Tricuspid Atresia also known as Tri Atresia is a form of hereditary heart disorder where in the tricuspid heart valve is either not present or developed abnormally. In this disorder the blood flowing from the right atrium to the right ventricle is blocked.
Causes
Tricuspid atresia is a rare type of hereditary heart disease which impacts about five in every 100,000 infants at birth. Other heart disorders are also seen in 20% of people with this disorder. In a normal person the blood would flow from the body in to the right atrium and via the tricuspid value to the right ventricle and then on to the lungs. In case the tricuspid value does not open then the blood can’t flow from the atrium to the right ventricle. The blood is not able to flow to the lungs from where it collects oxygen. Babies with this condition mostly have bluish staining of skin (cyanotic) and easily fall short of breath.
Symptoms
- Get easily tired
- Bluish discoloration of the skin (Cyanosis)
- Dyspnea (Shortness of breath)
- Poor growth
- Fast breathing
Diagnosis
This disorder can be diagnosed via routine pre-natal ultrasound scan at the time of examination of the baby just after birth. Cyanosis is present since birth. A mumur of the heart is present at birth and there is a possible increase of this mumur over a few months.
Tests may include:
- ECG
- Echocardiogram
- Cardiac catheterization
- MRI of the heart
- Chest x-ray
Treatment
Once this disorder is diagnosed, the infant is to be admitted in the NICU (neonatal intensive care unit). The baby may be put on to a ventilator (a breathing machine) in order to help the baby breath properly. To sustain the flow of blood to the lungs the infant is given Prostaglandin E1.
The ailment always needs to be operated. In case the heart is not able to pump sufficient blood to the lungs and other parts of the body, then the 1st surgery normally happens within the first couple of days of birth. In this process, a false shunt is put in to maintain the flow of the blood to the lungs. In few cases, this first surgical procedure is not essential.
After the surgery the baby is normally discharged. The kid will require to take one or more medications daily and will need to be closely monitored by his or her pediatric cardiologist, who would then determine the date for the next surgery.
The second surgical procedure is known as the Hemifontan or Glenn shunt procedure. This process links half of the veins that carry impure blood from the top half of the body straight to the pulmonary artery. This surgical procedure is normal done when the kid is 4 – 6 months of age.
In stage I and II, the kid may still seem to be cyanotic (bluish). Stage III, which is the final stage of the surgical procedure is known as the Fontan procedure. The remaining veins that carry impure blood from the body are linked directly to the pulmonary artery which leads to the lungs, and now the only job of the left ventricle is to pump to the body and not to the lungs. This surgery is generally performed between 18 months and 3 years of age. Once this final procedure is done the baby is no longer cyanotic (blue).
At times certain patients might need a few more surgeries during their 20s and 30s in case they are unable to control arrhythmias or the other complications that arise due to Fontan surgery. In some hospitals the alternative of heart transplantation is deemed to be a much better option in comparison to the 3 step surgical procedures, however; only few donated hearts are obtainable for little babies
Prognosis
Progress can be anticipated with most of the surgical procedures. Unpredicted death might occur due to heart arrhythmias.
Possible Problems
- arrhythmias (Irregular, fast heart rhythms)
- Chronic diarrhea (from an ailment known as protein loosing enteropathy)
- Heart failure
- Obstruction of the false or artificial shunt
- Fluid in the lungs (pleural effusion) and abdomen (ascites)
- Sudden death
- Strokes and other neurological complications
Time to get in touch with your child’s doctor
In case you see a change in the breathing pattern of the kid, a decrease in the feed (eats less) or you notice the mucous membranes or skin is turning bluish (cyanotic), you have to get in touch with your child’s doctor immediately. Normally heart defect is detected right after the birth.
Prevention
There is no known way to avert tricuspid atresia.
