A diagnosis of Tricuspid Atresia (TA) means a baby is born with a serious congenital heart defect where the tricuspid valve, which normally separates the right atrium and right ventricle, is missing. This prevents blood from flowing to the lungs in the usual way, resulting in low oxygen levels and the characteristic “blue baby” syndrome, or cyanosis. This condition requires intensive care, immediate stabilization, and a complex long-term plan.

Treatment is critical for survival and managing the symptoms of inadequate oxygenation, such as extreme fatigue, difficulty feeding, and breathing issues. While surgical repair is the definitive long-term solution, medications are an essential bridge. They are used immediately after birth to stabilize the baby and chronically throughout childhood and adulthood to support the single ventricle that must do the work of two (National Heart, Lung, and Blood Institute, 2023).

Overview of treatment options for Tricuspid Atresia

The core management of Tricuspid Atresia is a series of three palliative surgeries (often culminating in the Fontan procedure) designed to redirect blood flow to the lungs without involving the missing valve. Medications play a crucial, supportive role alongside these procedures.

The main goals of medication are:

1. Immediate stabilization in the newborn period to ensure blood mixing until the first surgical fix.
2. Long-term support of the single heart ventricle.
3. Fluid management to reduce the burden on the heart and circulatory system after surgery.

Medication is generally not a cure but a tool used to optimize heart function and reduce symptoms between the critical surgical stages.

Medications used for Tricuspid Atresia

The types of medications used depend on the patient’s age and stage of treatment:

1. Emergency Stabilization (Neonatal Period): The primary drug class used immediately after birth is Prostaglandins (specifically Prostaglandin E1). This medication is essential for infants whose survival depends on maintaining an open fetal blood vessel, the ductus arteriosus (PDA), which allows blood to flow from the aorta back toward the lungs.

2. Fluid and Heart Function Management (Long-Term): Once stabilized, patients are often placed on daily medications to support the heart and manage fluid balance:

• Diuretics: Medications like furosemide or spironolactone help the body excrete excess salt and water through the kidneys. This reduces overall blood volume, easing fluid retention and swelling (edema), which commonly occurs when the single ventricle struggles to pump efficiently.
• ACE Inhibitors/ARBs: These drugs, such as enalapril or captopril, are used to relax blood vessels. They lower the resistance the single ventricle must pump against, optimizing its function and potentially reducing the risk of long-term failure.
• Digoxin: This drug is sometimes used in infancy to temporarily improve the strength of the heart muscle contractions.

Studies show that aggressive medical management of fluid status and blood pressure is key to successful outcomes following the complex Fontan procedure (American Heart Association, 2024).

How these medications work

The goal of these medications is to compensate for the missing tricuspid valve and the underdeveloped right ventricle.

Prostaglandins work by preventing the muscular wall of the PDA from closing. This temporary measure allows blood that has already been oxygenated in the lungs to mix with unoxygenated blood, ensuring some oxygen reaches the body’s tissues.

Diuretics help the body maintain a balanced fluid status. By increasing urine production, they reduce the amount of fluid circulating in the blood vessels, which directly decreases the pressure and swelling in the body’s tissues.

ACE inhibitors and ARBs block the action of certain hormones that cause blood vessels to narrow. By widening the vessels, these drugs reduce the workload on the single functioning ventricle, allowing it to pump blood to the body with less effort.

Side effects and safety considerations

TA medication requires constant monitoring, especially in infants. Prostaglandin E1 needs intensive care due to the risk of apnea.

Long-term vigilance is also essential. Diuretics can cause electrolyte issues (like low potassium), requiring blood tests. ACE inhibitors and ARBs may cause hypotension or dizziness. Caregivers must closely watch children for dehydration (excessive thirst, dry mouth) or worsening cyanosis, which necessitates immediate medical attention.

Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care.

References

1. American Heart Association. https://www.heart.org
2. Mayo Clinic. https://www.mayoclinic.org
3. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov
4. MedlinePlus. https://medlineplus.gov