EA: What to Look For

EA: The Signs and Symptoms
Ebstein's Anomaly (EA) is usually identified before or just after birth, but can be identified in childhood, adolescence, or adulthood depending on how severe the tricuspid abnormality is. The signs and symptoms of EA vary depending on the age of the individual. 

Before Birth: EA can be detected before birth during routine ultrasounds. The backward flow of blood through the tricuspid valve can lead to symptoms of heart failure, incomplete lung development, or an enlarged heart.[1]

Newborn: A blue tone to the skin (cyanosis) in newborns indicates a low supply of oxygen in the blood and is a common sign of EA in newborns. When the blood pressure rises, such as when the newborn cries, the cyanosis may become more noticeable. Additional signs are abnormal heart beats (arrythmias), enlargement of the right side of the heart.[1]

Figure 1. A newborn with blue skin around the mouth (cyanosis)
which indicates a low oxygen supply in the blood. [2]

Infancy and Adolescence: In infancy and adolescence the symptoms of EA include cough, fatigue, shortness of breath, rapid and shallow breathing with great effort, fast heartbeat, and failure to thrive (difficulty gaining weight and growing appropriately). These symptoms are usually caused by failure of the right side of the heart to pump enough blood to the lungs and failure of the left side of the heart to pump enough blood to the body. The severity of these symptoms depends on how displaced the tricuspid valve is.[1]

Figure 2. The child at the left suffers from failure to thrive,
while the child at the right is a normal weight child of the
same age. The energy children expend to get enough oxygen
in the blood while suffering from EA can lead to the wasted
appearance of the child on the left. [3]

Adulthood: In adult patients EA may be detected by an abnormal electrocardiogram (ECG). Abnormal heart rythyms on the ECG are due to the stress of EA making the right side of the heart bigger than normal. The most common type of irregular rhythm of the heart in a patient with EA is supraventricular tachycardia (SVT), where the heartbeat is abnormally fast and prevents the lower chambers from completely filling with blood, decreasing the amount of blood that is pushed to the body. They may also show signs of atrial enlargement on the ECG.[1}

Figure 3. An ECG showing supraventricular tachycardia,
a rhythm common in adults with EA. [4]

References:

1. Galea J, Ellul S, Schembri A, et al. Ebstein Anomaly: A Review. Neonat Network.  September 2014: 33(5): 268-274.
2. Perioral Cyanosis. Stanford School of Medicine Web site. Available from: http://newborns.stanford.edu/PhotoGallery/PerioralCyanosis1.html. Accessed January 23, 2015.
3. Failure to Thrive. Mr. Doctor Web site. Available from: http://mrdoctor.org/failure-to-thrive-in-infants-and-children/ Accessed January 23, 2015.
4. Supraventricular Tachycardia (SVT). Still image from YouTube Web site. Available from; https://www.youtube.com/watch?v=zLzJUOa54fc. Accessed January 23, 2015.