Tricuspid Atresia is a type of congenital heart disease where the tricuspid valve, which is located between the right atrium and the right ventricle of the heart, is abnormally developed or completely absent. This defect prevents blood from flowing normally from the right atrium to the right ventricle, leading to several complications in blood circulation.

Characteristics of Tricuspid Atresia

  1. Absence of Tricuspid Valve: There is either a complete absence or abnormal development of the tricuspid valve.

  2. Hypoplastic Right Ventricle: Due to the absence of the tricuspid valve, the right ventricle is typically underdeveloped (hypoplastic).

  3. Atrial Septal Defect (ASD) or Patent Foramen Ovale (PFO): An opening between the left and right atria, allowing some blood to flow from the right to the left atrium.

  4. Ventricular Septal Defect (VSD): Many patients also have a VSD, which allows blood to flow from the left ventricle to the right ventricle.

Symptoms

Symptoms of tricuspid atresia are usually noticeable soon after birth and can include:

  • Cyanosis (bluish skin, lips, and fingernails)

  • Difficulty breathing

  • Poor feeding

  • Slow growth

  • Fatigue or lethargy

Diagnosis

Diagnosis typically involves several tests:

  • Echocardiogram: To visualize the structure and function of the heart.

  • Chest X-ray: To view the size and shape of the heart and lungs.

  • Electrocardiogram (ECG): To measure the electrical activity of the heart.

  • Cardiac catheterization: In some cases, to provide detailed information about the heart's structure and function.

Treatment

Treatment for tricuspid atresia requires surgery, usually in multiple stages:

  1. Palliative Surgery: In the first few days or weeks of life, surgery might be performed to improve blood flow to the lungs or to restrict excessive blood flow to the lungs.

  2. Glenn Procedure: Performed at around 4 to 6 months of age, this surgery connects the superior vena cava (the large vein carrying blood from the upper part of the body) directly to the pulmonary artery.

  3. Fontan Procedure: Usually performed between 2 and 4 years of age, this procedure directs blood from the lower body directly to the pulmonary artery.

In some cases, a heart transplant may be considered.

Long-term Outlook

The prognosis for individuals with tricuspid atresia varies and depends on the specifics of each case and the presence of other heart defects. Lifelong cardiac care is necessary, and there can be complications, including arrhythmias, heart failure, or problems related to the Fontan circulation. Regular follow-up with a cardiologist is essential for monitoring and managing these issues. Advances in surgical and medical treatments have significantly improved the outcomes for individuals with tricuspid atresia.