**Correction of Transposition of Great Arteries with Ventricular Septal Defect and Left Outflow Tract Obstruction with Double Arterial Translocation with Preservation of the Pulmonary Valve**

Gláucio Furlanetto and Beatriz H. S. Furlanetto *Furlanetto Institute Brazil* 

#### **1. Introduction**

Transposition of the great arteries (TGA) was first recognized by Mathew Bailie in 1797. The term transposition of the aorta and pulmonary artery (PA) was applied by Farre in 1814. Van Praagh proposed that TGA was one variety of malposition of the great arteries secondary to aberrations in conotruncal development. The other tipes of malposition include double-outlet right ventricle, double-outlet left ventricle, and anatomically corrected malposition. According the Congenital Heart Surgery Nomenclature and Database Project (James 2000), TGA always has discordant ventriculoarterial alignment such that the aorta arises entirely or in large party from the right ventricle (RV), the PA arises entirely or in large part above the left ventricle (LV) and concordant atrioventricular alignment is nearly always present. The term simple TGA has come to be used to denote those patients without associated ventricular septal defect (VSD) or left ventricular outflow tract obstruction (LVOTO) and nearly 75% of TGA patients have the simple type. Usually in the simple form of TGA the left ventricle doesn't has subpulmonary conus, so there is fibrous continuity between pulmonary and mitral valves, and tha aorta is anterior and to the right of the PA. The VSD occur in 40-45% of TGA patients and about 30% of these defects will be very small. LVOTO occurs in 25% of patients and is rare in patients with intact ventricular septum, ocurring in 5%. Approximated 30% of patients with TGA-VSD have LVOTO. A subvalvar LVOTO can be dynamic, localized fibrous ring, diffuse tunnel-like obstruction, muscular obstruction related to malposition of the outlet septum and LVOTO result from malposition of the mitral apparatus on the interventricular septum.

TGA is the second more common cyanotic congenital heart disease (CHD) and represents approximately 5-7% of all CHD and has a incidence of 20-30 in 100.000 live births, with a male preponderance of approximately 2:1. In patients with TGA, VSD and LVOTO early survival reachs 70% at one year and 29% at 5 years. Neonates with TGA, VSD and severe LVOTO have diminished pulmonary blood flow and they represents 5-8% of neonatal TGA population. Clinical findings are similar to those in the infants with tetralogy of Fallot with severe pulmonar stenosis.

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

without implant of pulmonary valve.

operation (Jatene, 1976) can be done.

the pulmonary valve (Furlanetto, 2010).

**surgical technique** 

homograft.

bovine jugular vein was used to enlarge the RV outflow tract.

valve was sutured into the right ventricular outflow [figure 3].

Tract Obstruction with Double Arterial Translocation with Preservation of the Pulmonary Valve 209

tissue, and the crista supraventricularis is transected anteriorly into the superior corner of the VSD. The ascending aorta is moved posteriorly and sutured to the posterior pulmonary annulus. The large anterior defect is now closed, from the margin of the VSD to the aortic root. A large pericardial patch is utilized to reconstruct the continuity of the RV outflow

The Nikaidoh procedure was modified by Hu (Hu, 2007). In this procedure the ascending aorta and the pulmonary trunk were transected and the coronary arteries were detached. The pulmonary root and the ascending aortic with the aorta valve were dissected out of ventricles. The subvalvar stenosis was relieved by resecting the conal septum, the VSD is closed and the detached ascending aorta with the aortic valve is translocated posteriorly and coronary arteries was reimplanted. After the Lecompte maneuver the pulmonary root is translocated anteriorly to the RV outflow. The pulmonary root is incised and a monocusp

Direct surgical relief of severe LVOTO depends on the anatomic type and severity of obstruction. In patients with mild LVOTO it can be resected and the Jatene arterial switch

Another surgical approach is the aortic root translocation plus arterial switch (Bautista-Hernandez, 2007). In this surgical technique proposed by del Nido a segment of the ascending aorta was transected from the right ventricle. The coronary arteries were excised as circular shape from the ascending aorta than the main pulmonary artery was transected. The ampliation of the LVOTO was made like the Nilaidoh procedure from the pulmonary annulus toward the VSD. The aortic autograft was sewn to the LV outflow and to the ascending aorta after the Lecompte maneuver. Reimplantation of the coronary arteries was performed into the neoaorta. The RV to pulmonary arteries continuity was established by a

We proposed a new approach for correction of TGA, VSD and LVOTO performing the double arterial translocation with preservation of the pulmonary valve. In this surgical technique the ascending aorta is translocated with the aortic valve and the coronary arteries to the left ventricle, after correction of left outflow tract obstruction and correction of the VSD, associated to pulmonary root translocation to the right ventricle, conserving integrally

