**4. Comments**

The surgical management of TGA with VSD and LVOTO is a surgical challenge. The Rastelli operation remains the most applied procedure for this congenital cardiopathy.

The most appropriate timing of the Rastelli operation is controversial. When it is performed during early infancy, it is a physiologic correction and avoids systemic hypoxemia. On the other hand, the palliative procedure of modified BT shunt performed during early infancy avoids early reoperation to change the valved conduit.

Correction of Transposition of Great Arteries with Ventricular Septal Defect and Left Outflow

hypoplasia and the valve could be bicuspid.

growth.

**5. Conclusion** 

**6. References** 

valve.

Tract Obstruction with Double Arterial Translocation with Preservation of the Pulmonary Valve 215

fibromuscular or a muscular obstruction. The valvar stenosis is caused by annular

The use of prosthetic valves and of a connection without valve in the pulmonary position in children brings about dysfunction of the right ventricle on medium/late-term follow up. Based on this fact, the preservation of the native pulmonary valve with mild to moderate residual gradient has been accepted in the correction of tetralogy of Fallot by Voges (Voges 2008), who admit the size of the pulmonary valve with z-score superior to -4. This concept can also be used in the conservation of the pulmonary valve performed at double arterial translocation with preservation of the pulmonary valve. It is possible that the pulmonary root could grow and the use of this surgical technique could diminish reoperation rates in these patients, but a late follow-up will be necessary to verify the potential of valve

In one child submitted to double arterial translocation with preservation of the pulmonary valve the preoperative color doppler echocardiography revealed a LVOTO gradient of 40mmHg. The transvalvar gradient between the right ventricle and the pulmonary trunk measured in the operating room after surgical correction was 25mmHg, and the postoperative doppler echocardiography revealed a pulmonary transvalvar gradient of 42mmHg. In another child, submitted to the same procedure, the preoperative color doppler echocardiography revealed a LVOTO gradient of 65mmHg. The transvalvar gradient between the right ventricle and the pulmonary trunk measured in the operating room after surgical correction was 30mm Hg and the postoperative doppler echocardiography revealed a pulmonary transvalvar gradient of 31mmHg. Heart computadorized angio-tomography performed during the immediate postoperative period showed appropriate positioning of the pulmonary trunk, the pulmonary artery and the aorta in both patients [Figure 4]. One third patient with preoperative Doppler echocardiography revealed a LVOT gradient of 31 mmHg. The pressure measurement after surgical correction revealed RV/LV systolic

We can conclude that there was an evolution in the approach of TGA, VSD and LVOTO. The modified BT shunt operation, the operation of Rastelli and more recently the Jatene operation, modified Nikaidoh and the double translocation with preservation of the pulmonary valve were responsible for part of this evolution. In spite of the increase of surgical difficulty, there was no significant difference in the mortality. A late follow-up of a larger series of children will be important to check the potential development of the pulmonary valve in the double arterial translocation with preservation of the pulmonary

Jaggers, J. J.; Cameron, D. E.; Herlong, R. Et al. (2000). Congenital Heart Surgery

Rastelli, C. ; Wallace, R.B. & Ongley, A. (1969). Complete repair of transposition of the great

a new surgical technique. *Circulation*, Vol.39, (Jannuary 1969), pp. 83-95.

Nomenclature and Database Project: transposition of the great arteries. *Ann Thorac* 

arteries with pulmonary stenosis. A review and report of a case corrected by using

pressure of 0.7 and pulmonary transvalvular gradient of 30 mmHg.

*Surg*, Vol 69 (3) suppl 1, PP. 205-235.

