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Ann Thorac Surg 2009;88:1975-1981. doi:10.1016/j.athoracsur.2009.07.019
© 2009 The Society of Thoracic Surgeons
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Sunil P. Malhotra
Ed Petrossian
Malcolm MacDonald
Olaf Reinhartz
Frank L. Hanley
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Right arrow Congenital - cyanotic

Original Articles: Pediatric Cardiac

Selective Right Ventricular Unloading and Novel Technical Concepts in Ebstein's Anomaly

Sunil P. Malhotra, MD*, Ed Petrossian, MD, V. Mohan Reddy, MD, Mary Qiu, BS, Katsushide Maeda, MD, Sam Suleman, BS, Malcolm MacDonald, MD, Olaf Reinhartz, MD, Frank L. Hanley, MD

Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Stanford, California

Accepted for publication July 2, 2009.

* Address correspondence to Dr Malhotra, Congenital Heart Center at the University of Florida, PO Box 100296, Gainesville, FL 32610-0296 (Email: spmalhotra{at}peds.ufl.edu).

Presented at the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.

Background: Favorable outcomes in Ebstein's anomaly are predicated on tricuspid valve competence and right ventricular function. Successful valve repair should be aggressively pursued to avoid the morbidity of prosthetic tricuspid valve replacement. We report our experience with valve-sparing intracardiac repair, emphasizing novel concepts and techniques of valve repair supplemented by selective bidirectional Glenn (BDG).

Methods: Between June 1993 and December 2008, 57 nonneonatal patients underwent Ebstein's anomaly repairs. The median age at operation was 8.1 years. All were symptomatic in New York Heart Association (NYHA) functional class II (n = 38), III (n = 17), or IV (n = 1). Preoperatively, 26 had mild or moderate cyanosis at rest. We used a number of valve reconstructive techniques that differed substantially from those currently described. BDG was performed in 31 patients (55%) who met specific criteria.

Results: No early or late deaths occurred. At the initial repair, 3 patients received a prosthetic valve. Four patients required reoperation for severe tricuspid regurgitation. Repeat repairs were successful in 2 patients. At follow-up (range, 3 months to 6 years), all patients were acyanotic and in NYHA class I. Tricuspid regurgitation was mild or less in 49 (86%) and moderate in 6 (11%). Freedom from a prosthesis was 91% (52 of 57).

Conclusions: Following a protocol using BDG for ventricular unloading in selected patients with Ebstein's anomaly can achieve a durable valve-sparing repair using the techniques described. Excellent functional midterm outcomes can be obtained with a selective one and a half ventricle approach to Ebstein's anomaly.






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