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Ann Thorac Surg 2008;86:160. doi:10.1016/j.athoracsur.2008.04.088
© 2008 The Society of Thoracic Surgeons

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Original Articles: Pediatric Cardiac

Invited Commentary

Hideki Uemura, MD, FRCS

Department of Cardiothoracic Surgery, Royal Brompton Hospital, Sydney St, London SW3 6NP, United Kingdom

(Email: huemura-cvs{at}umin.ac.jp).

Reports regarding repair of partial anomalous pulmonary venous connection (PAPVC) primarily present surgical procedures and results with short-term or intermediate-term follow-up. In this respect, the present study of superior sinus venosus defect by Luciani and colleagues [1] is informative. This defect is often discussed under the heading of secundum atrial septal defect (ASD), because of equivalent physiology. From a morphologic view, this malformation is regarded as deficient septation at the veno-atrial junction between the right superior pulmonary veins, superior vena cava, and the atria, and not just at the atrial level. It is not always obvious whether the PAPVC connects to the superior vena cava (SVC) or the right atrium (RA). Often the combined caval–pulmonary venous component is larger than the normal SVC–RA junction. This feature obviously has surgical relevance.

As for operative techniques, postoperative pulmonary venous obstruction is unlikely when the proximal veno-atrial junction is used as the pulmonary venous pathway in the so-called Warden procedure. However, reconstruction of the right SVC needs more attention. Because the transected SVC may be mildly twisted after anastomosis to the RA appendage, this situation is somehow different from a bidirectional cavopulmonary anastomosis. Another complicating factor is the presence of a left SVC. In such a circumstance, the right SVC is structurally smaller than normal, and therefore, the amount of blood flow across the reconstructed channel is less. Occlusion may eventually develop. Persistent left SVC may also be an issue when creating an intra-SVC–RA baffle ("single patch method"), because the right superior veno-atrial junction may not be large enough. An augmentation patch for closing the lateral atrial incision (described as the "two-patch method") may be a wise choice.

A lateral incision has two implications regarding rhythm. The incision may cut the sinus nodal artery if it transverses the right–posterior wall of the veno-atrial junction. Admittedly, this course is less common in PAPVC because of the abnormal anatomy. Another beneficial point is that a lateral incision creates the least likelihood of a re-entrant scar. By leaving space between the atriotomy and the inferior vena cava, slow conduction across the isthmus can be avoided. Upward extension of the incision reaches the venous component where atrial musculature is no longer present. Thus, atrial enlargement and histologic tissue damage to the RA is related to prolonged volume-overload and is responsible for postoperative atrial tachyarrhythmia. Therefore, age at the time of repair is an important factor.


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  1. Luciani GB, Viscardi F, Pilati M, Crepaz R, Faggian G, Mazzucco A. Age at repair affects the very long-term outcome of sinus venosus defect Ann Thorac Surg 2008;86:153-160.[Abstract/Free Full Text]

Related Article

Age at Repair Affects the Very Long-Term Outcome of Sinus Venosus Defect
Giovanni Battista Luciani, Francesca Viscardi, Mara Pilati, Roberto Crepaz, Giuseppe Faggian, and Alessandro Mazzucco
Ann. Thorac. Surg. 2008 86: 153-159. [Abstract] [Full Text] [PDF]




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Hideki Uemura
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Right arrow Congenital - acyanotic
Right arrowRelated Article


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