Ebstein's Anomaly In Children

When a pregnant woman takes lithium during her pregnancy, her baby is most likely to have congenital heart disease. Pregnancy-related lithium use and bipolar disorder in the mother increase the risk of Ebstein's abnormality.
The tricuspid valve in Ebstein's anomaly is essentially malformed, as seen in the image of the slide above, which shows a greatly enlarged right atrium due to an improperly situated tricuspid valve. It is positioned considerably lower than it ought to be.  Leakage from the tricuspid valve. It causes the right ventricle to atrialize and raises the risk of tricuspid regurgitation.

The heart seen in Ebstein's anomaly can be seen in the hand-drawn diagram above. The tricuspid valve's primary portion, which should have been a component of the right ventricle, is now a part of the right atrium due to its placement being significantly lower than where it should be.The right ventricle's atrialization is the term for this ailment. As a result, the right ventricle becomes extremely tiny, has a lower output, and reduces pulmonary blood flow. This ailment is the precursor to cyanosis.

The tricuspid valve that is now in place has a regurgitant quality, which causes some blood to return to the right atrium. Additionally, because the right atrium already has a significant volume of blood, blood volume transfers from the right atrium to the left atrium. These patients have an active right-to-left shunt. Ebstein's anomaly is characterized by this shunt as well as reduced pulmonary blood flow.

Also Read: Arrhythmias: Drug Principles And Indications

Key Features Of Ebstein's Anomaly

An abnormal tricuspid valve position that causes the right ventricle to atrialize. Tricuspid regurgitation, caused by a leaky tricuspid valve, will occur. (Over 70% of instances) . The right ventricular outflow path is frequently partially obstructed by the anterior leaflet of the tricuspid valve. Functional blockage of the right ventricle's outflow pathway will result from this. Only 30 to 40% of the patients have this.

The majority of these individuals require a right-to-left shunt at the atrium level in order to be compatible with life. (PFO is rare; ASD is common).
Because the pulmonary blood flow is often lower, the patent ductus arteriosus is necessary.

Also Read: Chest X-ray In Pediatric Cardiology

Clinical Features Of Ebstein's Anomaly 

The patient's newborn stage is when the most prevalent presentation occurs.  The ductus arteriosus closes immediately, resulting in gradual and severe cyanosis. Due to tricuspid regurgitation, some of these patients will have a pan-systolic or homosystolic murmur in the lower left sternal region. Some patients will also have mid-systolic ejection clicks.

The majority of these individuals also have triple and quadruple rhythms (S1, S2, S3, and S4).  Uncommon forms could be less well-known and become noticeable later in life.

Important Associations 

There are 2 associations of Ebstein’s anomaly.

 Firstly, it is discovered that cardiomyopathy affects 15–20% of the patients. The function of the left ventricle might be adversely affected by left ventricular non-compaction. There are moments when the left ventricle has higher blood flow than usual. As a result, the result is appalling. These kids do in fact make it.

Secondly, Ebstein's abnormality is linked to Wolff Parkinson White Syndrome in 10 to 15% of patients. The Kent pathway is an aberrant and abnormal route. This mechanism will make this syndrome more likely to result in supraventricular tachycardia. Adenosine can treat this tachycardia, but long-term beta-blocker medication may be necessary.

After surgery, it is frequently discovered that WPW syndrome requires further care, including radiofrequency ablation of the aberrant tract. If the patient experiences recurrent arrhythmias, their hemodynamic instability will not improve.

Investigations 

Chest X-ray

 A chest X-ray reveals the presence of significant cardiomegaly. The patient has a balloon-shaped heart, which is also referred to as a box-shaped heart occasionally. Sometimes the cardiac silhouette is so big that it almost completely fills the pulmonary shadow, extending from one end to the other. The edges also appear to be somewhat squared off.Pulmonary oligemia will result from decreased pulmonary blood flow, yet each patient will have varying degrees of oligemia.

Upon doing an ECG, it is discovered that:  Nearly 80% of these patients will exhibit signs of right-sided bundle branch block.  "Himalayan P waves," or taller and broader P waves, are discovered as a result of significant R A expansion.
For the patients, a two-dimensional echocardiography will be diagnostic.

Also Read: Fetal Circulation And Transition To Neonatal Circulation

Management

The majority of newborn babies will need a prostaglandin E1 effusion in order to be stabilized. The majority of patients then needed an emergency BT shunt to be implanted. Following an emergency BT Shunt, the patient may choose between a definitive repair and the creation of a right ventricular pulmonary artery conduit.

 If the patient's right ventricle is only slightly smaller, this is feasible. The patient may choose to have a modified Glenn shunt.

The Outcome of Ebstien's Anomaly

Approximately 18% of newborns with Ebstein's abnormality who have symptoms pass away during the neonatal stage.  Thirty percent of the patients pass away before turning ten.

Also Read: Arrhythmias in Children : NEET SS Pediatrics

 Definitive Repair Procedures in Ebstein's Anomaly

1. Danielson method- • Fifty percent of the time, this works.
2. The Carpentier approach, which works in 15% of situations.
3. Replacement of the tricuspid valve and closure of the ASD. This type of repair works on the remaining percentage.

Each of these procedures has certain indications and contraindications. These are intricate methods that are frequently determined to be outside the scope. The best course of action varies depending on the child's unique anatomy and is not always the same for all conditions.

Hope you found this blog helpful for your NEET SS Pediatrics Cardiology preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.