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J Thorac Cardiovasc Surg 2008;135:1182-1183
© 2008 The American Association for Thoracic Surgery

Brief Communication

Right ventricular exclusion procedure with total cavopulmonary connection: An alternative operative approach in adults with severe Ebstein anomaly

^a Children's Hospital Los Angeles, Los Angeles, Calif
^b University of Southern California Keck School of Medicine, Los Angeles, Calif

Received for publication August 27, 2007; accepted for publication October 15, 2007.

^_* Address for reprints: Anastasios C. Polimenakos, MD, Children's Hospital Los Angeles, Cardiothoracic Surgery, 4650 W. Sunset Blvd, Mailbox #66, Los Angeles, CA 90027. (Email: anapolisis@aol.com).

The first 20% of the full text of this article appears below.

Ebstein anomaly represents less than 1% of all congenital heart defects. This anomaly affects the tricuspid valve (TV), but also represents a malformation of the right ventricle (RV).¹

In symptomatic neonates, we found that single-ventricle palliation has its major impact on a combination of a poorly functioning RV, type IV Ebstein anomaly,¹ and an unguarded TV with RV outflow tract obstruction.² Our experience in older children and adults is similar to that in other published series on valve reconstruction. We describe an unusual presentation of adult Ebstein anomaly that prompted successful single-ventricle palliation.

Clinical Summary

A 19-year-old man with a diagnosis of symptomatic severe Ebstein anomaly and previous atrial septal defect device closure underwent a combined total cavopulmonary connection with fenestrated RV exclusion technique and explantation of the Amplatzer (AGA Medical Corp, Golden Valley, Minn) occlusion device. Before the implantation of the occlusion device, the patient was experiencing worsening cyanosis and exercise intolerance. Post-intervention, he presented with increasing frequency of palpitations . . . [Full Text of this Article]