There is a significant incidence of late mortality after Rastelli procedure, with survival rate of 82% at 5 years and 52% at 20 years. In 25 years of experience, Kreutzer noticed that the most common cause of late death was left ventricular failure, present in 25% of patients (Kreutzer, 2000). The intraventricular tunnel from left ventricle to aorta is tortuous and can develop stenosis in the late follow-up, which can explain the left ventricle disfunction. The presence of valved conduit dysfunction and right ventricular dysfunction may be associated with ventricular tachycardia and sudden cardiac death. Therefore, all patients submitted to Rastelli procedure will need to change the right ventricle to pulmonary artery conduit once. In the Lecompte procedure, the resection of the infundibular septum creates a straight, direct tunnel between the left ventricle to aorta occupying little space in the right ventricle outflow tract, in order to improve the intraventricular tunnel from the left ventricle to aorta. This procedure presents a better solution to the left ventricle outflow tract reconstruction, but in the right ventricle outflow the monocuspid pericardial valve used will develop some dysfunction on medium-term follow-up. The percentage of reoperation in patients submitted to LeCompte procedure is lower when compared to Rastelli procedure. A very interesting option to correct TGA, VSD and LVOTO is the Nikaidoh procedure. In this procedure, aortic translocation with the aortic valve and coronary arteries to the left ventricle, after enlargement of the left ventricular outflow and closure of the VSD with a single patch, creates an anatomical left ventricle outflow, but reconstruction of the right ventricle outflow without the pulmonary valve will cause right ventricle dysfunction. The Hu procedure utilizes the aortic translocation like the Nikaidoh procedure but translocating the pulmonary root and amplificating the pulmonary valve with a bovine jugular vein monocuspid to the right ventricle after performing the LeCompte maneuver performed to reconstruction of the right ventricle outflow. This procedure corrects the left ventricle outflow in an anatomical way, but the amplification of the pulmonary valve with a bovine jugular vein monocusp will certainly cause dysfunction of the pulmonary valve at mediumterm follow-up. The use of Silva procedure could avoid the reoperation to change the valved conduit. In this operation the pulmonary root is translocated with the pulmonary valve to the ventriculotomy on the right ventricle with expansion of the pulmonary root after dissection from the right ventricle. There is also the possibility of growth, because all the structures of the pulmonary root were preserved, but the correction of the left ventricle outflow tract is done like the Rastelli procedure with all disadvantages of this procedure. The surgery proposed by del Nido translocate a segment of the ascending aorta with anastomoses of the coronary arteries after enlargement of the left ventricle outflow tract employing a homograft to reconstruct the right ventricle outflow tract. Like in the Nikaidoh and Hu procedures the left outflow tract is anatomical but the use of a homograft at the right side will need change of the graft at medium follow-up.

The surgical technique that we performed, the double arterial translocation with preservation of the pulmonary valve, differs from all the other techniques used up to now because it corrects LVOTO through the aorta translocation with the aortic valve and coronary arteries after enlargement of the left outflow tract and closure of the VSD with a patch and corrects the right ventricle outflow with pulmonary root translocation with entire pulmonary valve to the right ventricle without the LeCompte maneuver and without right ventricolotomy. We believed that this procedure can be performed in patients with TGA, VSD and LVOTO when the pulmonary valve has an adequate annular diameter and the leaflets thickness is not too exacerbated. In these cardiopathies the LVOTO is subvalvar and valvar. The subvalvar stenosis is in the form of a localized fibrous ring, a tunnel-type fibromuscular or a muscular obstruction. The valvar stenosis is caused by annular hypoplasia and the valve could be bicuspid.

The use of prosthetic valves and of a connection without valve in the pulmonary position in children brings about dysfunction of the right ventricle on medium/late-term follow up. Based on this fact, the preservation of the native pulmonary valve with mild to moderate residual gradient has been accepted in the correction of tetralogy of Fallot by Voges (Voges 2008), who admit the size of the pulmonary valve with z-score superior to -4. This concept can also be used in the conservation of the pulmonary valve performed at double arterial translocation with preservation of the pulmonary valve. It is possible that the pulmonary root could grow and the use of this surgical technique could diminish reoperation rates in these patients, but a late follow-up will be necessary to verify the potential of valve growth.

In one child submitted to double arterial translocation with preservation of the pulmonary valve the preoperative color doppler echocardiography revealed a LVOTO gradient of 40mmHg. The transvalvar gradient between the right ventricle and the pulmonary trunk measured in the operating room after surgical correction was 25mmHg, and the postoperative doppler echocardiography revealed a pulmonary transvalvar gradient of 42mmHg. In another child, submitted to the same procedure, the preoperative color doppler echocardiography revealed a LVOTO gradient of 65mmHg. The transvalvar gradient between the right ventricle and the pulmonary trunk measured in the operating room after surgical correction was 30mm Hg and the postoperative doppler echocardiography revealed a pulmonary transvalvar gradient of 31mmHg. Heart computadorized angio-tomography performed during the immediate postoperative period showed appropriate positioning of the pulmonary trunk, the pulmonary artery and the aorta in both patients [Figure 4]. One third patient with preoperative Doppler echocardiography revealed a LVOT gradient of 31 mmHg. The pressure measurement after surgical correction revealed RV/LV systolic pressure of 0.7 and pulmonary transvalvular gradient of 30 mmHg.
