Repair of Ebstein’s anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up

Ebstein’s Anomaly

“…Marked cardiac enlargement, advanced echocardiographic severity score, cyanosis, and severe regurgitation of the tricuspid valve all predict neonatal death without surgery. 19,71 Biventricular repairs in combination with correction of all associated cardiac defects are feasible, and midterm results are good. 71 Conversion to a single-ventricle approach for symptomatic neonates also has been advocated.…”

“…19,71 Biventricular repairs in combination with correction of all associated cardiac defects are feasible, and midterm results are good. 71 Conversion to a single-ventricle approach for symptomatic neonates also has been advocated. 72 Results of tricuspid valve repair in young children have been reported and demonstrate low early mortality and good durability at late follow-up.…”

Ebstein-Anomalie des Fetus

“…In die ser prog nos tisch ungüns ti gen Grup pe der Eb stein-Ano malie, ins be son de re bei ei ner RA-Flä chen-Index >1 und feh len dem Vor wärts fluss über die Pul mo nal klap pe, ist ein ab war ten des Ma nage ment fast nie er folg reich. Der Einsatz ei ner prä-und post ope ra ti ven me chani schen Kreis lau fun ter stüt zung, die In hala ti on von Stick stoff mon oxid (NO) zur Sen kung des pul mo na len Ge fäß wi derstands und zur Dif fe ren zie rung zwi schen ei ner mor pho lo gisch de ter mi nier ten und ei ner funk tio nel len Pul mo nala tre sie [7], Kor rek tu r ope ra tio nen an der Tri ku spid alklap pe, Re sek ti on und Raf fung des ver grö-ßer ten rech ten Vor hofs so wie an de re neue ope ra ti ve Tech ni ken, wie die uni ven tri kulä re Kor rek tur mit Aus schal tung des rechten Ven tri kels durch Schaf fen ei ner funktio nel len Tri ku spid al klap pe na tre sie kön-nen zum Über le ben die ser schwer kranken Neu ge bo re nen bei tra gen. Da rü ber hinaus ste hen in Ein zel fäl len in ter ven tio nelle The ra pie ver fah ren zur Be hand lung einer Pul mo nal klap pe na tre sie und -ste no se so wie zum Ver schluss des Duc tus ar te riosus so wie bei schwe ren links ven tri ku lä rer Funk ti ons stö run gen eine Herz trans plan tati on zur Ver fü gung [5,9,13,14,15,18,19,32,33,35,36,47,50,51,52].…”

Novel Approaches to Cardiac Valve Repair

“…Early attempts at repair of the tricuspid valve in Ebstein's anomaly concentrated on "correcting" the downward displacement of the hinge mechanism of the septal and inferior leaflets into the right ventricle and obliterating the "atrialized" ventricle. More recent methods of repair 79,80 ( Figure 5) depend on creating a monocuspid valve using the reconstructed anterosuperior leaflet, which is almost always present as a large sail-like structure with adequate tissue for repair. It is equally important, however, to realize that this leaflet is highly abnormal (a fact recognized by Wilhelm Ebstein in his original article in 1866) 81 in shape, location, and size with limited mobility because of the presence of abnormal chordae and the frequent direct attachment of the free border of the cusp to the right ventricular myocardium.…”

“…It is equally important, however, to realize that this leaflet is highly abnormal (a fact recognized by Wilhelm Ebstein in his original article in 1866) 81 in shape, location, and size with limited mobility because of the presence of abnormal chordae and the frequent direct attachment of the free border of the cusp to the right ventricular myocardium. 79,80 These latter abnormalities can produce stenosis and abnormal orientation of the orifice of the valve. Vertical plication of the atrialized right ventricle as advocated by Carpentier and Chauvaud has the advantage of reducing the size of the annulus and the atrialized ventricle but carries a small risk of injuring the right coronary artery.…”