Trend Management Patient of Transposition Great Artery

dr. Radityo Prakoso, SpJP(K), FAPSIC

The most common form of cyanotic congenital heart disease (CHD) that usually
presents in the newborn period is transposition of the great arteries (TGA). TGA
comprises approximately 5% of all CHD. The prevalence of TGA during 2004-2006
is 3 per 10.000 live births. The incidence of TGA is 218 per million live births. TGA
can be caused for some factors, include genetic and noninherited / environmental risk
factors. Transposition of the great arteries is predominately seen in males, but rarely
associated with chromosomal abnormalities. The recurrence rate was found to be
1.7% in siblings. Pregestational diabetes is positively associated with TGA. High
maternal intake of vitamin A may significantly increase the risk of TGA. Maternal
exposure to ibuprofen, influenza, organic solvents, and pesticides have been reported
to increase the risk specifically for TGA, in addition to other CHD.

Patients with TGA almost have situs solitus, levocardia, concordant atrioventricular
connections, and right-hand ventricular topology (D-TGA or D-looped). The most
common associated lesions in TGA are the presence of a ventricular septal defect
(VSD), with or without additional presence of left ventricular outflow tract (LVOT)
obstruction. A common type of VSD encountered in TGA is a perimembranous VSD.
Another common type of VSD is the so-called malalignment type, characterized by
the malalignment between the outlet septum and the remainder of the muscular
ventricular septum. Malalignment of the outlet septum can occur posteriorly toward
the pulmonary outflow tract, and in this manner is responsible for one of the common
mechanisms of LVOT obstruction. A less common type of VSD in TGA is the
muscular defect. The independent occurrence of VSD and LVOT obstruction results
in four possible combinations :
1. TGA with intact ventricular septum (TGA-IVS)
2. TGA with VSD (TGA-VSD)
3. TGA with VSD and LVOT obstruction
4. TGA-IVS and LVOT obstruction (rarest form).

The physiology in TGA is more complex than that of simple left-to-right or right-to-
left shunts. The essence of the circulatory derangement is that the systemic and
pulmonary circulations are arranged in parallel rather than in series. The right
ventricle would be pumping blood that is essentially devoid of oxygen, while the left
ventricle keeps recirculating fully oxygenated blood to, and from, the lungs. The
levels of communication that allow blood to cross from one circuit to the other can
occur either centrally at the atrial (atrial septal defect or patent foramen ovale),
ventricular (VSD), or great artery level (patent ductus arteriosus), or can occur
peripherally at the naturally existing shunts at the level of the bronchial circulation
and thebesian veins.

The clinical features in TGA depends on the anatomic details of the lesion and the age
of presentation, which in most cases is the newborn period, but in developing
countries, late presentation can occur. Newborn with TGA-IVS present with cyanosis.
In the presence of a large VSD, pulmonary overcirculation or heart failure may
ensue as the pulmonary vascular resistance drops, and is manifested by tachypnea.
Patients with LVOT obstruction and VSD may present with profound cyanosis and
lethargy.

From physical examination, cyanosis is the common abnormality. Reverse differential

cyanosis (oxygen saturation higher in the lower extremities than upper extremities)
may be present in newborns with TGA and PDA with pulmonary hipertension or
coarctation of the aorta/interrupted aortic arch. In neonates with TGA-IVS, there is
usually little or no murmur heard as the pulmonary vascular resistance is relatively
high in the newborn period and the PDA is usually large. In the presence of large
VSD, a harsh holosystolic murmur / pansystolic murmur is heard at the left lower
sternal border along with a diastolic rumble at the apex. It is the evident of pulmonary
vascular resistance drops. In the presence of LVOT obstruction, a harsh systolic
ejection murmur of varying intensity depending on the degree of obstruction is heard
at the left sternal border.

The electrocardiogram (ECG) demonstrates right atrial enlargement (RAE) and right
ventricular hypertrophy (RVH). Biventricular hypertrophy (BVH) may be seen
particularly in cases of LVOT obstruction or significant left ventricular volume
overload.

