HEART CONDITIONS

Diseases and Treated

Dextro-Transposition of the Great Arteries

What Is
Dextro-Transposition of the Great Arteries?
Dextro-Transposition of the Great Arteries (D-Transposition of the Great Arteries, Dextro-TGA, or D-TGA). Is a birth defect in the large arteries of the heart. The primary arteries (the aorta and the pulmonary artery) are transposed. It is called a cyanotic congenital heart defect (CHD) because the newborn infant turns blue from lack of oxygen. In segmental analysis, this condition is described as ventriculoarterial discordance with atrioventricular concordance, or just ventriculoarterial discordance. d-TGA is often referred to simply as transposition of the great arteries (TGA); however, (TGA) is a more general term which may also refer to Levo-Transposition of the Great Arteries (l-TGA).
Another term commonly used to refer to both d-TGA and l-TGA is transposition of the great vessels (TGV), although this term might have an even broader meaning than TGA.

Prenatally, a baby with d-TGA experiences no symptoms as the lungs will not be used until after birth, and oxygen is provided by the mother via the placenta and umbilical cord; in order for the red blood to bypass the lungs in utero, the fetal heart has two shunts that begin to close when the newborn starts breathing; these are the foramen ovale and the ductus arteriosus. The foramen ovale is a hole in the atrial septum which allows blood from the right atrium to flow into the left atrium; after birth, the left atrium will be filled with blood returning from the lungs and the foramen ovale will close. The ductus arteriosus is a small, artery-like structure which allows blood to flow from the trunk of the pulmonary artery into the aorta; after birth, the blood in the pulmonary artery will flow into the lungs and the ductus arteriosus will close. Sometimes these shunts will fail to close after birth; these defects are called patent foramen ovale and patent ductus arteriosus, and either may occur independently, or in combination with one another, or with d-TGA or other heart and/or general defects.


What Causes Dextro-Transposition of the Great Arteries?
- The causes of congenital heart defects, such as d-TGA, among most babies are unknown.
- Some babies have congenital heart defects because of changes in their genes or chromosomes.
- Heart defects are also thought to be caused by the combination of genes and other risk factors such as things the mother comes in contact whit in her environment, or what the mother eats or drinks, or certain medications she uses.
What are the Signs & Symptoms of
Dextro-Transposition of the Great Arteries?
Due to the low oxygen saturation of the blood, cyanosis will appear in peripheral areas:
Around the mouth and lips, fingertips, and toes; these areas are furthest from the heart, and since the circulated blood is not fully oxygenated to begin with, very little oxygen reaches the peripheral arteries. A d-TGA baby will exhibit indrawing beneath the ribcage and "comfortable tachypnea" (rapid breathing); this is likely a homeostatic reflex of the autonomic nervous system in response to hypoxic hypoxia.
The infant will be easily fatigued and may experience weakness, particularly during feeding or playing; this interruption to feeding combined with hypoxia can cause failure to thrive.
If d-TGA is not diagnosed and corrected early on, the infant may eventually experience syncopic episodes and develop clubbing of the fingers and toes.


