Double-Decker Procedure for Partial Anomalous Pulmonary Venous Connection

The Warden procedure (1) has been performed for partial anomalous pulmonary venous connection in which the right upper pulmonary vein (RUPV) drains into the superior vena cava (SVC). This involves transecting the SVC and performing an end-to-end anastomosis with the right atrial appendage (RAA). However, the Warden procedure raises concerns regarding SVC stenosis and anastomotic stenosis. In response, the authors developed the double-decker procedure (2, 3, 4), which utilizes the upper outer wall of the SVC as the base of the systemic venous return pathway, creating a double-decker structure for both systemic and pulmonary venous return pathway. 
 
The patient was a 2-year-old male weighing 10.3 kg. Though median sternotomy, cardiopulmonary bypass was established. The venous cannulas were inserted into the SVC and the innominate vein, respectively. After cardiac arrest, an incision was made along the ridgeline of the right atrium (RA) from the right atrial appendage (RAA) toward the SVC-RA junction, stopping several millimeters short of the SVC-RA junction. The trabeculations within the RAA were excised as completely as possible. All procedures within the RA were performed through this incision, with no further incisions required. An autologous pericardial patch was sutured from the SVC opening to cover the venous sinus defect-type atrial septal defect (ASD), creating a pulmonary venous return pathway to the left atrium (LA).  

An inverted U-shaped incision was made on the upper wall of the SVC, opposite the azygos vein junction.  

This SVC upper wall flap was dropped into the SVC lumen and anastomosed to the SVC inner wall between the azygos vein opening and the RUPV opening, creating a septum within the SVC. To ensure smooth blood flow from the RUPV, the dropped SVC wall septum was anastomosed to the inner wall at a slight angle rather than perpendicular to the SVC longitudinal axis. This completed the pulmonary venous return pathway from the RUPV to the left atrium via the proximal SVC and the ASD.  

Aortic cross-clamping was released, and the heart resumed beating with normal sinus rhythm.  

The lower edge of the RAA incision was anastomosed to the anterior outer wall of the SVC, cranial to the sinus node artery. The left and right edges of the RAA incision were anastomosed to the respective lateral outer walls of the SVC, and then the distal end of the RAA opening was anastomosed to the distal end of the SVC incision. This completed the superior systemic venous return pathway. 
 
This technique avoids SVC stenosis because the SVC is not stretched. Furthermore, since there is no circumferential anastomotic site, anastomotic stenosis is also avoided. Since all repairs are performed using autologous tissue, there is potential for growth. The double-decker procedure is an excellent procedure applicable to both children and adults. In cases involving adults or adolescences, tension may develop on the right atrial wall when extending the RAA to the upper edge of the SVC incision. In such cases, a dome-shaped small patch may be added at the anastomotic tip (4). 

References

1. Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984;38:601-5.
2. Yamagishi M, Fujiwara K, Yaku H, Wada Y, Kitamura N. Repair of partial anomalous pulmonary venous connection with a minimal atriotomy. Jpn J Thorac Cardivascu Surg. 2000;48:370-2.
3. Hongu H, Yamagishi M, Maeda Y, Itatani K, Asada S, Fujita A, Yaku H. Double-decker repair of partial anomalous pulmonary venous return into the superior vena cava. J Thorac Cardiovasc Surg 2019;157:1970-7.
4. Masaaki Yamagishi. Partial Anomalous Pulmonary Venous Return. Surgical Treatment of Congenital Heart Disease. Understanding Morphology and Ultimate Three-Dimensional Reconstruction. Springer, 2025

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