In chest rontgen examination, the TGA where the aorta and pulmonary trunk exhibit a
direct anterior-posterior relationship, the mediastinal shadow is narrow and not as
prominent as usual, leading to the classic egg on a string appearance of the cardiac
silhouette.

Newborns with TGA-IVS may need to be intubated to control their airway due to
cardiorespiratory compromise or severe hypoxemia. Supplemental oxygen should be
administered and correction of metabolic derangements and acidosis should be
performed. Prostaglandin E1 infusion is critical for adequate mixing in these patients.
Low-dose prostaglandin E1 infusion at a rate of 0.0125 g/kg/min should be started.
This allows adequate patency of the duct (PDA) while at the same time enabling a
natural airway to be maintained without apnea and resultant intubation.

Ultimately, most patients will require a balloon atrial septostomy (BAS) soon after
diagnosis for adequate mixing to occur. Performing a BAS has the advantage of
enabling the prostaglandin E1 infusion to be discontinued and allowing the baby to
feed prior to the eventual operation. It is also important to realize that in some patients
with TGA-VSD with/without LVOT obstruction, adequate mixing may not occur at
the ventricular level. In some of these newborns, a BAS may need to be performed to
promote adequate mixing at the atrial level.

The procedure can be performed with or without intubation, depending on the clinical
status of the baby. Access can be obtained via the femoral vein or umbilical vein. The
femoral vein allows more force to be applied (and thus a larger defect to be created)
than the umbilical venous route by virtue of the longer distance from the end of the
sheath to the atrial septum. The main advantage of the umbilical venous route is that a
relatively large sheath can be placed in extremely small babies.

Several different balloon catheters have been used. The most common balloon
catheters used currently are the MillerEdwards (Miller) catheter and the NuMed Z-5
septostomy catheter. The Miller catheter requires a 7 F introducer sheath and does not
have a wire port. It can accommodate 4 cc of fluid in the balloon. There are two types
of the Z-5 septostomy catheters. One is a 13.5-mm balloon requiring a 6 F sheath and
is able to accommodate 2 cc of fluid; the other is a 9.5-mm balloon requiring a 5 F
sheath and able to accommodate 1 cc of fluid. Both of the Z-5 septostomy catheters
have the advantage of going over wires, should difficulty be encountered in crossing
the ASD. The smaller balloon is ideal when needed in very tiny premature newborns.
The newer Z-5 septostomy catheters have shown to be similarly effective in achieving
a desirable result, is safe and especially useful in smaller neonates

The balloon is first prepared to ensure no air is inside the balloon, by inflating and
deflating the balloon with saline (or contrast) prior to advancing it into the body. Care
should be taken to avoid air emboli during the introduction of the catheter through the
sheath; flushing the side port of the sheath during introduction is helpful. It is then
advanced into the left atrium and inflated. Care is taken to make sure that the balloon
is away from the left AV valve, left atrial appendage, and pulmonary veins. Once
fully inflated, it is rapidly pulled back across the atrial septum to the right atrium in a
controlled fashion. Care is taken not to withdraw the balloon rapidly back into the
inferior vena cava. The balloon is then deflated. This maneuver can be repeated as
necessary. After successful septostomy, no more restriction to withdrawing the
balloon is felt on subsequent pullbacks. A successful procedure is confirmed by
transthoracic echocardiography.

Complications are rare but can occur. These include damage to the cardiac and
vascular structures (e.g., atrioventricular valves/left atrial appendage/pulmonary
veins/hepatic veins/inferior vena cava), pericardial effusion, access site trauma, and
air embolism. Meticulous attention to procedural details limits the occurrence of
complications. Regardless, balloon atrial septostomy is a key and essential component
in the care of infants with TGA.

Refference :
Qureshi AM, Justino H, Heinle JS. transposition of the great arteries. In : Allen HD, Shaddy RE, Penny
DJ, Feltes TF, Cetta F. Moss and Adams heart disease in infants, children, and adolescents : including
the fetus and young adult. 9th ed. Wolters Kluwer; 2016. p. 1163-86.