HEART CONDITIONS Diseases and Treated FAQ's
D-TGA can sometimes be diagnosed in utero with an ultrasound after 18 weeks gestation.
However, if it is not diagnosed in utero, cyanosis of the newborn (blue baby) should immediately indicate that there is a problem with the cardiovascular system. Normally, the lungs are examined first, then the heart is examined if there are no apparent problems with the lungs. These examinations are typically performed using ultrasound, known as an echo-cardiogram when performed on the heart.
Chest X-rays and electrocardiograms (EKG) may also be used in reaching or confirming a diagnosis; however, an X-ray may appear normal immediately following birth. If d-TGA is accompanied by both a VSD and pulmonary stenosis, a systolic murmur will be present. On the rare occasion (when there is a large VSD with no significant left ventricular outflow tract obstruction), initial symptoms may go unnoticed, resulting in the infant being discharged without treatment in the event of a hospital or birthing center birth, or a delay in bringing the infant for diagnosis in the event of a home birth.
On these occasions, a layperson is likely not to recognize symptoms until the infant is experiencing moderate to serious congestive heart failure (CHF) as a result of the heart working harder in a futile attempt to increase oxygen flow to the body; this overworking of the heart muscle eventually leads to hypertrophy and may result in cardiac arrest if left untreated.
If the diagnosis is made in a standard hospital or other clinical facility, the baby will be transferred to a children's hospital, if such facilities are available, for specialized paediatric treatment and equipment.
- The patient will require constant monitoring and care in an intensive care unit (ICU),
- Palliative,
- Palliative treatment is normally administered prior to corrective surgery in order to reduce the symptoms of d-TGA (and any other complications), giving the newborn or infant a better chance of surviving the surgery. Treatment may include any combination of: - Minor Surgery,
- Cardiac Catheterization,
- Rashkind balloon atrial septostomy,
- Balloon angioplasty,
- Endovascular stenting,
- Angiography,
- This is a dynamic list and may never be able to satisfy particular standards for completeness. You can help by expanding it with reliably sourced entries,
- Cardiac catheterization is a minimally invasive procedure which provides a means of performing a number of other procedures,
- A balloon atrial septostomy is performed with a balloon catheter, which is inserted into a patent foramen ovale ( PFO ), or atrial septal defect ( ASD ) and inflated to enlarge the opening in the atrial septum; this creates a shunt which allows a larger amount of oxygenated ("red") blood to enter the systemic circulation,
- Angioplasty also requires a balloon catheter, which is used to stretch open a stenotic vessel; this relieves restricted blood flow, which could otherwise lead to congestive heart failure (CHF),
- An endovascular stent is sometimes placed in a stenotic vessel immediately following a balloon angioplasty to maintain the widened passage,
- Angiography involves using the catheter to release a contrast medium into the chambers and/or vessels of the heart; this process facilitates examining the flow of blood through the chambers during an echocardiogram, or shows the vessels clearly on a chest x-ray, MRI, or CT scan - this is of particular importance,
as the coronary arteries must be carefully examined and "mapped out" prior to the corrective surgery,
- It is commonplace for any of these palliations to be performed on a d-TGA patient,
- Moderate Left anterior thoracotomy,
- Isolated pulmonary artery banding (PAB),
- PAB (when coarctation or aortic arch repair also required),
- Right lateral thoracotomy,
- Blalock-Hanlon atrial septectomy,
- This is a dynamic list and may never be able to satisfy particular standards for completeness. You can help by expanding it with reliably sourced entries,
- Each of these procedures is performed through an incision between the ribs and visualized by echocardiogram; these are far less common than heart cath procedures,
- Pulmonary artery banding is used in a small number of cases of d-TGA, usually when the corrective surgery needs to be delayed, to create an artificial stenosis in order to control pulmonary blood pressure; PAB involves placing a band around the pulmonary trunk, this band can then be quickly and easily adjusted when necessary,
- An atrial septectomy is the surgical removal of the atrial septum; this is performed when a patent foramen ovale ( PFO ), or atrial septal defect ( ASD ) are not present and additional shunting is required to raise the oxygen saturation of the blood flowing eventually into the aorta,
- Major or Median sternotomy,
- PAB (when intracardiac procedures also required),
- Concomitant atrial septectomy,
- This is a dynamic list and may never be able to satisfy particular standards for completeness. You can help by expanding it with reliably sourced entries,
- In recent years, it is quite rare for palliative procedures to be done via median sternotomy. However, if a sternotomy is required for a different procedure, in most cases all procedures that are immediately required will be performed at the same time,
- Nasogastric tube (NG tube or simply NG),
- Intubation, oxygen mask, or nasal cannula,
- Intravenous drip (IV),
- Arterial line,
- Central venous catheter,
- Fingerprick,
- Sphygmomanometer,
- Pulse oximeter,
- EKG,
- Corrective,
- Nikaidoh,
- It was Bex who introduced in 1980 the possibility of aortic translocation. But Nikaidoh has put the procedure in practice in 1984.,
It results in an anatomical normal heart, even better than with an ASO, because also the cones are switched instead of only the arteries as with an ASO. The procedure is contra-indicated by certain coronary anomalies,