**2. Double arterial translocation with preservation of the pulmonary valve,** 

This procedure was achieved employing cardiopulmonary bypass with hypothermia at 25°C and myocardial protection with warm induction blood cardioplegic solution at a proportion of 3:1 followed by hypothermic cardioplegic solution and modified ultrafiltration. Initially an incision inferior to the aortic valve was made, excising the ascending aorta (including aortic valve and coronary arteries) beneath the annular level of aortic valve from the right ventricle. Then we performed the excision of the pulmonary root, including pulmonary valve, beneath the annular level of the pulmonary valve from the left ventricle [figure 1]. After section of the infundibular septum in direction to the VSD, the LVOT and the VSD was closed with glutharaldeide-fixed bovine pericardium patch [figure 2]. The resulting gap of the aortic translocation was partially closed with fresh autologous pericardium. Finally the ascending aorta with the aortic valve and the coronary arteries were sutured into the left ventricle outflow and the pulmonary root with the pulmonary

The physiologic abnormalitie in TGA is that systemic and pulmonary circulations function in parallel rather in series as in normal infance, the consequence is deficiency of oxygen supply to the tissues. The arterial oxigen saturation and the extend of intercirculatory mixing dependents on the number, size and position of the anatomic communications like atrial septal defect (ASD), VSD and persistent ductus arteriosus.

Infants with TGA, VSD and LVOTO have been successfully palliated with systemic-topulmonary anastomosis with a politetrafluorethilene (PTFE) modified Blalock-Taussig shunt. In these patients the intracardiac correction is carried out at later age. The optimal age for the performance of a corretive repair remains controversial because of the balance between palliation and correction. Palliation leads to the performance of Rastelli repair at an older age with larger conduits, therefore reducing the need for reoperations. However, palliation also leads to LV overloading, and cyanosis.

The conventional surgical technique to repair TGA, VSD and LVOTO is the Rastelli operation (Rastelli, 1969). This operation was described with the theoretic advantage of incorporating the left ventricle as the systemic ventricle for correction of TGA. This technique achieves redirection of the left ventricular outflows and relieves pulmonary stenosis by bypassing it. The repair consists of: 1) repair of the VSD with a patch in such a way to connect the LV with the aorta using the closure of the right ventricle outflow as part of the left ventricular outflow, 2) division of the pulmonary artery and oversewn the cardiac end and 3) reconstruction of the pulmonary artery with a valved conduit. However, the Rastelli operation is far from ideal because it is not feasible in many patients because of unfavorable intracardiac anatomy, it requires the use of a prosthetic conduit for the reconstruction of the pulmonary outflow tract and the intraventricular tunnel used at Rastelli operation is not ideal because it can show some degree of stenosis at medium follow-up.

The Lecompte procedure, or ''reparation a l'etage ventriculaire'' (Borromée, 1988) is a surgical procedure that resects the infundibular septum to creates a large communication between the LV and the aorta. The aim of this resection is to construct a straight and direct aortic outflow tract to the aortic valve. The aorta is connected to the LV with a patch smaller than in the Rastelli operation and the tunnel between the LV and aorta is situated just beneath the aorta orifice and occupies very little space in the RV cavity. The pulmonary artery is transected above the pulmonary valve and is translocated onto the subaortic ventricular incision and the pulmonary outflow tract was completed with an anterior patch and a monocusp pericardial valve.

Double-outlet LV is conventionally correct with an intraventricular tunnel or with an extracardiac conduit when pulmonary stenosis is present. Pulmonary root translocation with the pulmonary valve to the RV is an important alternative surgical technique to correct this CHD (Chiavarelli, 1992).

This concept was used to correct TGA, VSD and LVOTO (Silva, 2000). In this procedure the pulmonary artery and its branches were dissected from the aorta and its posterior connections. The pulmonary root was dissected from the LV with the pulmonary valve. Than the VSD is closed, diverting the blood from the LV to the aorta like in the Rastelli operation and the pulmonary root is translocated to the RV. The follow-up of two children showed that the pulmonary root diameter can grown.

The Nikaidoh procedure (Nikaidoh, 1984) changed the concept to treat the LVOTO. In this procedure, after dissection of the aorta and the main pulmonary artery, the ascending aorta was totally mobilized with the aortic valve and the coronary arteries, the pulmonary artery is transected just above the pulmonary valve. The pulmonary annulus, subpulmonic fibrous

The physiologic abnormalitie in TGA is that systemic and pulmonary circulations function in parallel rather in series as in normal infance, the consequence is deficiency of oxygen supply to the tissues. The arterial oxigen saturation and the extend of intercirculatory mixing dependents on the number, size and position of the anatomic communications like

Infants with TGA, VSD and LVOTO have been successfully palliated with systemic-topulmonary anastomosis with a politetrafluorethilene (PTFE) modified Blalock-Taussig shunt. In these patients the intracardiac correction is carried out at later age. The optimal age for the performance of a corretive repair remains controversial because of the balance between palliation and correction. Palliation leads to the performance of Rastelli repair at an older age with larger conduits, therefore reducing the need for reoperations. However,

