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.

Current Interventional and Surgical Management of Congenital Heart Disease

“…To minimize RV dilatation when it is dilated and poorly functioning, increasing heart rates (100-120 bpm) with temporary atrial pacing may be needed. 20,21,67,68 This helps to minimize RV distention by reducing filling time and minimizes tension on a tricuspid repair, annular, and ventricular suture lines. Low-dose vasopressin can be helpful with rightsided heart failure because systemic vasoplegia is not uncommon and may be caused by the use of preoperative afterload reducing agents, liver congestion, or humoral factors from atrial natriuretic peptide secondary to atrial dilation.…”

“…Medical therapy at hospital discharge for many patients undergoing valve surgery often includes β-blocker therapy. 67 In the setting of depressed systemic ventricular function, the addition of angiotensin-converting enzyme inhibitor is common. Short-term sildenafil (6-8 weeks) can be considered in patients with poor RV function, although evidence for this strategy is lacking.…”

Ebstein’s anomaly treated surgically in an adult female

“…In selected EA cases, TV plasty and reduction of RV size is believed to be the best method of correction. The formation of a "cone" or double-leaflet valve is recommended to ensure normal function of the newly created TV [4][5][6]. The septal and/or posterior cusp of the tricuspid valve is absent in high-grade EA (in our patient), which makes the reconstruction of a double-leaflet valve impossible.…”

Percutaneous Tricuspid Valve Replacement: Two Cases of Valve-in-Valve and Valve-in-Ring

“…Patients with Ebstein's anomaly often suffer from RV dysfunction and restrictive diastolic function and may have undergone numerous surgical repairs and replacements. Moreover, they are at increased risk for both atrial and ventricular arrhythmias [12]. As the morbidity and mortality risk associated with surgical TV replacement is often high [5], the excellent short-and intermediate-term outcomes of trans-catheter valve replacement make it a viable alternative.…”