Valve Atresia: Types, Symptoms, Causes, and More

Jan 08, 2024

Valve atresia is a congenital disorder (a condition present from birth) in which the heart valves do not fully form. It is diagnosed during pregnancy or shortly after birth. Valve atresia is considered a critical heart defect, occurring in about 1% of babies born each year (approximately 40,000 births).

The heart has four chambers, with a valve separating each chamber. Valves move blood from the heart to the lungs and deliver oxygen-rich blood to the rest of the body. Serious complications can occur if the heart valves are not ensuring proper blood flow.

This article will explore the types of valve atresia and symptoms, causes, and treatment of this congenital disorder.

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Valve atresia can occur in any of the four heart valves and disrupt regular blood flow. There are four major types of valve atresia.

Pulmonary valve atresia with intact ventricular septum is when the pulmonary valve does not form, but the wall that separates the right and left ventricles (the bottom part of the heart) is intact. As the fetus's heart develops during pregnancy, the right ventricle does not receive adequate blood flow, leading to problems pumping blood to the lungs and body.

A ventricular septal defect is a hole between the bottom chambers of the heart, which allows blood to flow in and out of the right ventricle. When this occurs, the right ventricle develops more than in babies with an intact ventricular septum.

Tricuspid atresia occurs when the tricuspid valve (the valve that controls blood flow from the upper right chamber of the heart to the lower right chamber) does not form. This stops blood flow to the lungs for oxygen and may require surgery soon after birth.

Aortic valvular atresia occurs when the flaps of the aortic valve are fused at birth, causing the right ventricle to be larger than normal and the left ventricle to be significantly smaller than normal. Having an undersized left ventricle deprives the body of adequate oxygen-rich blood.

Abnormal blood flow and lack of oxygen-rich blood caused by valve atresia can cause several symptoms in newborns, including:

The cause of most congenital heart disorders is unknown. Emerging research suggests that they are likely due to genetic abnormalities.

Maintaining proper nutrition and avoiding tobacco, alcohol, and certain medications during pregnancy are also important in ensuring optimal infant heart development. Maternal conditions, such as diabetes or obesity, have been linked to congenital disorders.

Women may undergo ultrasounds during pregnancy to ensure fetal growth and development are progressing as expected. If a critical congenital heart disorder, like valve atresia, is suspected during an ultrasound, a fetal echocardiogram might be done to verify the suspicion.

Even if the baby seems healthy at birth, symptoms of valve atresia can appear within a few hours. A healthcare provider may screen a newborn to identify signs of a critical congenital heart disorder.

Screening for valve atresia is a simple and painless procedure known as pulse oximetry. This involves placing sensors on the baby's skin to measure the oxygen levels in the blood. Low oxygen levels are a sign of a critical congenital heart disorder and would result in further testing.

All newborns should also receive a physical examination by a healthcare provider. During the exam, the provider will listen to the baby's heart for abnormal sounds, called murmurs, which can be heard using a stethoscope.

Maintaining proper blood flow patterns and supplemental oxygen support are often the initial interventions to ensure the baby is stable enough for treatment. Treatment will depend on the severity of the heart condition and the overall health of the baby and could include:

The baby will require monitoring by a healthcare provider for proper growth, progress, and potential health concerns, even if the critical congenital heart disorder is repaired. The long-term prognosis depends on the type of valve atresia and any other heart or lung disorders.

Many babies born with critical congenital heart disorders like valve atresia live into adulthood. Still, they tend to be more susceptible to abnormal heart rhythms, heart infections, and weakness of the heart muscles. Ongoing monitoring by a healthcare provider is necessary to ensure optimal health.

Valvular atresias are classified as critical congenital heart disorders. Often diagnosed in utero or shortly after birth, babies with critical congenital heart defects usually need surgical intervention to repair the heart or restore proper blood flow. Although critical congenital heart disorders can be repaired, people affected by this condition may require lifelong monitoring to prevent additional complications.

Hearing your baby might have a critical congenital heart disorder can be overwhelming. The earlier you receive a diagnosis, the earlier your baby can receive the treatment they need. Talk to your provider about your concerns and reach out to friends and family members for support.

Centers for Disease Control and Prevention. Congenital heart defects.

Centers for Disease Control and Prevention. Facts about pulmonary atresia.

Centers for Disease Control and Prevention. Facts about tricuspid atresia.

StatPearls. Aortic valvular atresia.

Medline Plus. Pulmonary atresia.

Heart.org. Single ventricle defects.

Centers for Disease Control and Prevention. Critical congenital heart defects.

Centers for Disease Control and Prevention. What are congenital heart defects.

By Pamela Assid, DNP, RNPamela Assid, DNP, RN, is a board-certified nursing specialist with over 25 years of expertise in emergency, pediatric, and leadership roles.