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J Thorac Cardiovasc Surg 2007;134:1381
© 2007 The American Association for Thoracic Surgery

Letter to the Editor

Surgery of Ebstein anomaly

Cardiovascular Surgery Department, Hôpital Européen Georges Pompidou, Paris, France

To the Editor:

I read with great interest the article by Quinonez and colleagues¹ concerning surgical treatment of the Ebstein anomaly. This very informative article underlines the role of the right ventricle in the outcome of this congenital anomaly. As my colleagues and I² pointed out almost 10 years ago, impairment of the right ventricle is a major prognostic factor after surgery for the Ebstein anomaly. Inasmuch as medical therapy is difficult to manage, it seemed to us that the association of a bidirectional cavopulmonary shunt (BCPS) would be helpful in the early follow-up period. This continues to be our policy.

The term used by the authors, "1.5-ventricle," does not seem to me to be adequate. The flow of the superior vena cava varies between one third and one half of the cardiac ouput.³ The 1.5-ventricle concept is valid when one of the ventricles is anatomically partially deficient.

The article by Quinonez and associates does not answer a question that arises immediately: what are the indications for the BCPS? Their definition of "failing right ventricle" is not clear. In their series, 2 children were free of symptom, whereas, on the other hand, 3 adult patients were on the transplantation list. Right ventricular enlargement could be an indicator, but we are still looking for quantitative data. In a study of the right and left ventricular volumes before and after surgery, my colleagues and I⁴ were unable to determine a threshold value for patients "at risk" and those that were "safe." In our experience, a large atrialized right ventricle (even if excluded after surgery), a very thin infundibular right ventricular wall, and/or a paradoxic septal motion are indications for a superior vena cava derivation. Among 105 patients with Ebstein repair and BPCS, we did not observe any deleterious effect of the shunt except for a transient swelling of the neck in 1 patient.

The BCPS does not solve all of the left and right ventricular problems. One of the patients in the authors’ series is not improved and 2 in our own series had to undergo transplantation.

However, the associated anastomosis is an excellent additional procedure in patients with difficult indications.

References

1. Quinonez LG, Dearani JA, Puga FJ, O’Leary PW, Driscoll DJ, Connolly HM, et al. Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg 2007;133:1303-1310.[Abstract/Free Full Text]
   
2. Chauvaud SM, Fuzellier JF, Berrebi A, Lajos P, Marino JP, Mihaileanu S, et al. Bi-directional cavopulmonary shunt associated with ventriculo and valvuloplasty in Ebstein’s anomaly: benefits in high risk patients. Eur J Cardiothorac Surg 1998;13:514-519.[Abstract/Free Full Text]
   
3. Seliem MA, Baffa JM, Vetter JM, Chen SL, Chin AJ, Norwood Jr WI. Changes in right ventricular geometry and heart rate early after hemi-Fontan procedure. Ann Thorac Surg 1993;55:1508-1512.[Abstract/Free Full Text]
   
4. Chauvaud SM, Hernigou AC, Mousseaux ER, Sidi D, Hébert JL. Ventricular volumes in Ebstein’s anomaly: x-ray multislice computed tomography before and after repair. Ann Thorac Surg 2006;81:1443-1449.[Abstract/Free Full Text]
   

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