Ebstein's anomaly is a rare congenital (meaning it is present at birth) heart defect in which the heart’s tricuspid valve is abnormal. The tricuspid valve separates the right lower heart chamber (right ventricle) from the right upper heart chamber (right atrium). The defect usually causes the valve to work poorly, often resulting in blood flowing back into the right atrium instead of to the right ventricle and out to the lungs. The backup of blood flow can lead to heart swelling and fluid buildup in the body. There may also be a narrowing of the valve that leads to the lungs (the pulmonary valve).
In many cases, patients also have a hole in the wall separating the heart's two upper chambers, and blood flow through this hole may cause oxygen-poor blood to go to the body. This can result in a blueish tint to the skin, a condition called cyanosis. The exact cause is unknown, but the use of certain drugs such as lithium or benzodiazepines during pregnancy may play a role. It is more common among whites. Treatments vary but may include medication, oxygen and other breathing support, and surgery to correct the faulty valve.
UAB Medicine operates a Marfan Syndrome and Related Disorders Clinic, which is a multi-specialty clinic that includes physicians from the UAB Department of Pediatric Cardiology and the UAB Department of Genetics. Services offered include physical exams, echocardiogram/EKG, and genetic testing for diseases and conditions of the aorta, when needed. The clinic’s genetic counselor works with patients to help them better understand their condition and evaluate their risk for medical issues based on genetic testing results.
Because Marfan syndrome can affect the heart, patient care also may be provided by the UAB Congenital Heart Disease Program. The program offers the most advanced care for congenital (present at birth) heart disease, which often requires lifetime monitoring and care. Our multi-specialty team of pediatric and adult cardiologists, cardiovascular surgeons, cardiovascular anesthesiologists, and maternal-fetal medicine specialists have unique expertise in treating patients before birth and into adulthood.
UAB Medicine’s modern ultrasound equipment allows many heart defects to be diagnosed before a child is born. Screening exams performed at 18-20 weeks are recommended for expecting mothers or fathers known to have congenital heart disease. If a defect is discovered, our experts provide prenatal treatment and develop a plan for delivery and treatment after birth.
Thanks to advances in pediatric congenital care, the life expectancy for most patients now reaches far into adulthood. However, more than half of the people with congenital heart problems stop seeing a cardiologist once they turn 18. UAB’s Alabama Adult Congenital Heart Disease Program is designed to prevent that gap in care. As the only adult congenital heart disease program in the state and one of only a few in the country, our expertise greatly increases the chances that symptoms will be identified early. This helps ensure that less serious problems are addressed before they develop into larger, more life-threatening issues such as heart failure, arrhythmia, residual congenital heart defects, endocarditis, and stroke.
Dr. Cribbs on Business Break
More than 20,000 adults enter the Adult Congenital Heart Disease population every year, but 60% are lost to follow-up care.
Care of Adults with Congenital Heart Disease
Fetal Cardiac Services at UAB Medicine
Drs. Joseph R. Biggio and Robb L. Romp discuss how the UAB Fetal Diagnosis and Care Center brings together experts at UAB Medicine and Children's of Alabama Joseph S. Bruno Pediatric Heart Center to care for high risk moms and babies from the moment a congenital abnormality is suspected.
Individuals born with congenital heart disease are now thriving into adulthood, but need lifelong follow-up care from sub-specialty experts.
Adult Congenital Heart Disease Risk Factors, Symptoms & Treatments
Some people are born with a defect or malformation in their heart or blood vessels, and this is called Congenital Heart Disease. UAB Cardiologist Edward Colvin, MD, talks to Daytime Alabama on WVTM-TV, Channel 13, in Birmingham, Ala., about the types of congenital heart disease and what adults with this disease should look for when choosing a doctor.
UAB is an active participant in research and clinical trials for the diagnosis and treatment of Epstein's Anomaly. We encourage you to speak to your physician about research and clinical trial options and browse the link below for more information.View Clinical Trials
Celebrating Easter Safely During COVID-19
As Easter approaches, celebrating the holiday during a pandemic may seem more optimistic than last year, but community members should remain diligent in their efforts to prevent the spread of COVID-19.
Here are a few tips for celebrating Easter more safely this year:
Consult with your primary care physician for any concerns you have about the vaccine. The vaccines approved for use in the United States are intended to train the body to successfully fight the virus and minimize symptoms. There is still a possibility of contracting COVID-19 or infecting others even after vaccination.
UAB Medicine currently offers four vaccination sites across central Alabama. For more information appointment requests and eligibility, visit our COVID-19 vaccination website.
Wear a mask
Wear a mask securely over your nose and mouth, and make sure it fits snugly along the sides of your face. Mandates and guidelines for masking may vary by location, although the CDC still recommends wearing a mask in public indoor spaces and around those outside your household. There’s a health bonus, too: masking has been shown to reduce those pesky springtime allergies caused by various pollens in the air, according to a recent study.
If your place of worship offers an online or streaming service, consider tuning in to reduce the amount of in-person contact. Reach out to family, friends, or members of your congregation to see if there is a way to help them celebrate virtually. Instead of a traditional Easter egg hunt, coordinate with neighbors to place large, bright-colored paper eggs in their windows for neighbors to “hunt” while on a family walk.
If you still plan to hold or attend an in-person gathering, be sure there is ample space to spread out. Avoid poorly ventilated indoor spaces and buffet-style dining with shared serving utensils. Consider enjoying your Easter meal, or other activities and crafts, outside.
Wash Your Hands
Continue good hand hygiene before, during, and after meals and events. Keep a bottle of hand sanitizer in your purse, backpack, or car for times when soap and water is not easily accessible.
If you or someone in your household is experiencing symptoms, or has recently been exposed to someone with COVID-19, cancel plans to gather with others and seek medical attention as necessary.
With the rollout of vaccine distribution across the United States, it is tempting to rush toward the light at the end of the tunnel. Continue taking precautions to protect yourself and those around you.