Ann Thorac Surg 2001;72:249-251
© 2001 The Society of Thoracic Surgeons
Case report
Repair of total anomalous pulmonary venous connection without cardiopulmonary bypass
Yoshio Ootaki, MDa,
Masahiro Yamaguchi, MDa,
Yoshihiro Oshima, MDa,
Naoki Yoshimura, MDa,
Shigeteru Oka, MDa
a Department of Cardiothoracic Surgery, Kobe Children’s Hospital, Kobe, Hyogo, Japan
Accepted for publication May 19, 2000.
Address reprint requests to Dr Ootaki, Department of Cardiothoracic Surgery, Kobe Children’s Hospital, 1-1-1 Takakuradai, Suma-ku, Kobe, Hyogo, 654-0081, Japan
e-mail: y.ootaki{at}nifty.ne.jp
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Abstract
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Two infants with asplenia syndrome and pulmonary venous obstruction underwent repair of total anomalous pulmonary venous connection without cardiopulmonary bypass in 1988. Using a side-biting clamp, an anastomosis between the left upper pulmonary vein and left superior vena cava-atrial junction was satisfactorily created without undue instability. Furthermore, both cases had the advantage of efficient growth in the anastomotic site postoperatively.
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Introduction
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Total anomalous pulmonary venous connection (TAPVC) is a rare cardiac anomaly often associated with other complex cardiac malformations. The early and long-term outcomes after surgical repair of isolated TAPVC are excellent in the current era [1–3]. However, previous reports have suggested that the association of TAPVC with a single ventricle, particularly in infants with right isomerism and pulmonary venous obstruction, risks a poor outcome [4–6]. We report a technique for relief of pulmonary venous obstruction without cardiopulmonary bypass in infants with this combination and the long-term fate of 2 infants who underwent this procedure in 1988.
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Case reports
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Patient 1
The patient, a girl, was born on May 26, 1988. Cyanosis was noted 1 day after her birth and prostaglandin E1 was started immediately after an echocardiographic diagnosis of TAPVC with pulmonary venous obstruction. Emergency cardiac catheterization revealed: (1) single, primitive ventricle; (2) probable asplenia; (3) common atrioventricular valvular regurgitation; (4) pulmonary atresia with patent ductus arteriosus; (5) bilateral superior vena cava; and (6) paracardiac and subdiaphragmatic TAPVC, and TAPVC was obstructed below the pulmonary venous confluence and the junction of the hepatic vein. An emergency operation was performed 6 hours after the catheterization. With the use of a side-biting clamp, the anastomosis between the left upper pulmonary vein and the left SVC-atrial junction was performed in stable condition through a left thoracotomy without the use of cardiopulmonary bypass, the descending vertical vein was left alone, and a left modified-Blalock-Taussig shunt was created in addition. She was extubated 3 days after respiratory support. The child did well at home, and cardiac catheterization at 5 years and 8 months of age showed that the left upper pulmonary venous pressure was 17 mm Hg, the mean atrial pressure was 15 mm Hg, and there was no pulmonary venous obstruction. However, she could not be a candidate for Fontan or Glenn operation. She was found apneic and pulseless at home approximately 1 year later.
Patient 2
The patient, a boy, was born on August 15, 1991. Cyanosis was noted when he was 36 days old. An echocardiogram showed suspicion of an anomalous pulmonary venous connection. The cardiac catheterization revealed: (1) single, primitive ventricle; (2) probable asplenia; (3) common atrioventricular valve; (4) pulmonary stenosis, (5) bilateral superior vena cava; and (6) subdiaphragmatic TAPVC (obstructed). An operation was performed when he was 56 days old. The left upper pulmonary vein was anastomosed to the left SVC-atrial junction through a left thoracotomy without cardiopulmonary bypass (Fig 1). The anastomosis became 13 mm in length. He was extubated the next day and did well at home. Pulmonary venous obstruction was not confirmed by echocardiogram and catheterization at 4 years and 6 months of age, the left upper pulmonary venous pressure was 9 mm Hg, and the mean atrial pressure was 8 mm Hg. When he was 6 years and 8 months of age, a fenestrated total cavo-pulmonary connection was performed and the descending vertical vein was ligated. At operation, the previous anastomosis between the left upper pulmonary vein and the left SVC-atrial junction was noted to have grown to over 20 mm in diameter.
