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Improving results of atrioventricular valve repair in challenging patients with heterotaxy syndrome*

Published online by Cambridge University Press:  04 March 2010

Noritaka Ota ,
Yoshifumi Fujimoto ,
Keiichi Hirose ,
Yuko Tosaka ,
Tomohiro Nakata ,
Yujiro Ide  and
Kisaburo Sakamoto
Noritaka Ota
Affiliation:
Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka, 420-8660, Japan
Yoshifumi Fujimoto
Affiliation:
Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka, 420-8660, Japan
Keiichi Hirose
Affiliation:
Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka, 420-8660, Japan
Yuko Tosaka
Affiliation:
Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka, 420-8660, Japan
Tomohiro Nakata
Affiliation:
Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka, 420-8660, Japan
Yujiro Ide
Affiliation:
Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka, 420-8660, Japan
Kisaburo Sakamoto*
Affiliation:
Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka, 420-8660, Japan
*
Correspondence to: Kisaburo Sakamoto, Shizuoka Children’s Hospital, 860 Urushiyama, Aoi-ku, Shizuoka City, Shizuoka 420-8660 Japan. Tel: +81 54 247 6251; Fax: +81 54 247 6259; E-mail: ksakamoto@sch.pref.shizuoka.jp
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Abstract

Objectives

Heterotaxy syndrome, which is often associated with atrioventricular valvar regurgitation, has been considered a risk factor for the surgical repair for Fontan candidates. The results of atrioventricular valve repair in this challenging patient population remain largely unknown.

Methods

From July, 1994 to January, 2007, 25 patients with the heterotaxy syndrome consisting of 22 right isomerism and three left isomerism presented to the Shizuoka Children’s Hospital Japan with significant (3–4+) atrioventricular valvar regurgitation necessitating atrioventricular valve repair. The clinical and Doppler/echocardiographic data were retrospectively reviewed to determine the efficacy of the repair and patient outcome.

Results

Patients were divided into two groups on the basis of atrioventricular valvar regurgitation at the most recent follow-up: those with a successful late outcome, (0–2) and those with a poor outcome (3–4). There were 17 (67%) patients with a successful outcome and 8 (33%) with a poor outcome. The repair technique including leaflet apposition was predictive of a successful outcome (p = 0.003). Overall survival was 64% (16/25). Survival was 88.2% (15/17) for patients with a successful result versus 12.5% (1/8) for those with a poor outcome (p = 0.0007). Of the 15 survivors, 13 have reached final completion of the Fontan circulation, and two currently remain at the bi-directional Glenn shunt stage.

Conclusion

Atrioventricular valve repair can be accomplished in this challenging patient population with excellent results. The combination of the leaflet apposition technique and the Kay suture can be performed with an excellent outcome in the majority of patients with heterotaxy syndrome, even with significant atrioventricular valvar regurgitation.

Keywords

Atrioventricular regurgitationleaflet appositionsingle ventricle
Type
Original Articles
Information
Cardiology in the Young , Volume 20 , Issue 1 , February 2010 , pp. 60 - 65
Copyright
Copyright © Cambridge University Press 2010

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Footnotes

*

This paper was presented at the Inaugural Meeting of The World Society for Pediatric and Congenital Heart Surgery in Washington.

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