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Learning about Ebstein’s Anomaly

Neonatal Ebstein’s Anomaly carries a poor prognosis, with a reported survival of only 68% in one series. Surgery is required in the presence of heart failure of profound cyanosis and there are different options for the surgical repair. Such as: Biventricular repair, single ventricle pathways with right ventricular exclusion, cardiac transplantation (rare). A Brazilian surgeon Dr Da Silva had developed the Cone Repair technique which has ever since been followed by many hospitals.

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Articles of Interest

Ebstein’s anomaly: contemporary management strategies

Ebstein’s anomaly (EA) is a rare congenital cardiac anomaly. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. Presentation varies from a severe symptomatic form during the neonatal period to an incidental detection later in life due to the wide morphological variation of the condition. The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. Some neonates initially committed to a single ventricle pathway may be converted to a biventricular repair by a delayed TV valvuloplasty. The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability.

Da Silva's cone repair for Ebstein's anomaly: effect on right ventricular size and function

Da Silva's cone repair for Ebstein's anomaly creates excellent valve function in all patients. Consecutively, the size of the RV decreases and the antegrade net stroke volume increases 6 months after the operation.

Ebstein Anomaly: A Complex Heart Defect

Ebstein anomaly is a rare heart defect, comprising less than 1% of all congenital heart issues. It happens in less than 1 in every 10,000 live births. While some instances are familial and inherited, most cases are spontaneous with no known cause. Etiology of the condition may be multifactorial, potentially involving environmental exposures and genetic predisposition.

Ebstein's Anomaly

Ebstein's anomaly is a rare congenital malformation that is characterized by abnormalities of the tricuspid valve and the right ventricle. The morphology of the abnormalities is highly variable, but in general, there is downward displacement of the septal and posterior leaflets in a spiral fashion.

Ebstein’s Anomaly

Ebstein’s anomaly is a complex congenital anomaly with a broad anatomic and clinical spectrum. Management is complex and must be individualized. Precise knowledge about the different anatomic and hemodynamic variables, associated malformations, and management options is essential.

Ebstein’s anomaly of the tricuspid valve: from fetus to adult

This congenital malformation is characterised by any degree of inferior displacement of the proximal attachments of the septal and posterior leaflets of the tricuspid valve from the atrioventricular ring. It occurs in about 1–5 per 200 000 births and represents <1% of all congenital heart disease.

Ebstein’s Anomaly: A Rare Disease Treated with World-class Care Here at Home

During his time at Children’s Hospital of Pittsburg of UPMC, Dr. Sharma worked with Dr. Jose da Silva, the foremost authority in the world on Ebstein’s Anomaly. In the early 1990s, while trying to repair the tricuspid valve in patients with Ebstein’s Anomaly, Dr. da Silva was concerned about the limitations of multiple techniques that were available at the time. As such, Dr. da Silva developed a novel repair method called the “Cone” procedure pioneered in his native Brazil (Arq Bras Cardiol, volume 82 (no 3), 217-20, 2004).

Ebstein’s anomaly: an electrophysiological perspective

Ebstein’s anomaly of the tricuspid valve (EA) is an uncommon congenital cardiac malformation. It can present with atrioventricular tachycardia (AVRT), atrioventricular nodal re-entrant tachycardia (AVNRT), atrial arrhythmias, and rarely with ventricular tachycardia.

Ebstein’s Anomaly: Esme’s Story

Esme’s heart condition, Ebstein’s Anomaly, wasn’t detected during Stacey’s pregnancy. Esme appeared to be perfectly healthy after birth, but a routine pulse oximetry test showed that her blood oxygen level was low. This resulted in her referral and speedy diagnosis...

What is Ebstein’s anomaly?

The tricuspid valve is normally situated between the two chambers on the right side of the heart, the right atrium and right ventricle. It normally has 3 flaps also known as leaflets. In Ebstein’s anomaly, the tricuspid valve sits lower than normal in the right ventricle and the leaflets are abnormally formed. This leads to blood leaking backwards from the right ventricle into the right atrium (tricuspid valve regurgitation) and it causes the right atrium to enlarge and not work as it should.

Resources

Da Silva Center for Ebstein's Anomaly

As a trainee in cardiovascular surgery at the Cleveland Clinic Foundation in the late 1970s, José Pedro da Silva, MD, developed a special interest in patients with a rare heart defect known as Ebstein’s anomaly that was challenging to repair.

Ebstein's Anomaly Foundation

ABOUT US Arden with her Husband Sven I was born with Ebstein’s Anomaly in 1996 and had my first surgery in May of 2007. After working with many of the top surgeons around the country, it’s clear that advances in new valves and new techniques in surgery will greatly improve the outcomes of kids and adults going forward. I will graduated from Wake Forest University in 2018 and will continue to work with my foundation and help kids and families understand this rare condition.

StatPearls

Ebstein anomaly is a rare congenital cardiac abnormality involving the tricuspid valve and the right ventricle (RV). Ebstein anomalies comprise < 1% of congenital heart defects The anomaly was initially described by the pathologist Wilhelm Ebstein...