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Fig 1. The operative scheme in patient 2. (A) The left upper pulmonary vein and left superior vena cava-atrial junction were anastomosed with the use of side-biting technique. (B) The anastomosis became 13 mm in length. (LSVC = left superior vena cava; LUPV = left upper pulmonary vein; LLPV = left lower pulmonary vein.)
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Comment
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In the absence of associated cardiac anomalies, surgical management of TAPVC is excellent in the current era [1–3]. However, surgical repair of TAPVC associated with right isomerism may still be a challenge [4–6] because total intracardiac repair is not advisable in neonates or infants and surgical repair must be palliative, which would require a cardiopulmonary bypass.
The technique described herein has the major advantage of avoiding a cardiopulmonary bypass. The rapid postoperative recovery was remarkable, especially in patient 2, who was extubated the next day after the operation. A similar approach was reported by Lamberti and associates [7]. An anastomosis was created between the pulmonary venous confluence and the right atrium with the use of a side-biting clamp. However, this technique may not always be possible, as Lamberti pointed out, because the confluence of the veins can be narrow. Our technique, an anastomosis created between left upper pulmonary vein and left SVC-atrial junction, has an advantage of less risk of obstructing the pulmonary venous confluence and deteriorating infantile condition. With our technique, as the left upper pulmonary vein-common pulmonary vein junction is only side clamped, the obstruction to the venous return from contralateral pulmonary vein and also from ipsilateral lower pulmonary vein is unlikely to occur. The patients tolerated the interval of the pulmonary venous occlusion without undue instability. In patients not associated with bilateral SVC, it is possible to create an anastomosis between the right upper pulmonary vein and atrium using the same technique.
The obvious disadvantage of this approach to TAPVC is that the veins will drain into the right atrium. Physiologically, this has no significant effect, because the patients with asplenia syndrome have a common mixing lesion. There is the theoretical disadvantage that the left ventricle will have less impetus to grow; however, this is of little import, because the subsequent corrective operation would probably involve bicaval-pulmonary artery anastomosis.
There are no reports regarding the long-term fate of the anastomotic site of repaired TAPVC without cardiopulmonary bypass. In patient 2, we noted a very acceptable growth of the anastomotic site. Patency of the anastomosis was also confirmed at the time of cardiac catheterization 5 years after the operation in patient 1.
In summary, we report a technique for relieving pulmonary venous obstruction in infants with TAPVC, right isomerism, and bilateral SVC without a cardiopulmonary bypass, and the long-term fate of 2 infants who underwent this procedure back in 1988. We conclude that, in infants with asplenia syndrome, bilateral SVC, and TAPVC with pulmonary venous obstruction, while this is a technique that rarely may be indicated in unusual circumstances, relief of pulmonary venous obstruction can be safely accomplished without cardiopulmonary bypass, and furthermore, two cases had an advantage of an efficient growth in the anastomotic site postoperatively.
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References
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Raisher B.D., Grant J.W., Martin T.C., Srauss A.W., Spray T.L. Complete repair of total anomalous pulmonary venous connection in infancy. J Thorac Cardiovasc Surg 1992;104:443-448.[Abstract]
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Cobanoglu A., Menasche V.D. Total anomalous pulmonary venous connection in neonates and young infants: repair in the current era. Ann Thorac Surg 1993;55:43-49.[Abstract]
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Lupinetti F.M., Kulik T.J., Beekman R.H., Crowley D.C., Bove E.L. Correction of total anomalous pulmonary venous connection in infancy. J Thorac Cardiovasc Surg 1993;106:880-885.[Abstract]
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Donato R.D., Carlo D., Squitieri C., et al. Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy. Ann Thorac Surg 1987;44:35-39.[Abstract]
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Caldarone C.A., Najm H.K., Kadletz M., et al. Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. Ann Thorac Surg 1998;66:1521-1526.[Abstract/Free Full Text]
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Gaynor J.W., Collins M.H., Rychik J., Gaughan J.P., Spray T.L. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1999;117:506-514.[Abstract/Free Full Text]
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Lamberti J.J., Waldman J.D., Methewson J.W., Kirkpatrick S.E. Repair of subdiaphragmatic total anomalous pulmonary venous connection without cardiopulmonary bypass. J Thorac Cardiovasc Surg 1984;88:627-630.[Abstract]
Related Article
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Invited commentary
- John J. Lamberti
Ann. Thorac. Surg. 2001 72: 251.
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Abstract
Introduction
