Tricuspid atresia. A defect that blocks the flow of blood from the right atrium to the right ventricle of the heart .
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- 1 Causes, incidence and risk factors
- 2 Symptoms
- 3 Signs and tests
- 4 Treatment
- 5 Forecast
- 6 Complications
- 7 Situations requiring medical assistance
- 8 References
Causes, incidence and risk factors
Tricuspid atresia is a rare form of congenital heart disease that affects about 5 out of every 100,000 live births. 20% of patients with this condition will also have other heart problems.
Normally, blood flows from the body to the right atrium, then through the tricuspid valve to the right ventricle, and then continues to the lungs. If the tricuspid valve does not open, blood cannot flow from the right atrium into the right ventricle and ultimately cannot enter the lungs where it has to go to collect oxygen (get oxygenated).
Babies with tricuspid atresia are generally cyanotic (bluish discoloration of the skin) and easily have difficulty breathing.
- Cyanosis (bluish discoloration of the skin ).
- Tendency to fatigue.
- Difficulty breathing (dyspnea.
- Fast breathing.
- Poor growth.
Signs and tests
This condition can be discovered during a routine prenatal ultrasound or when the baby is examined shortly after birth. The cyanosis is present from birth. Often, a heart murmur occurs that can increase in intensity in a few months.
The exams can be the following:
- Chest x – ray .
- Cardiac catheterization.
- MRI of the heart .
Once the diagnosis is made, the baby will be taken to the neonatal intensive care unit (NICU), and a respirator (ventilator) may be needed to help him breathe. Also, a medicine called prostaglandin E1 is used to maintain blood circulation to the body.
This condition always requires surgery. If the heart is unable to pump enough blood to the lungs and the rest of the body, the first surgery usually occurs within the first few days of life. In this procedure, a shunt or artificial communication is inserted to maintain blood flow to the lungs . In some cases, this first surgery is not necessary.
After this, the baby usually leaves the hospital. The child will need to take one or more medications daily and should be closely followed by a pediatric cardiologist, who will determine when the second stage of surgery should be performed.
The second stage of the surgery is called a Glenn shunt or Hemifontan procedure. This procedure connects half of the veins that carry blue blood from the upper half of the body directly to the pulmonary artery . Surgery is usually performed when the child is between 4 and 6 months old.
During stage I and II, the child may still appear somewhat bluish (cyanotic).
Stage III, the final step, is called the Fontan procedure. The rest of the veins that carry blue blood from the body connect directly to the pulmonary artery that leads to the lungs, and the right ventricle now only needs to pump into the body and no longer to the lungs. This surgery is usually carried out between 18 months and 3 years of age. After this final step, the baby will no longer turn blue (cyanotic).
Some patients may need more surgery when they reach their 20s or 30s if they have difficult to control arrhythmias or other complications from the Fontan procedure. In a few hospitals, they consider a heart transplant a better alternative to the 3-step surgical process, but there are few donated hearts available for young children.
Improvement can be expected with most surgical procedures. Sudden death can occur when there are cardiac arrhythmias.
- Fast and irregular heart rhythms (arrhythmias).
- Chronic diarrhea (from a disease called enteropathy causing protein loss).
- Heart failure .
- Fluid in the abdomen (ascites) and in the lungs ( Pleural effusion ).
- Obstruction of artificial communication.
- Strokes and other neurological complications.
- Sudden death.
Situations requiring medical assistance
If your baby has new changes in breathing patterns, eats less (decreased feeding), or the skin or mucous membranes are turning blue (cyanotic), contact the doctor immediately.