Tricuspid atresia is absence of the tricuspid valve accompanied by a hypoplastic right ventricle. TA occurs equally in boys and girls. As a result of tricuspid atresia, the lungs usually don’t receive enough blood to supply the rest of the body with the oxygen it needs. In babies born with tricuspid atresia, the tricuspid valve is absent and the right ventricle is small. The deformity consists of a complete lack of formation of the tricuspid valve with absence of direct connection between the right atrium and right ventricle. If your baby is born with tricuspid atresia, blood cannot flow from the body through the heart and into the lungs to pick up oxygen as it normally would. The tricuspid valve, normally located between the right atrium and the right ventricle, does not develop properly during pregnancy. Because of the lack of an A-V connection, an atrial septal defect (ASD) must be present to maintain blood flow. Tricuspid atresia occurs in two out of every 10,000 live births. It makes up 1 to 2 percent of all cases of congenital heart disease. Twenty percent of patients with this condition will have also have other heart problems.
Infants with tricuspid atresia generally are cyanotic ( bluish discoloration of skin ) due to the mixing of deoxygenated blood with the normally oxygenated blood in the aorta. There are several anatomic variations that influence the symptoms and course of treatment in any given patient. There may be a hole in the ventricular septum, called a ventricular septal defect. Children with tricuspid atresia will need 2 or 3 open-heart operations during childhood. Tricuspid atresia is usually detected in infancy because of presenting cyanosis, congestive heart failure, and growth failure. Parents provide a history of poor skin coloration ranging from pallor to frank cyanosis. Some children will only be mildly cyanotic, while others will not have enough oxygen in the blood to meet the body’s needs. A patent ductus arteriosus also allows blood to pass through from the aorta to the pulmonary artery and receive oxygen from the lungs. Surgery to restore normal blood flow to the lungs is necessary in children with tricuspid atresia.
Causes of Tricuspid atresia
The common causes and risk factor’s of Tricuspid atresia include the following:
The exect cause of Tricuspid atresia is unknown.
Tricuspid atresia occurs due to abnormal development of the fetal heart during the first 8 weeks of pregnancy.
Heredity or Down syndrome, may increase your baby’s risk of tricuspid atresia.
Most of the time, this heart defect occurs sporadically (by chance), with no clear reason for its development.
Symptoms of Tricuspid atresia
Some symptoms related to Tricuspid atresia are as follows:
Blue color of the skin, lips, and nail beds.
Cool, clammy skin.
Rapid heart rate.
Clubbing of fingers or toes.
Fatigue and weakness.
Sudden weight gain from fluid retention.
Treatment of Tricuspid atresia
Here is list of the methods for treating Tricuspid atresia:
An intravenous (IV) medication called prostaglandin E1 is given to keep the ductus arteriosus from closing.
Balloon atrial septostomy (Rashkind’s procedure) may be done as part of the initial catheterization to improve the right-to-left atrial shunt when the interatrial communication is inadequate.
The intelligent application of palliative procedures to control the amount of pulmonary blood flow in this lesion improves the survival of infants with tricuspid atresia.
Patients should receive antibiotic prophylaxis before undergoing any bacteremia-producing surgery or procedures.
Surgery is necessary to repair the abnormal circulation created by tricuspid atresia. A surgery called the Fontan procedure is the best option for treatment of tricuspid atresia. In the most common situation, a series of operations is required.