Triatrial septal defect syndrome in children symptoms causes and treatment

Cor Triatriatum in Children is one of the most serious congenital heart defects, directly affecting blood flow within the child’s heart and the amount of oxygen delivered to the body. Infants with this condition may show symptoms such as cyanosis (bluish skin), breathing difficulties, and growth delays from the very first days of life.In this Dalili Medical Guide, we will explore the different types of cor triatriatum, its causes, the most common symptoms, and the latest diagnostic and treatment methods—providing parents with all the essential information they need to monitor their child’s health and safeguard their life.

What is Tricuspid Atresia?
It is a congenital heart defect in which the tricuspid valve is absent or closed, preventing blood from flowing normally from the right atrium to the right ventricle.

Can a child live a normal life after treatment?
Most children, after the final surgery (Fontan procedure), can live an almost normal life. However, regular follow-ups with a pediatric cardiologist are essential. Some children may remain at risk for heart problems or have limited physical activity.

Is Tricuspid Atresia genetic?
In most cases, it is not directly inherited. However, genetic abnormalities or a family history of heart disease may increase the risk.

When do symptoms usually appear?
Often from the first days of life or within the first few months—especially cyanosis (bluish skin) and breathing difficulties during feeding.

Do all children require the same surgery?
No, the surgical approach depends on the size of the right ventricle, the presence of pulmonary artery obstruction, and any additional heart defects.

Are medications enough to treat the condition?
No, medications are not a cure. They only help relieve symptoms and prepare the child for surgery.

Can the child play like other children after surgery?
After the final surgery, most children can engage in near-normal activities. Some may need activity limitations and ongoing monitoring.

Can the condition be detected during pregnancy?
Yes, through fetal echocardiography between 18–22 weeks of pregnancy, especially if risk factors are present.

What are the main complications after surgery?

Arrhythmias (irregular heartbeats)
Blood clots
Narrowing of blood vessels or heart valves
Mild persistent cyanosis in some cases

Are long-term complications possible?
Yes. Even after surgery, some children may need additional procedures or ongoing medications to support heart and lung function.

How should a child be monitored after treatment?

Regular visits to a pediatric cardiologist
Echocardiography follow-ups
Monitoring growth and nutrition
Tracking physical activity and pulmonary pressure

Is natural delivery possible or is C-section preferred?
Natural delivery is possible in most cases. The choice depends on the baby’s condition and the mother’s health, as advised by the cardiologist and obstetrician.

Does Tricuspid Atresia affect intelligence?
It usually does not directly affect intelligence. However, chronic low oxygen levels and feeding difficulties may delay physical and cognitive development if not treated early.

Can the condition be detected right after birth?
Yes, through visible cyanosis, breathing difficulties during feeding, and heart tests like echocardiography.

What is the typical age for the final surgery (Fontan)?
Usually between 2 and 5 years, depending on the child’s condition, right ventricle size, and pulmonary artery pressure.

Do children often need more than one surgery?
Yes, most require multiple procedures:

Initial shunt or ductus arteriosus opening
Final Fontan surgery
Sometimes additional operations to adjust vessels or valves

Can children with this condition play sports?
After the Fontan surgery, most can participate in moderate physical activities. Intense sports should be discussed with the cardiologist.

Are lifelong medications necessary after surgery?
Sometimes. Children may need blood thinners or heart-support medications depending on their case.

Warning signs that require urgent medical attention:

Sudden or worsening cyanosis
Severe breathing difficulty
Fainting or loss of consciousness
Significant swelling in the abdomen or limbs

Can mothers with this condition become pregnant in the future?
In rare cases, mothers may have similar congenital heart defects. Pre-pregnancy consultation with a cardiologist is crucial to assess risks.

Is Tricuspid Atresia common?
No, it is very rare, accounting for only 1–3% of congenital heart defects.

Types of Tricuspid Atresia in children
Tricuspid Atresia is not a single type—it varies depending on the right ventricle size, pulmonary artery status, and blood flow direction:

1️⃣ By size of the right ventricle

Very small right ventricle (Hypoplastic Right Ventricle): Most common. Blood bypasses through an atrial septal defect (ASD) into the left ventricle.
Moderately sized right ventricle: The ventricle is present but underdeveloped, with partial blood flow.
Normal or large right ventricle: Rare, often with additional defects like ventricular septal defect (VSD) or pulmonary obstruction.

2️⃣ By presence of pulmonary artery obstruction

With pulmonary stenosis/atresia: Blood flow to the lungs is very limited, causing severe cyanosis from birth.
Without pulmonary stenosis: Blood still reaches the lungs, though less efficiently, with milder cyanosis.

1️⃣ Peripheral Vision Training

Since central vision is gradually affected, children learn to rely more on their peripheral vision.
Exercises may include:

Tracking moving objects using side vision.
Practicing object recognition from different viewing angles.

2️⃣ Visual Focus Improvement Exercises

Games and activities that stimulate attention to larger details, such as:

Matching large shapes.
Reading large print words.
Drawing and tracing bold lines.

3️⃣ Light Adaptation Training

Helping the child adjust gradually to bright or dim lighting to reduce sensitivity and discomfort.

