Ebstein anomaly review: what’s now, what’s next?

Abstract

Ebstein anomaly accounts for 1% of all congenital heart disease. It is a right ventricular myopathy with failure of tricuspid valve delamination and highly variable tricuspid valve morphology that usually results in severe regurgitation. It is the only congenital heart lesion that has a range of clinical presentations, from the severely symptomatic neonate to an asymptomatic adult. Neonatal operation has high operative mortality, whereas operation performed beyond infancy and into adulthood has low operative mortality. Late survival and quality of life for hospital survivors are excellent for the majority of patients in all age brackets. Atrial tachyarrhythmias are the most common late complication. There have been more techniques of tricuspid repair reported in the literature than any other congenital or acquired cardiac lesion. This is largely due to the infinite anatomic variability encountered with this anomaly. The cone reconstruction of Ebstein anomaly can achieve near anatomic restoration of the tricuspid valve anatomy. Early and intermediate results with these repairs are promising. Reduced right ventricular function continues to be a challenge for some patients, as is the need for reoperation for recurrent tricuspid regurgitation. The purpose of this article is to outline the current standard of care for diagnosis and treatment of Ebstein anomaly and describe innovative strategies to address poor right ventricular function and associated right-sided heart failure.

Keywords:

• cone reconstruction
• Ebstein anomaly
• stem cell therapy

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. The authors gratefully acknowledge Rhonda Brincks for her preparation of this manuscript.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Ebstein anomaly is a right ventricular myopathy with severe tricuspid regurgitation and reduced right ventricular function.

• Clinical presentation can range from the severely symptomatic neonate to asymptomatic adult.

• Essential imaging consists of echocardiography for valve anatomy and magnetic resonance imaging for ventricular dimensions and function.

• Neonatal operation has high operative mortality, operation in child and adulthood has low operative mortality.

• Atrial tachyarrhythmias are common pre- and postoperatively and electrophysiologic mapping/ablation may be required in many.

• Numerous tricuspid repair techniques exist, and results are variable due to the rarity of this lesion and the unpredictable anatomic findings encountered with each patient.

• The cone reconstruction can achieve nearly anatomic restorations of tricuspid valve anatomy.

• Poor right ventricular function can be a challenge for some patients and surgical and regenerative medicine techniques continue to evolve.