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Tricuspid atresia is a serious congenital heart disease caused by the failure of the tricuspid valve of the heart to develop. It occurs when solid tissue replaces the tricuspid valve. This strip of tissue prevents blood from flowing correctly from the right atrium to the right ventricle. With this, the body does not receive adequate oxygen. This condition is also known as tricuspid valve atresia or TV atresia.
Normally, the tricuspid valve connects two chambers on the right side of your heart: the right atrium (upper chamber) and the right ventricle (lower chamber). Tricuspid atresia occurs when solid tissue replaces the tricuspid valve. Due to this obstruction, the right ventricle is often smaller and undeveloped.
Tricuspid atresia prevents enough blood from passing through the heart and into the lungs, where it would receive oxygen. As a result, the lungs are unable to give adequate oxygen to the rest of their body. People with this condition fatigue quickly and are frequently out of breath. Their skin and lips could seem blue or gray.
Although there is no definite cure for tricuspid atresia. Several surgeries at various ages may improve a baby’s heart function. Many people survive surgery and reach adulthood. If the operations do not help or their heart fails to function properly, a heart transplant can be performed.
Symptoms
The symptoms of tricuspid atresia are experienced immediately or within a week after birth. Most babies may experience:
- Cyanosis or lips and skin that are bluish in color.
- Breathing problems
- Quick exhaustion, particularly during feeding
- Delayed development and not gaining adequate weight.
Symptoms of heart failure are also common in babies with tricuspid atresia, such as:
- Tiredness and lack of strength
- Breathing difficulties and rapid breathing
- Rapid weight increase due to fluid retention
- Legs, ankles, and foot swelling
- Ascites or swelling in the abdomen
Consult a healthcare provider if you notice any symptoms in your baby, particularly changes in their skin tone, difficulty breathing, slow growth, or poor weight gain. Immediate consultation may help to prevent complications from developing. In most cases, severe congenital cardiac abnormalities are detected before or shortly after birth.
Causes
Although there are documented risk factors for tricuspid atresia, the specific cause is unknown. Congenital heart disorders begin when a fetus is still in the uterus while the heart is developing.
Tricuspid atresia is a congenital heart disorder in which the tricuspid valve does not exist. Blood cannot flow normally from the upper to lower chambers on the right side of the heart without this valve. Instead, blood travels directly from the right upper chamber of the heart to the left upper chamber via a hole in the wall between them. The hole is either a congenital cardiac defect known as an atrial septal defect or a naturally occurring opening known as the foramen ovale.
A patent foramen ovale occurs when the foramen ovale fails to close after birth. Some newborns with tricuspid atresia may have an additional opening between the two lower chambers of the heart, which is called a ventricular septal defect or VSD. Blood can flow through this opening and into the right ventricle, where it is then pumped into the lungs. When blood flows through these abnormal channels, high-oxygen and low-oxygen blood can mix, which does not occur in a normal heart. If the VSD is large, too much blood may flow to the lungs, leading to cardiac failure.
Generally, the flow of blood is determined by whether or not there are any other structural issues with the heart. In some babies with tricuspid atresia, blood flows from the left lower heart chamber into the body’s major artery, the aorta. The blood then travels to the lungs via a temporary connection known as the ductus arteriosus, which usually closes after birth.
Risk factors
Tricuspid atresia is a complex congenital heart condition that, while rare, is still one of the more prevalent types. It affects approximately 1 in every 10,000 newborns, without any significant difference between genders.
There are certain risk factors associated with tricuspid atresia. Individuals with Down syndrome or a parent who had a congenital heart abnormality are more prone to develop tricuspid atresia or another congenital heart condition.
Additional risk factors include having a birthing parent who:
- Had a viral disease during the first trimester of pregnancy, such as German measles
- Congenital heart disease in the family
- Drank alcohol excessively when pregnant
- Use tobacco prior to or during pregnancy
- Had diabetes that was poorly managed during pregnancy
- Taken certain drugs, such as anti-seizure, acne, or bipolar disorder medications while pregnant
Diagnosis
Tricuspid atresia can be detected during a routine prenatal ultrasound before a baby is born. If suspected, healthcare providers typically confirm the diagnosis of tricuspid atresia through an echocardiogram, which uses harmless sound waves to create moving images of the heart on a video screen.
After the baby is born, a healthcare provider will conduct a thorough examination of the baby’s heart and lungs and listen for any irregularities in the heart’s rhythm or sounds, such as a heart murmur. If a baby exhibits symptoms such as blue or gray skin, difficulty breathing, or a heart murmur, the healthcare provider may suspect tricuspid atresia. A heart murmur may be caused by changes in blood flow to and from the heart.
Tests that may be performed to diagnose tricuspid atresia can include:
- Echocardiogram. The echocardiography monitors blood flow and can reveal whether the tricuspid valve is absent, and if the right ventricle is smaller than usual. It also shows how much blood is passing through the holes in the child’s septum, or the wall that separates the heart chambers. Heart defects such as a ventricular septal defect or an atrial septal defect may be detected through this test. The sound waves provide dynamic images of blood circulation through the heart and heart valves.
- Electrocardiogram. An ECG or EKG can reveal irregular cardiac rhythms by measuring the electrical activity of the heart and how quickly or slowly it beats. This simple, noninvasive test can aid in diagnosing a cardiac condition. During the procedure, a computer translates the information into a wave pattern that the doctor can interpret.
- Pulse oximetry. This uses sensors to detect a baby’s oxygen level. Pulse oximetry is a straightforward and painless procedure. The equipment is usually placed to the hand or foot.
