83:217-221.[Medline]
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Van Son J.A., Hambsch J., Mohr F.W. Modified repair of mixed anomalous pulmonary venous connection. Ann Thorac Surg 1998;65:1441-1442.[Abstract/Free Full Text]
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Serraf A., Belli E., Roux D., Sousa-Uva M., Lacour-Gayet F., Planché C. Modified superior approach for repair of supracardiac and mixed total anomalous pulmonary venous drainage. Ann Thorac Surg 1998;65:1391-1393.[Abstract/Free Full Text]
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Warden H.E., Gustafson R.A., Tarnay T.J., Neal W.A. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984;38:601-605.[Abstract/Free Full Text]
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De Leon S.Y., Gidding S.S., Ilbawi M.N., et al. Surgical management of infants with complex cardiac anomalies associated with reduced pulmonary blood flow and total anomalous pulmonary venous drainage. Ann Thorac Surg 1987;43:207-211.[Abstract/Free Full Text]
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