Tricuspid Atresia

Tricuspid atresia is a rare congenital heart defect where the tricuspid valve, which separates the right atrium and right ventricle, is missing or abnormally developed. This condition occurs in about 1 in every 10,000 births.

Cause

The exact cause of tricuspid atresia is not fully understood, but it is believed to occur during fetal development when the heart is forming. Genetic factors and environmental influences may contribute to the development of this condition.

Symptoms

Infants with tricuspid atresia often exhibit symptoms shortly after birth, including:

• Cyanosis (blue discoloration of the skin)
• Shortness of breath
• Rapid breathing
• Fatigue
• Poor feeding
• Sweating

Diagnosis

Tricuspid atresia is typically diagnosed using a combination of the following tests:

• Chest X-ray
• Echocardiogram (ultrasound of the heart)
• Electrocardiogram (ECG or EKG)
• Cardiac catheterization
• Magnetic resonance imaging (MRI) or computed tomography (CT) scan

Treatment

Treatment for tricuspid atresia usually involves a combination of surgical procedures and medical management. The goal of treatment is to improve blood flow to the lungs and increase oxygenation of the blood.

• Surgical procedures may include:
• Blalock-Taussig shunt: creates a connection between the subclavian artery and pulmonary artery
• Glenn procedure: connects the superior vena cava to the pulmonary artery
• Fontan procedure: redirects blood flow from the right atrium to the pulmonary artery
• Medical management may include:
• Oxygen therapy
• Medications to manage symptoms and improve heart function

Prognosis

The prognosis for infants with tricuspid atresia has improved significantly with advances in surgical techniques and medical management. However, the condition can still be associated with significant morbidity and mortality.

• Infants who undergo successful surgery may experience:
• Improved oxygenation of the blood
• Increased energy levels
• Improved growth and development
• Long-term complications may include:
• Heart failure
• Arrhythmias
• Pulmonary hypertension

Follow-up Care

Regular follow-up care with a cardiologist is essential for managing tricuspid atresia and preventing long-term complications. This may include:

• Regular echocardiograms and ECGs to monitor heart function
• Medication management to control symptoms and prevent complications
• Lifestyle modifications, such as avoiding strenuous exercise and maintaining a healthy weight