The conventional surgical technique to repair TGA, VSD and LVOTO is the Rastelli operation (Rastelli, 1969). This operation was described with the theoretic advantage of incorporating the left ventricle as the systemic ventricle for correction of TGA. This technique achieves redirection of the left ventricular outflows and relieves pulmonary stenosis by bypassing it. The repair consists of: 1) repair of the VSD with a patch in such a way to connect the LV with the aorta using the closure of the right ventricle outflow as part of the left ventricular outflow, 2) division of the pulmonary artery and oversewn the cardiac end and 3) reconstruction of the pulmonary artery with a valved conduit. However, the Rastelli operation is far from ideal because it is not feasible in many patients because of unfavorable intracardiac anatomy, it requires the use of a prosthetic conduit for the reconstruction of the pulmonary outflow tract and the intraventricular tunnel used at Rastelli operation is not

The Lecompte procedure, or ''reparation a l'etage ventriculaire'' (Borromée, 1988) is a surgical procedure that resects the infundibular septum to creates a large communication between the LV and the aorta. The aim of this resection is to construct a straight and direct aortic outflow tract to the aortic valve. The aorta is connected to the LV with a patch smaller than in the Rastelli operation and the tunnel between the LV and aorta is situated just beneath the aorta orifice and occupies very little space in the RV cavity. The pulmonary artery is transected above the pulmonary valve and is translocated onto the subaortic ventricular incision and the pulmonary outflow tract was completed with an anterior patch

Double-outlet LV is conventionally correct with an intraventricular tunnel or with an extracardiac conduit when pulmonary stenosis is present. Pulmonary root translocation with the pulmonary valve to the RV is an important alternative surgical technique to correct

This concept was used to correct TGA, VSD and LVOTO (Silva, 2000). In this procedure the pulmonary artery and its branches were dissected from the aorta and its posterior connections. The pulmonary root was dissected from the LV with the pulmonary valve. Than the VSD is closed, diverting the blood from the LV to the aorta like in the Rastelli operation and the pulmonary root is translocated to the RV. The follow-up of two children

The Nikaidoh procedure (Nikaidoh, 1984) changed the concept to treat the LVOTO. In this procedure, after dissection of the aorta and the main pulmonary artery, the ascending aorta was totally mobilized with the aortic valve and the coronary arteries, the pulmonary artery is transected just above the pulmonary valve. The pulmonary annulus, subpulmonic fibrous

atrial septal defect (ASD), VSD and persistent ductus arteriosus.

ideal because it can show some degree of stenosis at medium follow-up.

palliation also leads to LV overloading, and cyanosis.

and a monocusp pericardial valve.

showed that the pulmonary root diameter can grown.

this CHD (Chiavarelli, 1992).

tissue, and the crista supraventricularis is transected anteriorly into the superior corner of the VSD. The ascending aorta is moved posteriorly and sutured to the posterior pulmonary annulus. The large anterior defect is now closed, from the margin of the VSD to the aortic root. A large pericardial patch is utilized to reconstruct the continuity of the RV outflow without implant of pulmonary valve.

The Nikaidoh procedure was modified by Hu (Hu, 2007). In this procedure the ascending aorta and the pulmonary trunk were transected and the coronary arteries were detached. The pulmonary root and the ascending aortic with the aorta valve were dissected out of ventricles. The subvalvar stenosis was relieved by resecting the conal septum, the VSD is closed and the detached ascending aorta with the aortic valve is translocated posteriorly and coronary arteries was reimplanted. After the Lecompte maneuver the pulmonary root is translocated anteriorly to the RV outflow. The pulmonary root is incised and a monocusp bovine jugular vein was used to enlarge the RV outflow tract.

Direct surgical relief of severe LVOTO depends on the anatomic type and severity of obstruction. In patients with mild LVOTO it can be resected and the Jatene arterial switch operation (Jatene, 1976) can be done.

Another surgical approach is the aortic root translocation plus arterial switch (Bautista-Hernandez, 2007). In this surgical technique proposed by del Nido a segment of the ascending aorta was transected from the right ventricle. The coronary arteries were excised as circular shape from the ascending aorta than the main pulmonary artery was transected. The ampliation of the LVOTO was made like the Nilaidoh procedure from the pulmonary annulus toward the VSD. The aortic autograft was sewn to the LV outflow and to the ascending aorta after the Lecompte maneuver. Reimplantation of the coronary arteries was performed into the neoaorta. The RV to pulmonary arteries continuity was established by a homograft.

We proposed a new approach for correction of TGA, VSD and LVOTO performing the double arterial translocation with preservation of the pulmonary valve. In this surgical technique the ascending aorta is translocated with the aortic valve and the coronary arteries to the left ventricle, after correction of left outflow tract obstruction and correction of the VSD, associated to pulmonary root translocation to the right ventricle, conserving integrally the pulmonary valve (Furlanetto, 2010).
