EA: The Treatment Options

What are the Treatment Options for Ebstein's Anomaly (EA)?
If your child was recently diagnosed with EA, you are probably wondering what treatment options are available. 

Depending on the severity of  your child's symptoms, there are several options. Those children with mild EA who are free of symptoms require no medical treatment, although they should be closely monitored by the pediatric health provider. If your child is showing symptoms of EA, a healthcare provider may suggest medical treatments or surgery. 

Medical Treatments
The medical treatments for EA focus on treating the secondary effects - enlargement of the right side of the heart, the backflow of blood through the tricuspid valve, and right sided heart failure.[1] Issues on the right side of the heart result in a backup of blood in the rest of the body. This causes peripheral edema (swelling due to poor blood flow from the legs and arms back to the heart). Diuretic medications (those that help the body remove excess fluid in the urine) are a common treatment because they reduce the peripheral edema and the stress on the right side of the heart.[1] EA patients may also be given an anticoagulant medication to prevent clots of blood from forming.[1] Blood clots can be formed due to the slow flow of blood in the arms and legs, these clots can travel through the blood stream to the lungs causing a build up of fluid in the lungs and infection. 
Patients who are not showing symptoms should go to the doctor to followup every two or three years, but if symptoms arise they should go to the doctor and follow up every 6 months and discuss medical treatment options with their healthcare provider.[1]

The University of Washington has developed a model called the The Seattle Heart Failure Model that patients and their healthcare providers can use to identify patients who are at high risk for poor outcomes with EA and require more specialized treatment and intervention. Take this form, or the web address, to your child's doctor (or your doctor) to develop a treatment plan that fits your needs.[2]

Surgical Treatments
EA patients with arrhythmia of the heart (heart murmurs or abnormal heartbeats) usually must have surgery to repair the heart. Surgery will be performed on patients who have difficulty exercising due to the heart defect, cyanosis (blue tones to the skin), those who develop emboli (blood clots which travel through the blood stream), those with cardiomegaly (an increasing heart size), and those with right ventricular dilation (a size increase of the lower right heart chamber).[1] However, surgery is usually not performed on newborn babies due to immaturity of the respiratory system.[1]  These surgeries focus on rebuilding the tricuspid valve which is pushed downward into the right ventricle in EA.[1] The surgical techniques used depend on the patient and the severity of EA, but all aim to reduce the backflow of blood through the tricuspid valve, reduce arrhythmias, closing the foramen ovale, and placing a pacemaker (a device that controls and regulates heart beats) if necessary.[1] Your healthcare provider and heart surgeon will discuss these options with you. 
The following video is a surgical technique used to repair the tricupid valve - "Cone Reconstruction":

References
1. Arya P, Beroukhim R. Ebstein Anomaly: Assessment, Management, and Timing of Intervention. Curr Treat Options in Cardio Med. 2014. 16(10): 1-13. doi: 10.1007/s11936-014-0338-x. 
2. Seattle Heart Failure Model. University of Washington Web site. Available from: https://depts.washington.edu/shfm/. Accessed February 10, 2015.