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Eur J Cardiothorac Surg 2002;21:474-477
© 2002 Elsevier Science NL

Totally anomalous pulmonary venous connection directly to the superior caval vein

^a Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK
^b Institute of Child Health, University College London, London WC1N 1EH, UK

Received 6 August 2001; received in revised form 22 November 2001; accepted 17 December 2001.

* Corresponding author. Tel.: +44-20-7404-4383; fax: +44-20-7831-4931
e-mail: delevm{at}gosh.nhs.uk

Objective: Totally anomalous pulmonary venous connection directly to the superior caval vein is unusual. It is frequently associated with major congenital heart defects such as the syndrome of right isomerism. While improved results have been reported recently for the isolated form, complex cases are still associated with a higher mortality. Methods: We undertook surgical correction in nine patients with direct pulmonary venous connection to the superior caval vein investigated in our institution from 1991 to 1999. In four of these patients, the venous anomaly was an isolated finding, while five patients with isomeric right atrial appendages had associated cardiac malformations rendering them unsuitable for biventricular repair. In one patient with an isolated form, the venous drainage was obstructed. Two patients with isomerism had previously undergone construction of an aortopulmonary shunt. Other associated surgical procedures in the patients with right isomerism were establishment of bidirectional cavo-pulmonary anastomoses in four cases and banding of the pulmonary trunk in one. Results: There were neither early nor late deaths. Reoperation was needed in one patient because of pulmonary venous stenosis. In the five patients with right isomerism, two later underwent successful creation of the Fontan circulation. Conclusion: It is unusual to find direct drainage of all the pulmonary veins to the superior caval vein. When seen, the venous pathway is only rarely obstructed. For this reason, when associated with right isomerism, an aortopulmonary shunt should be constructed as initial palliation, with later repair of the anomalous pulmonary venous drainage at the time of construction of a bidirectional cavo-pulmonary anastomosis. When using this policy, the surgical results can be as good for the complicated variant as for the isolated form.

Key Words: Right isomerism • Asplenia syndrome • Visceral heterotaxy • Surgical correction

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