Harald Kaemmerer, Annalisa Angelini, Pedro Trigo-Trindade, Christian Schreiber, Juan V. Comas, Jörg Stein
Ebstein anomaly (EA) is a rare congenital abnormality affecting the tricuspid valve (TV) and the right ventricle. It is characterized by downward displacement of the functional tricuspid annulus, atrialization of a portion of the right ventricle with dilatation of the right atrioventricular junction, thinning of the affected wall and plastering of the leaflets to the underlying ventricular myocardium (lack of leaflet delamination) with fenestration, redundancy and tethering of the anterior leaflet. Functionally the valve is usually incompetent. Accessory conduction pathways are frequently found. Several classifications to describe the TV anomaly have been proposed, for surgical decision-making the Carpentier classification (Types A-D) is widely used. In asymptomatic patients conservative treatment is recommended. In symptomatic patients, interventional or surgical treatment has to be evaluated. According to the native anatomy of the TV, different surgical options (TV repair or TV replacement, +/- other options) may apply. To avoid TV replacement several repair techniques have been developed, including annuloplasty, closure of commissures, and creation of a “monocusp” valve. More recently, da Silva proposed the “cone reconstruction” of the TV leaflet tissue. Furthermore, patients with right ventricular failure may benefit from a cavo-bipulmonary anastomosis. In the current era, results of surgery in adults have low early mortality in experienced centres, and long-term survival is satisfactory. Prognosis after surgical repair depends on post-operative improvement and lower age at surgery. Apart from the residual hemodynamic problems, arrhythmia management remains challenging. In any case, the management of patients with EA is complex and has to be individualized. Close collaboration with a cardiologist experienced in congenital heart disease is mandatory.
Ebstein's malformation: recent advances
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