Tricuspid Atresia - Symptoms, Diagnosis, Treatment, and Preventions

Introduction

Tricuspid atresia is a congenital disability of the heart's tricuspid valve (that controls blood flow from the right atrium to the right ventricle). Tricuspid atresia is caused when the tricuspid valve is not formed, which leads to no flow of blood from the upper right chambers (right atrium) through the lower right chambers (right ventricle) to the lungs for oxygen. The condition is a sporadic and most critical congenital disability of the heart that needs surgery immediately after the birth of a baby. The leading cause of tricuspid atresia is unknown but can occur due to genetic and chromosomal changes, medication effects, and other factors surrounding the mother during pregnancy.

What Are the Symptoms of Tricuspid Atresia?

The symptoms of tricuspid atresia include:

• Rapid breathing.

• Shortness of breath.

• Cyanosis or bluish discoloration of lips and skin.

• Problems with feeding babies.

• Poor weight gain.

• Slow body growth.

• Unusual heart sounds.

• Sweating in newborns while feeding.

• Sudden weight gain due to fluid retention.

• Weakness and fatigue.

What Are the Risk Factors for Tricuspid Atresia in Newborns?

Tricuspid atresia is a congenital heart defect, but the condition can occur in newborns due to various other risk factors such as:

• Smoking during pregnancy or even before that.

• Alcohol consumption during pregnancy.

• Parents have a history of congenital heart defects.

• A baby is born with an extra chromosome 21 called Down syndrome.

• Use certain medications during pregnancy, including anti-seizure

• Medications, ache drugs, and drugs for bipolar disorders.

• A mother has viral illness and rubella (German measles).

• A mother has poorly managed diabetes.

What Are the Complications Associated With Tricuspid Atresia?

Complications with tricuspid atresia may include:

• Heart failure.

• Irregular heart rhythms.

• Fluid in lungs (pleural effusion) and abdomen (ascites).

• Liver or kidney disease.

• Artificial shunt blockage.

• Nervous system complications such as stroke.

• Sudden death.

How Is Tricuspid Atresia Diagnosed?

Tricuspid atresia is diagnosed in the following ways:

• During pregnancy, doctors can do prenatal tests or screenings to check for congenital abnormalities and other conditions.

• For example, an ultrasound can detect the tricuspid atresia and provide the suspect with information that indicates tricuspid atresia in a baby.

• The proper diagnosis can be made by carrying out an echocardiogram of the baby during pregnancy to study the structure and working of the heart with the congenital disability.

• After the baby is born, the tricuspid atresia symptoms can be seen clinically anytime after the birth.

• They show signs like bluish-colored lips and skin due to lack of oxygen in the blood, breathing defects, problems with feeding, and sleepiness.

• The doctor may suggest an echocardiogram (ECG) to check the structure and blood flow of the heart.

• Also, cardiac catheterization can be done by inserting a thin tube via a blood vessel into the heart.

• This method helps check the heart's blood pressure and oxygen level. In addition to this, an electrocardiogram (EKG) test can also be used to check the electrical activity of a person’s heart.

• In addition to these diagnostic tools, MRI (magnetic resonance imaging), chest X-rays, and CT (computed tomography) scans of the heart can also help confirm the diagnosis of tricuspid atresia.

What Are the Treatment Options for Tricuspid Atresia?

Nutritional Therapy - Babies with tricuspid atresia often have low weight issues as they become tired during feeding. A high-calorie formula is incorporated into the baby’s diet to maintain a healthy weight, and feeding tubes can be used in some extreme cases of feeding difficulties.

Medication - Medicines are given to some newborns and children to lower blood pressure, strengthen heart muscles, and treat fluid retention in the body caused by the tricuspid atresia condition.

Surgery - Surgical treatment is recommended by the doctor depending upon the presence of congenital disabilities and the severity of the condition. Some surgeries are performed soon after the baby is born with tricuspid atresia, and other procedures may be done later in life. The surgical treatment usually restores normal heart functioning but does not correct the tricuspid atresia. Certain medications are also given after and before surgeries to treat the symptoms of tricuspid atresia. The surgical procedures are as follows:

Fontan Procedure: This is usually performed around the age of two years. In this procedure, the inferior vena cava (the vessel which carries oxygen-poor blood from lower body parts to the heart, allowing the remaining blood from the body to go to the lungs) and the main pulmonary artery are connected. This procedure helps to separate oxygenated and deoxygenated blood in the heart and also, thus, cures the bluish discoloration of the infant’s skin.

The Glenn Procedure - The surgical method is mainly used in babies when they are four to six months old. In this procedure, the doctor establishes the direct connection between the superior vena cava (the vessel which carries oxygen-poor blood from upper body parts to the heart) and the main pulmonary artery. This method helps the blood from the body flow directly into the lungs and bypass the heart.

Balloon Septostomy - This procedure is usually done during the initial days or weeks after the baby is born and creates a hole between the left and right upper heart chambers and enlarges the atrial septal defect. This procedure is done to mix more oxygenated and deoxygenated blood to get more oxygen-rich blood into the body.

Shunt Procedure- This surgical procedure is performed within the first two weeks after the baby is born. The doctor forms a passage to allow blood to flow into the lungs. The baby’s skin may appear bluish after this procedure because oxygenated and deoxygenated blood can still mix within the heart.

How to Prevent Tricuspid Atresia?

The preventive measures include:

• Women must get vaccinated for German measles before pregnancy.

• Do not consume alcohol during pregnancy.

• Avoid taking medications for acne and seizures during pregnancy.

• The doctor should adequately manage diabetes and the mother herself to avoid the risk to the baby's health.

• If the mother has a congenital disability, proper care for a high-risk pregnancy is essential.

What Is the Differential Diagnosis for Tricuspid Atresia?

• Pulmonary atresia.

• Atrial septal defect.

• Tricuspid stenosis.

• Tetralogy of Fallot.

• Pulmonic stenosis.

Conclusion

Tricuspid atresia is an uncommon congenital disability and can be treated with various surgical methods. This condition is estimated to be 1.2 per 10000 live births, and newborns who do not receive treatment during the first year of life die due to this congenital disability. However, modern diagnostic tools and surgical methods have improved the overall quality of life of people with tricuspid atresia, thus improving their functional capacity in adulthood.