Ebstein's Malformation of the Tricuspid Valve

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Abstract
Two cases of complete correction of Ebstein's malformation are presented. The deformed tricuspid valve was excised. A ball-valve prosthesis was anchored at the true annulus. This procedure removes the obstruction to right ventricular outflow, eliminates tricuspid insufficiency, increases right ventricular diastolic capacity, and improves the pumping action of the right ventricle by increasing the size and effectiveness of the pump chamber. Preoperatively, both patients were cyanotic because of large right-to-left shunts at the atrial level. No attempt was made to close the associated atrial septal defect in our first case. This patient continued to shunt right-to-left postoperatively but expired early in the postoperative period as a result of right-heart failure. A flap-valve type closure of the large associated atrial septal defect in the second patient with Ebstein's malformation permitted right-to-left shunting until the right ventricle became competent to pump the entire venous return to the lungs. About two weeks after surgery the right-to-left shunting ceased in this patient. Both patients developed complete heart block during the tricuspid valve prosthesis insertion. This complication was compensated for by a myocardial electrode and external pacemaker in the early postoperative interval. The second patient was discharged in an asymptomatic condition following insertion of an implantable pacemaker. A possible method for avoiding heart block in these cases in the future is discussed.

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