- Chest X-ray. A chest X-ray generates an image of the heart, lungs, and bones by using radiation beams to produce images of the body’s interior. This imaging technique provides information about the health of the heart and lungs and can be used to calculate the size of the heart and its chambers. Additionally, a chest X-ray can reveal fluid accumulation in the lungs.
- Cardiac catheterization. Also known as cardiac cath or coronary angiograph is an invasive imaging treatment that allows the doctor to assess the function of the heart. Cardiac catheterization is rarely used to detect tricuspid atresia. However, it may be performed before tricuspid atresia surgery to evaluate the heart.
During the procedure, a catheter, a narrow, flexible tube is placed into a blood artery, generally in the groin area, and directed into the heart. Dye is injected into the heart chambers via the catheter. The dye makes the chambers visible on X-ray pictures. The color images of the contrast material enable the doctor to determine the location of the coronary artery constriction or blockage, examine the size and shape of heart chambers and/or blood vessels, and detect abnormal leaks or holes.
Treatment
If your infant is diagnosed with tricuspid atresia, it is advisable to seek medical attention at a healthcare facility where skilled surgeons and healthcare professionals with expertise in managing complex congenital heart disease are available.
It is currently not possible to replace a tricuspid valve in cases of tricuspid atresia. However, there are multiple surgical procedures that can be performed to enhance blood flow through the heart and to the lungs in children with this condition. Symptoms can be managed with the help of appropriate medications.
Treatment possibilities can vary from medications to surgical procedures, and it is crucial to have lifelong monitoring and follow-up.
- Medications: Medication given to babies with tricuspid valve atresia at birth can help maintain their patent ductus arteriosus open. This provides an alternative method of delivering oxygen to the baby’s lungs, since the standard approach does not work without a tricuspid valve. The extra blood vessel allows blood to pass from their aorta to their pulmonary artery, which usually closes after birth. Infants may be administered the hormone prostaglandin before heart surgery to assist broaden and keep the ductus arteriosus open.
Tricuspid atresia medications may be given to build up the cardiac muscle, reduce blood pressure, and get rid of any excess fluid in the body. To assist the newborn, breathe better, more oxygen may be administered.
- Surgeries or other procedures: Tricuspid atresia in infants usually requires multiple heart surgeries or procedures. Some of these interventions are temporary measures intended to improve blood flow quickly, while others are permanent solutions.
The surgical procedures used to treat tricuspid atresia can be open-heart surgery or minimally invasive heart surgery, depending on the particular type of congenital heart defect involved. Tricuspid atresia surgery includes the following procedures:
- Balloon septoplasty. If your baby’s heart condition requires it, a surgical procedure can be performed through cardiac catheterization to widen an existing opening and improve the flow of oxygen-rich blood to the body. This intervention may be necessary as an urgent measure shortly after birth if the baby appears severely cyanotic. However, this type of surgery is seldom necessary for this particular cardiac defect. Depending on the specific circumstances, your baby may also require one of the two following surgical procedures:
- Modified Blalock-Taussig (BT) shunt. A blue-tinted infant, likely due to poor oxygenation, may undergo a surgical procedure in which their healthcare provider inserts an artificial tube between a major artery in their chest and the arteries of their lungs. This intervention serves to augment blood flow to the lungs and enhance oxygen delivery throughout the body. Typically performed in the first few days of life, this surgical procedure is an open operation.
- Pulmonary artery band placement. In cases where a baby with tricuspid atresia also has a ventricular septal defect, a surgical procedure may be performed. The surgeon will place a band around the main artery of the lungs to decrease the volume of blood flowing from the heart to the lungs. This is done to treat heart failure in babies who have too much blood flowing to their lungs. The band used in this procedure is flexible and placed around the pulmonary artery. This surgery is typically done within a few weeks after the baby is born.
- Glenn procedure. The Glenn procedure involves the removal of the first shunt followed by the direct connection of one of the major veins that typically carries blood back to the heart to the lung artery. This procedure is performed to reduce the pressure on the heart’s lower left chamber, thus lowering the risk of damage to it. It can be carried out once the baby’s lung pressures have decreased, which usually occurs with age.
Performing the Glenn procedure creates a foundation for a more lasting corrective surgery known as the Fontan procedure.
- Fontan procedure. A common approach for heart surgery in young children between the ages of 2 to 5 involves establishing a path that allows blood, which would normally go to the right heart, to flow directly into the pulmonary artery instead. This procedure is known as the Fontan procedure. While the short-term and intermediate-term prospects for infants who undergo the Fontan procedure are usually positive, it is important to conduct periodic evaluations to detect any potential complications, such as heart failure.
- Atrial septostomy. Although this procedure is rarely needed in tricuspid atresia, it can be a life-saving measure soon after birth when the baby appears very blue due to lack of oxygen. The procedure involves using a balloon to widen the opening between the upper chambers of the heart, allowing more blood to flow from the right upper chamber to the left upper chamber.
For babies with tricuspid atresia, the first few months of their life are the most dangerous, but complications can occur after any surgery. They may need lifelong follow-up care to allow a cardiologist to check their health. Many children who have congenital cardiac problems, such as tricuspid atresia, have normal lives. However, doctors may advise them to limit physically demanding activities.
Many people with tricuspid atresia are living into adulthood. One study showed a 61% survival rate 20 years later. Individuals who have had tricuspid atresia surgery can have children, although their pregnancies are high-risk.