- In 1984, Nikaidoh introduced a surgical approach for the management of TGA, VSD, and pulmonary stenosis (PS), which he called “aortic translocation and biventricular outflow tract reconstruction.” The repair consisted of harvesting the aortic root from the right ventricle, with or without the coronary arteries attached, and relieving the LVOTO by dividing the outlet septum and pulmonary valve annulus. The LVOT is then restored by posteriorly translocating the aortic root and closing the VSD. Finally, the right ventricular outflow tract is reconstructed with a pericardial patch. This is a technically challenging procedure but results in a more “normal” anatomic repair. The main thing is the repositioning of the native aortic root over to the LV cavity, avoiding the creation of a long tortuous intraventricular tunnel. This technique appears to prevent the development of LVOTO, which is a frequent complication of the Rastelli repair. The addition of the Lecompte maneuver may prevent branch pulmonary artery stenosis that may occur secondary to compression of the PA by the posteriorly displaced, translocated aortic root. It creates a direct RV to PA anastomosis and avoiding the use of a conduit, which should decrease the incidence of RVOT reinterventions,
- Lecompte,
- Since 1981 LeCompte has put his LeCompte manoeuvre in use. This is used with the REV (Réparation à l'Etage Ventriculaire). This surgery is like the Rastelli procedure, but with the use of the pulmonary artery without a conduit,
- Rastelli,
- When an arterials switch operation (ASO) is not possible e.g. in case of LVOTO an option is the Rastelli procedure. The pulmonal artery is shifted with help of conduit to the right ventricle. It has been used since 1960s. It has a disadvantage that the conduit does not grow, so re-operation is necessary,
- Arterial switch,
- Arterial switch procedure,
- Immediate post-operative (Jatene procedure) d-TGA + VSD neonate,
- The Jatene procedure surgery is the preferred, and most frequently used, method of correcting d-TGA; ideally, it is performed on an infant between 8–14 days old,
- The heart and vessels are accessed via median sternotomy, and a cardiopulmonary bypass machine is used; as this machine needs its "circulation" to be filled with blood, a child will require a blood transfusion for this surgery. The procedure involves transecting both the aorta and pulmonary artery; the coronary arteries are then detached from the aorta and reattached to the neo-aorta, before "swapping" the upper portion of the aorta and pulmonary artery to the opposite arterial root. Including the anaesthesia and immediate postoperative recovery, this surgery takes an average of approximately six to eight hours to complete,
- Some arterial switch recipients may present with post-operative pulmonary stenosis, which would then be repaired with angioplasty, pulmonary stenting via heart cath or median sternotomy, and/or xenograft,
- One day post-operative (Jatene procedure) d-TGA + VSD neonate,
- Atrial switch,
- In some cases, it is not possible to perform an arterial switch, either because of late diagnosis, sepsis, or a contraindicative coronary artery pattern,
In the case of sepsis or late diagnosis, a delayed Arterial Switch can sometimes be made possible by PAB, which may also require a concomitant construction of an aortic-to-pulmonary artery shunt,
- When an arterial switch is impossible, an atrial switch will be attempted using either the Senning or Mustard procedure. Both methods involve creating a baffle to redirect red and blue blood flow to the appropriate artery. Since the late 1970s the Mustard procedure has been preferred,
- Post-operative,
- Following corrective surgery but prior to cessation of anesthesia, two small incisions are made immediately below the sternotomy incision which provide exit points for chest tubes used to drain fluid from the thoracic cavity, with one tube placed at the front and another at the rear of the heart,
- The patient returns to the ICU post-operatively for recovery, maintenance, and close observation; recovery time may vary, but tends to average approximately two weeks, after which the patient may be transferred to a Transitional Care Unit (TCU), and eventually to a cardiac ward,
- Post-operative care is almost similar to the palliative care received, with the exception that the patient no longer requires PGE or the surgical palliation procedures. Additionally, the patient is kept on a cooling blanket for a period of time to prevent fever, which could cause brain damage. The sternum is not closed immediately which allows extra space in the thoracic cavity, preventing excess pressure on the heart, which swells considerably following the surgery; the sternum and incision are closed after a few days, when swelling is sufficiently reduced,
- Follow-up,
- The infant will continue to see a cardiologist on a regular basis. Although these appointments are required less frequently as time goes on, they will continue throughout the lifetime of the individual, and may increase in the event of complications or as the individual approaches middle age,
- The cardiology exam may include an echocardiogram, EKG, and/or cardiac stress test in addition to consultation,
- Additionally, some individuals may require ongoing medication therapy at home, which may include diuretics (such as furosemide or spironolactone), analgesics (such as paracetamol), cardiac glycosides (such as digoxin), anticoagulants (such as heparin or aspirin), or other medications. If the individual has undergone stenting, an anticoagulant will be a necessity to prevent build-up around the stent(s), as the body will perceive the foreign body as a wound and attempt to heal it,
- Some patients who had alternate corrective surgery, such as the Mustard or Senning procedure, may have issues with SA and VA nodal transmissions in later life,
Typical symptoms include palpitations and problems with low heart rates. This is commonly solved with a Pacemaker unit, providing scar tissue from the original operation does not block its functionality,
- More recently, ACE inhibitors have been prescribed to patients in the hope of relieving stress on the heart.
With simple D-TGA, if the foramen ovale and ductus arteriosus are allowed to close naturally, the newborn will likely not survive long enough to receive corrective surgery. With complex D-TGA, the infant will fail to thrive and is unlikely to survive longer than a year if corrective surgery is not performed.
In most cases, the patient's condition will deteriorate to the point of inoperability if the defect is not corrected in the first year.
While the "Foramen Ovale" and "Ductus Arteriosus" are open after birth, some mixing of red and blue blood occurs allowing a small amount of oxygen to be delivered to the body; if ASD, VSD, PFO, and/or PDA are present, this will allow a higher amount of the red and blue blood to be mixed, therefore delivering more oxygen to the body, but can complicate and lengthen the corrective surgery and/or be symptomatic.
Modern repair procedures within the ideal timeframe and without additional complications have a very high success rate.