Tricuspid Atresia: Information on Causes, Diagnosis & Treatments

Heart Blow Flow Abnormal Tricuspid atresia is a congenital heart defect, meaning the child was born with the condition where the tricuspid valve between the two chambers of the heart does not form. Instead, the child was born with solid tissue between the right atrium (right upper chamber) and right ventricle (lower right chamber). This creates a serious problem where the flow of blood cannot appropriately move through the heart to the lungs, depriving the body of much-needed oxygen.

Tricuspid atresia displays itself in numerous anatomic variations that will display different symptoms and require different treatments to repair that specific defect. The defect is usually diagnosed a day or two after birth when the ductus arteriosus, which connects the artery to the lungs (pulmonary artery) to the aorta that usually closes naturally a few days after birth.

The defect causes the baby to become tired easily that results in bluish skin and shortness of breath. The condition can be treated through a series of surgeries will into adulthood. However, successful tricuspid atresia surgeries can improve the patient’s outcome to live a long healthy life.

Who Is at Risk for Tricuspid Atresia?

Nearly all cases the tricuspid Theresia are part of a congenital heart defect, where the child is born with the problem. However, numerous factors can increase the potential risk of a child being born with the defect. These include:

  • Mothers who drink alcohol while pregnant

  • Mothers who smoke before and during pregnancy

  • Mothers who have acquired rubella (German measles) or other viral illnesses during the early stages of pregnancy

  • Mothers with poorly managed diabetes

  • Mothers diagnosed with the autoimmune disorder lupus

  • Mothers who take certain drugs while pregnant including antidepression medication, anti-seizure drugs, and numerous bipolar disorder drugs

  • Children born with Down syndrome

What Causes Tricuspid Atresia?

The defect develops while the fetus is growing in the mother’s womb when the heart is in its development stage. Some hereditary risk factors including Down syndrome can increase the chances of developing tricuspid atresia.

Tricuspid atresia describes a defect where the right side of the heart fails to pump blood to the lungs properly. During formation of the heart, solid tissue (instead of a valve) blocks blood flow to the right ventricle from the right atrium. This often results in a hypoplastic (underdeveloped) right ventricle. Because of the defect, the flow of blood is pumped through a hole in the septum wall that usually closes a few days after birth. Generally, emergency surgery is required when the defect is identified. This is followed by numerous surgeries that will occur through childhood and into adulthood to ensure the heart functions normally.

Many babies born with the defect will have other defects that caused blood flow impairment. Early identification is made by the presentation of its symptoms.

Common Symptoms

The most common tricuspid atresia symptoms that tend to develop right after birth include:

  • Cyanosis where the lips and skin appear with the blue tinge

  • Dyspnea where the baby has difficulty in breathing

  • Tiring easily, especially when the child is being fed

  • Poor weight gain

  • Slow growth

  • Weakness

  • Fatigue

  • Sudden weight gain caused by the retention of fluid

  • Ascites (abdominal swelling)

  • Edema where the feet, ankles or legs swell

Diagnosing Tricuspid Atresia

Many babies are diagnosed with tricuspid atresia while Stone the womb using prenatal ultrasound technology. During the procedure, the doctor can identify the defect and prepare an effective treatment once the child was born.

After the child’s birth, the doctor might suspect there is tricuspid atresia or another heart defect by blue tinge skin and difficulty in breathing immediately after birth or within a few days. The doctor might detect a heart murmur when using a schedule scope through a comprehensive physical examination after the child was born. The murmur usually presents itself as an abnormal wishing sound that identifies a turbulent flow of blood.

For an accurate diagnosis of tricuspid atresia, the doctor will likely order a variety of tests that include:

  • Echocardiogram – Using an echocardiogram to diagnose tricuspid atresia is an effective way to rule out other conditions of the heart that are affecting the flow of blood to the lungs. The test reveals the defect and whether the right ventricle is abnormally small. It can also measure the levels of blood that can move through the hole in the septum where the valve is missing.

  • Electrocardiogram (ECG) – This test records the heart’s electrical activity and can verify if the chambers of the heart are enlarged.

  • Pulse Oximetry Test – The oxygen in the blood is measured using this test where sensors attached to the end of the finger.

  • Chest X-ray – X-ray technology can provide a detailed image to verify the enlargement of the heart and/or heart chambers. X-rays also reveal detailed information on the flow of blood to the lungs.

  • MRI – The doctor may order an MRI (magnetic resonance imaging) scan that uses radio waves and powerful magnets to capture an image of the heart muscle. This allows the doctor to identify or evaluate muscle damage, birth defects, heart tumors, or other serious problems.

  • Cardiac Catheterization – The doctor or technician will insert a flexible, thin catheter (tube) into a blood vessel, usually in the groin, that is guided up to and into the heart to accurately diagnose tricuspid atresia. Sometimes the test is performed before tricuspid atresia repair surgery to examine the heart muscle.

Only after the defect has been identified, diagnosed and verified can the doctor develop an effective treatment plan that typically requires numerous surgical procedures.

Tricuspid Atresia Treatments

To date, medical science has yet to determine how to replace a defective tricuspid valve. Instead, doctors will treat the condition using surgical procedures that provide an adequate flow of blood to the lungs from the heart. This will ensure the baby receives sufficient oxygen levels to the bloodstream.

Typically, the child will require an initial surgery to correct the defect followed by palliative surgeries that provide temporary fixes that increase the flow of blood immediately as the child develops. These procedures include:

  • Atrial Septostomy – The doctor will enlarge the heart’s upper chamber (atria) opening to provide room for more blood flow into the left atrium from the right atrium.

  • Shunting – Installing a shunt (bypass) from the aorta to the pulmonary arteries can ensure the baby’s lungs receive adequate blood flow.

  • Pulmonary Artery Band Placement – Sometimes there is too much blood flowing from the heart to the lungs, requiring the surgeon to restricts some flow using a pulmonary artery band.

  • Glenn Procedure – At some point during the child’s development, the shunt will become too small requiring the Glenn procedure that is performed in preparation for the Fontan procedure, a permanent corrective surgery.

  • Fontan Procedure – Nearly all children suffering from tricuspid are atresia will have the corrective Fontan procedure after they breached their second birthday. This procedure develops a pathway for oxygen-poor blood to redirect into the lungs.

  • Medications – Prior to surgery, the cardiologist often recommends that the child takes some form of prostaglandin drug that dilates (widens) the ductus arteriosus.

With follow-up care throughout the child’s lifetime into adulthood, most born with this defect can live normal healthy lives.

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Sources:

https://www.cincinnatichildrens.org/health/t/tricuspid

https://www.cdc.gov/ncbddd/heartdefects/tricuspid-atresia.html

https://my.clevelandclinic.org/health/articles/tricuspid-atresia

http://www.mayoclinic.org/diseases-conditions/tricuspid-atresia/basics/definition/con-20026734

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