Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair

Abstract

Addition of a bidirectional cavopulmonary shunt to cone reconstruction of the tricuspid valve should be considered for young patients with severe Ebstein anomaly who are at high risk of right ventricular failure after the operation.

Restoration of Tricuspid Valve Mechanism at the Level of Displaced Septal and Posterior Leaflets in Ebstein's Anomaly

“…There is a general consensus that operative mortality in Ebstein's anomaly has been reduced dramatically with the introduction of a bidirectional superior cavopulmonary anastomosis in association with tricuspid valve repair . Various preoperative and intraoperative parameters, including right ventricular morphology and function, preoperative cyanosis, postoperative right atrial pressure, functional tricuspid orifice size, and postoperative hemodynamic instability, have been suggested to indicate the need for an additional bidirectional superior cavopulmonary anastomosis. There are also pitfalls of the bidirectional superior cavopulmonary anastomosis, such as facial edema and upper extremity numbness (particularly in the morning), jugular vein engorgement, headache, central venous thrombosis, and vascular inaccessibility through the superior vena cava to the heart.…”

One and a Half Ventricle Repair in Association with Tricuspid Valve Repair According to “Peacock Tail” Technique in Patients with Ebstein's Malformation and Failing Right Ventricle

“…3 Since the first reported cases of the BDG in patients with Ebstein's malformation, 14 few reports have demonstrated that the addition of a BDG to the TV repair decreases mortality. [1][2][3][4][5][6][15][16][17] The BDG in the setting of RV failure: (1) reduces the venous return to the enlarged, dysfunctional RV and (2) optimizes the LV preload. The construction of a BDG in patients with moderate or severe TVR reduces the regurgitation grade, allowing less aggressive TV interventions, 5 and decreasing the incidence of reoperation in patients with residual TVR.…”

“…Surgical therapy for Ebstein's malformation should restore TV competence and address the inadequacy of the inefficient RV. All the reported surgical strategies have in common the detachment and reimplantation of the major leaflets and complete plication of the atrialized portion of the RV …”

“…All the reported surgical strategies have in common the detachment and reimplantation of the major leaflets and complete plication of the atrialized portion of the RV. [1][2][3][4][5][6][7] When patients with Ebstein's malformation are diagnosed after childhood or adolescence, they present with cyanosis, arrhythmias, and severe RV dysfunction, frequently accompanied by left ventricular (LV) dysfunction. At this point the isolated conventional repair is either impossible or accompanied by an increased mortality and morbidity and poor functional results.…”

Anomalia de Ebstein

“…In this context, even in the presence of these risk elements, the cone technique can be applied, according to Liu et al 4 in 30 of these patients, of which in 20 of them Glenn's bidirectional procedure was also employed concurrently (1,5 ventricle repair) vis-à-vis marked dilatation and right ventricular dysfunction, in an average age of 60 months (ranging from 2 to 192 months). The results obtained by these authors 4 were also satisfactory, with reappearance of moderate to severe tricuspid regurgitation in four (13.3%) patients, with one death (3.3%). As already shown, Glenn's bidirectional operation is useful in combination with the cone plastic surgery in the presence of ventricular dysfunction, and cardiac transplantation is employed in rare extreme cases of ventricular dysfunction associated.…”