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Condition

Tricuspid Atresia

Condition 101

What is the definition of Tricuspid Atresia?

Tricuspid atresia is a type of heart disease that is present at birth (congenital heart disease), in which the tricuspid heart valve is missing or abnormally developed. The defect blocks blood flow from the right atrium to the right ventricle. Other heart or vessel defects are usually present at the same time.

What are the alternative names for Tricuspid Atresia?

Tri atresia; Valve disorder - tricuspid atresia; Congenital heart - tricuspid atresia; Cyanotic heart disease - tricuspid atresia

What are the causes for Tricuspid Atresia?

Tricuspid atresia is an uncommon form of congenital heart disease. It affects about 5 in every 100,000 live births. One in five people with this condition will also have other heart problems.

Normally, blood flows from the body into the right atrium, then through the tricuspid valve to the right ventricle and on to the lungs. If the tricuspid valve does not open, the blood cannot flow from the right atrium to the right ventricle. Because of the problem with the tricuspid valve, blood ultimately cannot enter the lungs. This is where it must go to pick up oxygen (becomes oxygenated).

Instead, the blood passes through a hole between the right and left atrium. In the left atrium, it mixes with oxygen-rich blood returning from the lungs. This mix of oxygen-rich and oxygen-poor blood is then pumped out into the body from the left ventricle. This causes the oxygen level in the blood to be lower than normal.

In people with tricuspid atresia, the lungs receive blood either through a hole between the right and left ventricles (described above), or through maintenance of a fetal vessel called the ductus arteriosus. The ductus arteriosus connects the pulmonary artery (artery to the lungs) to the aorta (main artery to the body). It is present when a baby is born, but normally closes by itself shortly after birth.

What are the symptoms for Tricuspid Atresia?

Symptoms include:

  • Bluish color to the skin (cyanosis) due to low oxygen level in the blood
  • Fast breathing
  • Fatigue
  • Poor growth
  • Shortness of breath

What are the current treatments for Tricuspid Atresia?

Once the diagnosis is made, the baby will often be admitted to the neonatal intensive care unit (NICU). A medicine called prostaglandin E1 may be used to keep the ductus arteriosis open so that blood can circulate to the lungs.

Generally, patients with this condition require surgery. If the heart is unable to pump enough blood out to the lungs and rest of the body, the first surgery most often takes place within the first few days of life. In this procedure, an artificial shunt is inserted to keep blood flowing to the lungs. In some cases, this first surgery is not needed.

Afterward, the baby goes home in most cases. The child will need to take one or more daily medicines and be closely followed by a pediatric cardiologist. This doctor will decide when the second stage of surgery should be done.

The next stage of surgery is called the Glenn shunt or hemi-Fontan procedure. This procedure connects half of the veins carrying oxygen-poor blood from the upper half of the body directly to the pulmonary artery. The surgery is most often done when the child is between 4 to 6 months old.

During stage I and II, the child may still look blue (cyanotic).

Stage III, the final step, is called the Fontan procedure. The rest of the veins carrying oxygen-poor blood from the body are connected directly to the pulmonary artery leading to the lungs. The left ventricle now only has to pump to the body, not the lungs. This surgery is usually performed when the child is 18 months to 3 years old. After this final step, the baby's skin is no longer blue.

What is the outlook (prognosis) for Tricuspid Atresia?

In most cases, surgery will improve the condition.

What are the possible complications for Tricuspid Atresia?

Complications may include:

  • Irregular, fast heart rhythms (arrhythmias)
  • Chronic diarrhea (from a disease called protein-losing enteropathy)
  • Heart failure
  • Fluid in the abdomen (ascites) and in the lungs (pleural effusion)
  • Blockage of the artificial shunt
  • Strokes and other nervous system complications
  • Sudden death

When should I contact a medical professional for Tricuspid Atresia?

Contact your health care provider right away if your infant has:

  • New changes in breathing patterns
  • Problems eating
  • Skin that is turning blue

How do I prevent Tricuspid Atresia?

There is no known way to prevent tricuspid atresia.

REFERENCES

Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 20th ed. Philadelphia, PA: Elsevier; 2017:chap 58.

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease in the adult and pediatric patient. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 75.

Latest Research

Latest Advance
Study
  • Condition: Tricuspid Atresia or Variant
  • Journal: European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
  • Treatment Used: Total Cavopulmonary Connection (TCPC) Conversion with an Extracardiac Conduit
  • Number of Patients: 36
  • Published —
This study evaluated the long-term therapeutic effect of total cavopulmonary connection (TCP) conversion with an extracardiac conduit.
Latest Advance
Study
  • Condition: Patients with End-Stage Complex Congenital Heart Disease (CHD) or Cardiomyopathy (CMP) Considered for Transplantation
  • Journal: International journal of cardiology
  • Treatment Used: Catheter ablation
  • Number of Patients: 5
  • Published —
This study reported experience using catheter ablation to treat recurrent arrhythmia in patients with congenital heart disease (CHD) or cardiomyopathy (CMP) considered for transplantation.

Clinical Trials

Clinical Trial
Device
  • Status: Recruiting
  • Study Type: Device
  • Participants: 20
  • Start Date: April 21, 2019
Non-invasive Assessment of Liver Function in Patients Undergoing Heart and Liver Transplant Evaluation