Bluish color of baby's skin: could be tricuspid atresia

By Cristiano Antonino On Dec 15, 2022

Tricuspid atresia is a congenital heart defect that is manifested by the bluish color of the baby’s skin. It can be diagnosed during pregnancy or soon after birth

Tricuspid atresia is a congenital heart defect that arises during fetal life due to a failure of the tricuspid valve to develop

Normally, oxygen-poor blood returns to the right atrium via the hollow veins, passes into the right ventricle, which pumps it through the pulmonary arteries to the lungs where it is oxygenated.

Oxygen-rich blood then returns from the lungs to the left atrium, passes into the left ventricle, and is pumped into the aorta to reach the whole body.

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In tricuspid atresia, however, the valve has not formed

In its place is fibrous tissue that closes it, preventing the passage of oxygen-poor blood from the right atrium to the right ventricle.

The right ventricle is often small (hypoplastic) due to reduced blood flow as early as fetal development.

In tricuspid atresias that are not life-threatening, there is at least one defect in the wall (septum) separating the two atria or the septum separating the two ventricles (usually both).

This makes it possible for non-oxygenated blood to mix with oxygenated blood.

The ductus arteriosus of Botallo is an artery that, in fetal life, allows the passage of blood from the pulmonary arteries to the aorta.

It normally closes during the first few hours of life.

If it remains open after birth, it allows the passage of blood from the aorta to the pulmonary arteries and then to the lungs where it is oxygenated.

It can therefore improve the situation of infants with tricuspid atresia

Tricuspid atresia accounts for a small percentage of all congenital heart defects and affects approximately 5 in 100,000 live births.

It occurs with the same frequency in both sexes.

In about 20 percent of cases it is associated with other cardiac malformations such as pulmonary stenosis, persistence of the left superior vena cava, or transposition of the great arteries.

It is unclear whether there is a genetic predisposition to the development of the disease, but there are certainly families with an increased frequency of this heart defect.

Factors that increase the risk are: infections during pregnancy such as rubella, relatives with congenital heart disease, late age of conception of the child, obesity, poor glycemic control of the mother, use of alcohol, smoking or drugs during pregnancy.

Tricuspid atresia is manifested by cyanosis (bluish color of the child’s skin) due to the mixing of non-oxygenated blood with oxygenated blood from the left ventricle to the whole body.

Some children are moderately cyanotic and can survive long enough; others need early surgery because the body’s oxygen demands are not being met.

Of course, symptoms differ from patient to patient depending on the severity of the situation.

The most common are cyanosis, increased frequency of breathing that is also labored, and increased heart rate (tachycardia).

Other symptoms such as heart murmurs, lower limb swelling, collection of fluid in the cavity of the peritoneum (ascites) and fluid retention may also occur.

Tricuspid atresia can be diagnosed during pregnancy by fetal echocardiography

At birth, the presence of cyanosis immediately raises suspicion of congenital heart disease.

It is almost always associated with heart murmurs that alert the neonatologist.

Subsequently, instrumental examinations such as: electrocardiogram, echocardiogram, cardiac catheterization and MRI are used.

The baby is initially stabilized in the intensive care unit. In some cases, prostaglandins are indicated, which keep the ductus arteriosus open, thus allowing flow to the pulmonary arteries.

If the interatrial communication is found to be small, cardiac catheterization may be indicated either for diagnostic purposes or to enlarge the interatrial defect (Raskind’s procedure).

These procedures allow stabilization of the patient and give the opportunity to decide on the most appropriate surgical therapy

Pulmonary flow modulation interventions;
Reduced pulmonary flow (pulmonary hypoflow): Blalock-Taussig shunt. Creates a passage of blood to the lungs. It is a connection between the subclavian artery and the right pulmonary artery with the interposition of a Gore-Tex tube of appropriate size for the infant’s weight. Moderate cyanosis persists after this intervention;
Increased pulmonary flow (pulmonary overflow): Banding of the pulmonary artery. It is performed by going to reduce the diameter of the pulmonary artery trunk;
Glenn intervention: is performed at about 4-6 months of age. It is the first intervention in patients with adequate pulmonary flow who do not require neonatal palliative procedures (Blalock-Taussig Shunt or Pulmonary Banding). It consists of a connection between the superior vena cava and the right pulmonary artery. In this way, blood from the head and arms ends up in the pulmonary circulation to be oxygenated. The flow from the inferior cava still returns to the right atrium and goes to mix with the blood from the left atrium, as a result, the baby will still remain more to less cyanotic. This operation is necessary as an intermediate step toward definitive palliation;
Fontan surgery: is usually performed after the age of two years. A connection is made between the inferior vena cava and the right pulmonary artery with the interposition of a Gore-Tex conduit, leaving the connection between the superior vena cava and the right pulmonary artery in place (Glenn’s operation). With these connections, all the blood from the hollow veins is brought to the lungs, oxygenating it, thus eliminating cyanosis and excluding the right ventricle from the pulmonary circulation.

The procedures are all performed under general anesthesia.

A period of ICU care and observation follows.

After discharge from the ICU, the child is transferred to the inpatient ward and held for a few more days to perform follow-up examinations.

The child is then discharged with medical therapy, the need for periodic cardiology checkups, and some physical activity limitations.

A cardiac catheterization for functional evaluation of the heart and lungs is required before Glenn and Fontan’s surgery.

The child will remain cyanotic until Fontan’s surgery, growing slower than average due to reduced oxygen availability.

Following Fontan surgery, by increasing blood oxygenation, children resume growth and in some cases can achieve normal growth.

After the surgeries, the child requires lifelong cardiology checkups for therapeutic adjustments and cardiac function checks (about every 3-6 months).

There is a significant risk determined by the duration of single-ventricle circulation of developing cardiac dysfunction, arrhythmias, and other complications.

Pregnancy and the need for other surgeries later in life may expose one to greater risks and require careful cardiologic evaluation first.

The single ventricle may deteriorate in function over time, necessitating the need for cardiac transplantation in adulthood.

Regular follow-up at a specialized congenital heart disease center that cares for both children and adults who have grown up with congenital heart disease is essential.

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