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Ann Thorac Surg 1998;65:1391-1393
© 1998 The Society of Thoracic Surgeons

Modified Superior Approach for Repair of Supracardiac and Mixed Total Anomalous Pulmonary Venous Drainage

Alain Serraf, MDa, Emré Belli, MDa, Daniel Roux, MDa, Miguel Sousa-Uva, MDa, François Lacour-Gayet, MDa, Claude Planché, MDa

a Department of Pediatric Cardiac Surgery, Marie-Lannelongue Hospital, Le Plessis-Robinson, France

Accepted for publication December 10, 1997.

Address reprint requests to Dr Serraf, Marie-Lannelongue Hospital, 133 Ave de la Résistance, 92350, Le Plessis-Robinson, France
e-mail: (aserraf{at}centre-chirurgical-marie-lannelongue.asso.fr)


    Abstract
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 Footnotes
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
Background. The main goal in the surgical repair of total anomalous pulmonary venous drainage is to reestablish a wide patent connection between the common pulmonary vein and the left atrium. Several techniques have been proposed for achieving this objective, each of which has advantages and disadvantages. The superior approach between the superior vena cava and the ascending aorta was introduced in 1976 for the repair of supracardiac forms of total anomalous pulmonary venous drainage, but it often provides a less than optimum exposure, particularly in tiny infants. We proposed a modification of this approach that includes division of the ascending aorta and offers excellent exposure.

Methods. Seventeen patients (15 neonates and 2 infants) with supracardiac total anomalous pulmonary venous drainage (n = 13) or mixed forms of total anomalous pulmonary venous drainage (n = 4) underwent surgical repair with the use of the modified superior approach. Circulatory arrest was not required in 10 patients and the mean cross-clamp time was 32.5 ± 13.8 minutes.

Results. There was 1 postoperative death resulting from intractable pulmonary hypertension in a compromised infant who was referred to our unit receiving extracorporeal membrane oxygenation. One patient with common hypoplasia underwent reoperation twice at 2 months and then 3 months after the first procedure. All the other patients had a smooth postoperative course, and midterm evaluation showed a widely patent anastomosis between the common vein and the left atrium.

Conclusions. The modified superior approach for the repair of supracardiac total anomalous pulmonary venous drainage can be useful to enhance exposure during surgical repair and may contribute to improved patient outcome.


    Introduction
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 Abstract
 Introduction
 Patients and methods
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Since the first successful repair of total anomalous pulmonary venous drainage (TAPVD), many surgical teams have reported constant improvement in patient outcome [13]. This improvement has been related to a better understanding of the different anatomic forms of the disease, to elucidation of the physiologic importance of preoperative pulmonary venous obstruction and right ventricular overload, and to refinements in techniques of surgical repair and neonatal perfusion. The main goal of operation is to reestablish a wide patent connection between the common pulmonary vein and the left atrium.

The classic technique for surgical repair of TAPVD initially was described by Kirklin [4]. Through a transatrial approach, the posterior wall of the left atrium is opened, the underlying common pulmonary vein is opened, and anastomosis is performed. Although this approach has proved to be safe and reproducible, it requires several atrial incisions and is associated with a high incidence of postoperative and long-term supraventricular arrhythmias. However, this technique has the advantage of being applicable to most anatomic forms of TAPVD.

Another technique for connecting the common vein to the left atrium involves elevating the heart apex cephalad and to the right. However, this technique has the disadvantage that the anastomosis is performed in a vertical plane that has to be horizontal, and twisting of the anastomosis might occur. In 1976, Tucker and colleagues [5] proposed the use of a superior approach between the superior vena cava and the ascending aorta for the repair of supracardiac forms of TAPVD. This approach offers good exposure to the roof of the left atrium as well as to the common pulmonary vein, but it can present spatial limitations, particularly in tiny infants.

In addition to the classic anatomic forms of TAPVD, there is a mixed type of TAPVD that is difficult to repair and is associated with higher mortality and morbidity, particularly in neonates and young children [6]. Although previously described techniques have been shown to produce satisfactory results, we have been confronted with individual cases in which these techniques did not offer optimum exposure. Therefore, enriched by the surgical experience with the arterial switch operation, we present herein a surgical modification of the superior approach for enhanced exposure of supracardiac forms of TAPVD and of mixed forms of TAPVD characterized by discontinuity of the right and left pulmonary venous drainage as a result of division of the ascending aorta.


