If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
En-bloc Rotation of the Truncus Arteriosus—A Technique for Complete Anatomic Repair of Transposition of the Great Arteries/Ventricular Septal Defect/Left Ventricular Outflow Tract Obstruction or Double Outlet Right Ventricle/Left Ventricular Outflow Tract Obstruction
The Rastelli procedure has been traditionally used for repair of transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO), or double outlet right ventricle with subpulmonary VSD and LVOTO.
The Rastelli procedure in its original version involves an intracardiac baffle, which forms the left ventricular outflow tract from the VSD to the aortic valve. This results in a long intracardiac tunnel and has an inherent tendency to obstruction. A homograft or xenograft is also needed for reconstruction of the right ventricular outflow tract. Resection of the conal septum and direct anastomosis between the main pulmonary artery and the right ventricle as in the Lecompte procedure,
or reparation a l'etage ventriculaire (REV), can minimize these problems but does not completely solve them. Although there is growth potential in the pulmonary artery after a REV procedure, there is no valve in the right ventricular outflow tract. Therefore, one can expect similar problems as follow tetralogy repair with a transannular patch.
Aortic translocation (Nikaidoh procedure) brings the aorta directly over the left ventricular outflow tract.
The pulmonary valve is also removed in this procedure. In its original version, it does not place a valve in the right ventricular outflow tract and does not include a backwall to the pulmonary artery. In many patients, the right coronary artery has to be transferred anteriorly, as reported by Dr. Nikaidoh himself, as well as other authors.
In many patients suitable for the Rastelli, REV, or Nikaidoh procedures, the stenosis in the left ventricular outflow tract is subvalvar. The pulmonary valve is often bicuspid or dysplastic. However, the valve may be appropriate for use in the right ventricular outflow tract. En-bloc rotation of the complete truncus arteriosus (the aortic and pulmonary roots) enables one to bring the aortic root over the left ventricular outflow tract, and the pulmonary root over the right ventricular outflow tract. This procedure was first published by Yamagishi and coworkers in a case report in 2003.
The pulmonary valve was either bicuspid or dysplastic but competent and with a sufficient orifice. The remaining two patients in our series required a pulmonary transannular patch. Subvalvar enlargement of the pulmonary root was usually adequate to relieve LVOTO. The coronary anatomy is critical in this procedure. An important coronary artery crossing the right ventricular outflow tract close to the aortic root makes this procedure impossible.
The procedure begins with a median sternotomy, partial resection of the thymus, and opening of the pericardium by harvesting a pericardial patch. The anatomy is assessed carefully. The coronaries are inspected. To date, we have done this procedure only if the coronary anatomy is usual for transposition. We do not do it if the circumflex coronary artery originates from the right coronary. A major coronary artery crossing the right ventricular outflow tract close to the aortic root (right coronary artery from left anterior descending coronary artery, or left anterior descending coronary artery from right coronary artery) is also a contraindication to this procedure. Cardiopulmonary bypass is instituted. Arterial cannulation is done in the ascending aorta or proximal aortic arch. Bicaval venous cannulation is used. A left atrial vent is inserted. A line for cardioplegia is put into the ascending aorta at the place of the planned transection, as in an arterial switch procedure.
To date, our results with this procedure are very encouraging. Since 2003 we have a series of eight patients. All of them survived the procedure. All patients have growth potential in the reconstructed right and left ventricular outflow tracts. Two patients came to us via a human aid organization from Albania, so they are not in our follow-up program. None of the six patients who have been followed for up to 5 years has a gradient in the left ventricular outflow tract. One patient has an echocardiographic gradient across the pulmonary valve of approximately 40 mmHg. This has not increased over the last 3 years. Another patient shows a gradient at the pulmonary bifurcation of 36 mm Hg. To date she has not needed any intervention. One patient needed a permanent pacemaker for second-degree heart block 2 months after the operation.
Complete repair of transposition of the great arteries with pulmonary stenosis.