4️⃣ Daily Exercises and School Activities

Encouraging the use of visual aids during reading or play.
Breaking down long visual tasks into shorter sessions to minimize eye strain.

⚠️ Important Notes

These exercises are not a cure but help children gain independence and maximize their remaining vision.
Exercises should be supervised by a pediatric vision specialist or a retinal specialist to ensure effectiveness and prevent visual fatigue.

Managing Stargardt Disease in Children

Stargardt disease is a genetic condition that causes progressive vision loss in children. While no definitive cure exists yet, several strategies can help children adapt and improve their quality of life:

1️⃣ Visual Support

Use of visual aids such as screen magnifiers or handheld magnifiers.
Access to large-print books or adapted tablets.
Proper lighting during reading or studying to ease vision.

2️⃣ Adaptations at School and Home

Adjusting seating so the child has a clear view of the board.
Providing clear, large-sized learning materials.
Training the child to use peripheral vision in daily activities.

3️⃣ Eye Protection from Light

Wearing UV-protective sunglasses outdoors.
Avoiding prolonged exposure to bright lights or screens.

4️⃣ Regular Medical Follow-ups

Routine visits to a retinal specialist or pediatric vision specialist.
Periodic check-ups to monitor disease progression and plan appropriate support strategies.

5️⃣ Psychological and Social Support

Explaining the condition to the child in a simple and reassuring way.
Encouraging participation in activities suited to their visual ability.
Joining support groups for children and families to share experiences and advice.

6️⃣ Exploring Future Treatment Options

Gene therapy and stem cell treatments may offer possibilities in the future.
Staying updated with the latest medical research through a retinal specialist could open new opportunities.

Diagnostic Tests for Tricuspid Atresia in Children

B. Chest X-ray

Shows the size and shape of the heart.
Detects ventricular enlargement or abnormalities in the pulmonary arteries.

C. Echocardiography

The most important test for diagnosis.
Shows:
- Absence or blockage of the tricuspid valve.
- Size of the right ventricle.
- Presence of atrial or ventricular septal defects (ASD/VSD).
- Blood flow through arteries and connecting channels.

D. Cardiac MRI / CT Scan

Used in some cases to provide detailed anatomy of the heart and pulmonary arteries before surgery.

E. Cardiac Catheterization

Performed in specific cases to measure pressures inside the heart and blood vessels.
Helps plan surgical interventions.

Medical Treatment of Tricuspid Atresia in Children

Medication is not a definitive cure because this is a structural congenital condition, but drugs help relieve symptoms and prepare the child for surgery.

1️⃣ Medications to Improve Pulmonary Blood Flow

Prostaglandin E1 (PGE1):
- Given in the first days of life if the ductus arteriosus (PDA) is closed.
- Keeps the ductus arteriosus open so blood can reach the lungs.

2️⃣ Medications to Reduce Fluid Retention or Manage Heart Failure

Diuretics (e.g., Furosemide): Reduce fluid retention in the body, liver, and lungs.
Digoxin: Improves heart contraction and helps blood circulation.

3️⃣ Blood Thinners (in certain cases)

Low-dose Aspirin or anticoagulants are prescribed after some surgeries to reduce the risk of blood clots.

⚠️ Important Notes

Medications are not a permanent treatment.
Surgery is essential to correct or redirect blood flow to the lungs.
Regular follow-up with a pediatric cardiologist is crucial to adjust medications.

Surgical Treatment of Tricuspid Atresia in Children

Surgery is the cornerstone of treatment since this is a congenital structural defect that prevents normal blood flow. The aim is to redirect blood so that the lungs receive enough oxygenated blood, supporting the child’s growth.

1️⃣ PDA Surgery or Pulmonary Connection

Done in the first weeks or months of life.
Purpose: Increase blood flow to the lungs if pulmonary blood supply is very low.
Prostaglandin may be given before surgery to keep the PDA open.

2️⃣ Shunt Operation (Systemic-to-Pulmonary Shunt)

Example: Blalock-Taussig Shunt.
Creates an alternative pathway for blood to reach the lungs.
Usually performed in the first year of life.

3️⃣ Fontan Procedure (Final Surgery)

Typically performed between ages 2–5 years depending on the child’s growth and heart condition.
Concept: Diverts venous blood directly to the pulmonary arteries without passing through the absent/small right ventricle.
Outcome: Improves oxygenation, reduces cyanosis, enhances growth and activity.

4️⃣ Additional Surgeries if Needed

Repairing or enlarging septal defects (ASD/VSD).
Correcting pulmonary artery obstructions or valve issues after initial surgeries.

⚠️ Important Notes

Surgery is not a 100% cure but significantly improves quality of life.
After surgery, ongoing monitoring is required for:
- Pulmonary artery pressures.
- Heart function.
- Complications such as blood clots or arrhythmias.

Prevention of Tricuspid Atresia in Children

Tricuspid atresia is a congenital heart defect present from birth. While the exact cause is often unknown, certain steps may reduce the risk of congenital heart defects:

1️⃣ Good Nutrition Before and During Pregnancy

Take folic acid before conception and during the first trimester to lower the risk of heart defects.
Follow a doctor-supervised regimen of essential vitamins and minerals.

2️⃣ Avoid Harmful Substances