    Patients and methods
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 Footnotes
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
Patients
Between July 1994 and April 1997, 17 patients referred to Marie Lannelongue Hospital for the repair of supracardiac TAPVD (n = 13) or mixed forms of TAPVD (n = 4) underwent operation with the use of the modified superior approach. There were 10 boys and 7 girls; 15 of the patients were neonates and the other 2 were 8 months old and 2 years old, respectively. The median body weight at operation was 3.2 kg (range, 1.7 to 8 kg). Ten of the patients presented with severe low cardiac output caused by obstruction of the pulmonary venous return. Patient demographic and anatomic data are listed in Table 1. All 4 patients with mixed forms of TAPVD presented with two left pulmonary veins draining into a vertical vein and two right pulmonary veins draining into the superior vena cava. In addition, 2 of them had an obstruction at the junction of the left pulmonary veins and the vertical vein.


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Table 1. Patient Characteristics

 
Surgical technique
Operation was undertaken as soon as the diagnosis was made. It was conducted under cardiopulmonary bypass with bicaval venous cannulation and deep hypothermia. Only 7 patients underwent deep hypothermic circulatory arrest. Myocardial protection usually was ensured by a single dose of blood cardioplegia delivered to the aortic root.

After the institution of cardiopulmonary bypass and ligation of the ductus arteriosus, if any, the ascending aorta was dissected free of the pulmonary trunk (Fig 1). The right pulmonary artery also was dissected free and mobilized. The common pulmonary vein then was identified below the right pulmonary artery and the ascending aorta. It was dissected free of its pericardial attachments to the greatest extent possible. The aorta was cross-clamped, cardioplegia was delivered, and the aorta was divided 1 cm above the coronary ostia. The proximal stoma was held down gently by a stay suture, the right pulmonary artery was retracted upward, and the pulmonary trunk was retracted leftward.



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Fig 1. Surgical repair in the supracardiac form of total anomalous pulmonary venous drainage. See text for details.

 
At this stage, exposure of the roof of the left atrium as well as the common vein was excellent without any need to pull on surrounding structures. The roof of the left atrium was opened widely and care was taken to avoid the sinus node artery when present. The common pulmonary vein then was opened parallel to the left atrial incision and the lungs were inflated gently to evacuate the remaining blood in the pulmonary vascular bed. The anastomosis was completed with a running 8/0 suture. No attempt was made to control the different pulmonary veins to avoid potential sites of late pulmonary vein stenosis. The aorta was reconstructed by terminoterminal anastomosis in a standard manner. Air was removed from the heart and the cross-clamp was removed.

In the patients who had mixed forms of TAPVD characterized by discontinuity of the right and left pulmonary venous drainage, the left pulmonary vein was anastomosed to the roof of the left atrium using the modified superior approach (Fig 2). A right atriotomy then was performed and the left atrium was tunnelized to the junction of the right atrium and the superior vena cava through an enlarged foramen ovale. For tunnel construction, a polytetrafluoroethylene patch was used and was secured with a 7/0 suture. The superior vena cava then was divided proximal to the right pulmonary veins. The distal stump, including the right pulmonary veins, was closed with a 6/0 suture, allowing these veins to drain into the left atrium through the constructed intraatrial tunnel. The proximal stump of the superior vena cava then was anastomosed to the right atrial appendage after wide mobilization of the superior vena cava. The anastomosis was performed with a running 7/0 absorbable suture.



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Fig 2. Surgical repair in the mixed form of total anomalous pulmonary venous drainage. See text for details.

 

    Results
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 Footnotes
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
One 6-hour-old infant died in the early postoperative period of intractable pulmonary hypertension. This infant was born with severe bilateral parenchymal pulmonary damage and bilateral pneumothorax and was transferred to our unit receiving extracorporeal membrane oxygenation. Despite postoperative extracorporeal membrane oxygenation therapy, he still had pulmonary hypertensive crisis and showed no improvement in pulmonary function. Postmortem examination revealed a wide patent anastomosis between the common vein and the left atrium and severe parenchymal lesions with giant telangiectasis.

