OVERVIEW

What is transposition of the great arteries in newborns?

Transposition of the great arteries (TGA), also known as transposition of the great vessels, is a severe congenital heart defect. It occurs when the aorta and pulmonary artery are abnormally connected, causing abnormal blood flow in the body and disrupting normal circulation, preventing the body from receiving oxygen and nutrients.

If TGA is the only heart defect, the newborn's pulmonary blood flow forms a closed loop, preventing oxygen from reaching the body. Severe hypoxia develops rapidly, and without prompt surgical intervention, the infant will quickly succumb.

If other heart defects, such as ventricular septal defect (VSD) or atrial septal defect (ASD), are also present, the child may survive longer, allowing time for surgical treatment.

Can transposition of the great arteries lead to infant death?

Without treatment, infants with TGA rapidly develop severe, irreversible hypoxia, leading to early death. In cases of isolated TGA, if the ductus arteriosus remains open after birth, it serves as a lifeline. Once it closes, the infant faces life-threatening risks.

Overall, about 30% of untreated infants die within one week of birth, 50% within one month, and 90% within one year.

Is transposition of the great arteries common in newborns?

TGA is not a common congenital heart defect, occurring in approximately 2.3 per 10,000 live births. However, if not diagnosed and treated promptly, the mortality rate is high. About 50% of TGA cases are accompanied by a ventricular septal defect.

SYMPTOMS

What are the symptoms of transposition of the great arteries in newborns?

The most prominent symptom is cyanosis (bluish discoloration of the skin), including purple or darkened skin, nail beds, lips, hands, and feet. This color change persists continuously for 24 hours and is unaffected by activity.

Oxygen therapy does not improve cyanosis and may even worsen hypoxia. Later symptoms may include rapid breathing, lethargy (no crying or movement), cold extremities, and signs of shock.

CAUSES

What are the common causes of transposition of the great arteries in newborns?

During the embryonic period, harmful external stimuli during cardiac development or congenital developmental abnormalities caused by genetic mutations.

When do symptoms of transposition of the great arteries appear in newborns?

• Since the fetus receives oxygen and nutrients from the mother through the placenta during fetal development, it does not affect fetal growth. Symptoms gradually appear after the newborn is born.
• Children with simple transposition of the great arteries exhibit symptoms earliest. Symptoms may appear on the first day after birth.

DIAGNOSIS

What tests are needed for transposition of the great arteries in newborns? Why are these tests performed?

Cardiac ultrasound, chest X-ray, complete blood count, oxygen saturation monitoring, blood gas analysis, BNP, coagulation function, biochemical tests.

• Cardiac ultrasound: To assess structural changes in the heart and determine the type of cardiac developmental abnormalities and vascular pathways.
• BNP: To evaluate functional changes in the heart.
• Chest X-ray: To assess lung conditions.
• Complete blood count, coagulation function, biochemical tests: To evaluate overall systemic status, detect infections or coagulation disorders, and assess liver and kidney function.
• Oxygen saturation monitoring, blood gas analysis: To determine oxygen levels in the body. Comparing oxygen saturation before and after oxygen therapy helps better assess the condition.

What are the risks of transposition of the great arteries in newborns?

Heart failure, shock—these complications may threaten the patient's life and lead to death.

TREATMENT

How is transposition of the great arteries in newborns treated?

Patients with transposition of the great arteries are critically ill and require comprehensive treatment in the intensive care unit.

• First, clarify the abnormalities in the child's cardiac development. For infants with simple transposition of the great arteries and a patent ductus arteriosus, avoid administering high concentrations of oxygen and use medications like alprostadil to keep the ductus arteriosus open. Then, collaborate with cardiac surgeons to determine the optimal timing for surgery. Additionally, effective prenatal diagnosis can improve survival rates.

• For complex congenital heart disease, protect cardiac function and seek surgical opportunities. Once the infant's condition stabilizes, surgical correction is best performed within the first week after birth.

• For patients without any other cardiac defects, the arterial switch operation (ASO) is recommended. Typically, ASO has replaced the earlier atrial switch procedures developed by Mustard and Senning.

• For patients with a ventricular septal defect (VSD), the preferred surgical approach is ASO combined with VSD closure.

• For patients with dextro-transposition of the great arteries (D-TGA), a large VSD, and significant pulmonary stenosis, another surgical method, the Rastelli procedure, should be considered. In some cases, ASO may be performed with or without repair of left ventricular outflow tract obstruction.

Can transposition of the great arteries in newborns be cured?

Once transposition of the great arteries is detected, the child's cardiac condition must be evaluated as soon as possible to understand the blood flow pattern. Appropriate surgical intervention can save the child's life. Some cardiac abnormalities are highly complex and may require multiple corrective surgeries.

Do newborns with transposition of the great arteries require follow-up after discharge?

Postoperative follow-up at a specialized cardiac clinic is necessary to assess cardiac function and detect any complications. Regular pediatric follow-ups are also important to monitor developmental progress.

Follow-up includes focused medical history review, physical examination, electrocardiogram (ECG), and detailed imaging studies such as echocardiography and/or magnetic resonance imaging (MRI).

What risks may arise after surgical treatment for transposition of the great arteries in newborns?

• Long-term follow-up is essential for patients who undergo ASO, as 5%–25% of cases may develop complications requiring reintervention. The most common conditions necessitating reintervention include pulmonary artery stenosis, coronary artery insufficiency, neoaortic root dilation, and neoaortic valve regurgitation.
• Patients who undergo atrial switch procedures may experience complications such as right ventricular failure and arrhythmias, though this procedure has largely been replaced by ASO.
• Older patients who received atrial switch procedures are at risk of atrial arrhythmias and progressive heart failure, which are the primary causes of late mortality.

How long can a child live after undergoing ASO for transposition of the great arteries?

The long-term survival rate for patients with dextro-transposition of the great arteries after ASO is excellent. Some studies report a 20-year survival rate of up to 97% after discharge.

DIET & LIFESTYLE

What should be noted in the daily life of newborns with transposition of the great arteries after surgery?

• There are no special dietary restrictions—just maintain balanced nutrition. For newborns and infants, breastfeeding is preferred when possible.
• Pay attention to preventing complications.
• Post-surgery, children need to take oral medications as prescribed and attend regular follow-ups. Infants with abnormal heart function should avoid excessive milk intake, and changes in urine output and weight should be monitored.

PREVENTION

Can Transposition of the Great Arteries in Newborns Be Prevented?

There is no definitive method for prevention.

• Avoid exposure to harmful substances, contact with individuals with colds, and contracting illnesses or taking medications during early pregnancy to reduce the risk of abnormal heart development.
• Attend scheduled prenatal checkups. Fetal ultrasound can detect most congenital heart defects, though transposition of the great arteries is often harder to identify. If detected early, consider delivering at a hospital with strong cardiac surgical capabilities, undergoing further tests to assess fetal development, or termination of pregnancy as an option.