All the other patients were weaned smoothly from cardiopulmonary bypass and were discharged from the hospital. The mean duration of cardiopulmonary bypass was 85.2 ± 23.5 minutes, the mean aortic cross-clamp time was 32.5 ± 13.8 minutes, and the mean duration of deep hypothermic circulatory arrest (7/16 patients) was 29.1 ± 12.3 minutes. Five patients required delayed sternal closure and the mean time to extubation was 3.8 ± 2.5 days. Paroxysmal pulmonary hypertensive crisis developed in 6 patients and was controlled easily with the use of inhaled nitric oxide. Pulmonary vein stenosis developed in 1 patient with a hypoplastic common vein (2.5 mm), requiring reoperation at 2 months and then again at 3 months after the first procedure. The patient ultimately died of pulmonary hypertension after the third operation. Postmortem examination revealed a wide patent central anastomosis between the common vein and the left atrium and bilateral intrahilar multiple pulmonary vein stenoses.

All the survivors have been followed up for a mean of 28.4 ± 12 months. They all are in New York Heart Association class I without medication. Echocardiographic studies have revealed no gradient across the anastomosis and satisfactory growth of the reconstructed superior vena cava.


    Comment
 Top
 Footnotes
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
The goal of any surgical technique for the repair of TAPVD is to achieve a widely patent anastomosis between the pulmonary venous return and the left atrium. Several techniques have been proposed to meet this purpose. We believe that some of them require too many atrial incisions, with the potential for arrhythmias, and others do not offer adequate exposure. The superior approach has the advantage of exposing the common vein and the left atrium in the same plane and thereby reducing the risk of kinking at the anastomotic site. We modified this approach by dividing the ascending aorta to enhance the exposure. This procedure allowed an easy approach to the retroaortic space for access to the common vein. Because of the better exposure, most of the patients did not require circulatory arrest.

In those forms of mixed TAPVD with associated right pulmonary venous drainage to the superior vena cava and left pulmonary venous drainage to the innominate vein, combination of the modified superior approach and the more classic Warden technique for sinus venosus repair [7] allowed a satisfactory outcome in all patients. This latter technique could be used even in neonates, and the follow-up echocardiographic studies demonstrated normal growth of the new right atrium-to-superior vena cava junction. Anastomosis of the left veins to the roof of the left atrium using the modified superior approach was preferred over the more accepted direct anastomosis of the veins to the left atrial appendage because the appendage is not always sufficiently developed to allow for a large anastomosis.

In conclusion, the modified superior approach is a useful adjunct to the surgical techniques for repair of supracardiac TAPVD. In mixed forms of TAPVD, it can be combined with the Warden technique even in neonates.


    Footnotes
 Top
 Footnotes
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
This article has been selected for the open discussion forum on the STS Web site: http://www.sts.org/annals


    References
 Top
 Footnotes
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 

  1. Sano S., Brawn W.J., Mee R.B.B. Total anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 1989;97:886-892.[Abstract]
  2. Serraf A., Bruniaux J., Lacour-Gayet F., et al. Obstructed total anomalous pulmonary venous return. J Thorac Cardiovasc Surg 1991;101:601-606.[Abstract]
  3. Bando K., Turrentine M.W., Ensing G.J., et al. Surgical management of total anomalous pulmonary venous connection. Thirty year trends. Circulation 1996;94(Suppl II):12-16.
  4. Kirklin J.W. Surgical treatment of anomalous pulmonary venous connections. Mayo Clin Proc 1953;28:476-479.[Medline]
  5. Tucker B.L., Lindesmith G.G., Stiles Q.R., Meyer B.W. The superior approach for correction of the supracardiac type of total anomalous pulmonary venous return. Ann Thorac Surg 1976;22:374-377.[Abstract]
  6. Delius R.E., de Leval M.R., Elliott M.J., Stark J. Mixed total pulmonary venous drainage: still a surgical challenge. J Thorac Cardiovasc Surg 1996;112:1581-1588.[Abstract/Free Full Text]
  7. 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]



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