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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: [email protected]
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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.

References

1.Driscoll, DJ, Offord, KF, Feldt, RH, Schaff, HV, Puga, FJ, Danielson, GK. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992; 85: 469–496.CrossRefGoogle ScholarPubMed
2.Gentles, TL, Mayer, JE Jr, Gauvreau, K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiocvasc Surg 1997; 114: 376–391.CrossRefGoogle ScholarPubMed
3.Hashmi, A, Abu-Sulaiman, R, McCrindle, BW, Smallhorn, JF, Williams, WG, Freedom, RW. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998; 31: 1120–1126.Google Scholar
4.Gaynor, JW, Collins, MH, Rychik, R, Gaughan, JP, Spray, TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1999; 117: 506–514.Google Scholar
5.Gilljam, T, McCrindle, BW, Smallhorn, JF, Williams, WG, Freedom, RM. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 2000; 36: 908–916.CrossRefGoogle Scholar
6.Humed, RA, Feldt, RH, Porter, CJ, Julsrud, PR, Puga, FJ, Danielson, GK. The modified Fontan operation for Asplenia and polysplenia syndromes. J Thorac Cardiovasc Surg 1988; 96: 212–218.Google Scholar
7.Culbertson, CB, George, BL, Day, RW, Laks, H, Williams, RG. Factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure. J Am Coll Cardiol 1992; 20: 678–684.Google Scholar
8.Smith, A, Ho, SY, Anderson, RH, Connell, MG, Arnold, R, Wilkinsion, JL. The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialized conduction system. Cardiol Young 2006; 16: 437–454.CrossRefGoogle Scholar
9.Kim, SJ, Kim, WH, Lee, Y. Improving results of the Fontan procedure in patients with heterotaxy syndrome. Ann Thorac Surg 2006; 82: 1245–1251.Google Scholar
10.Van Praagh, S, Santini, F, Sanders, S, Flyler, DF. Cardiac malpositions: congenital heart disease in visceral heterotaxy (the asplenia and polysplenia syndromes). In: Fyler DC (ed.). Nadas’ Pediatric Cardiology. Hanley & Belfus, Philadelphia, 1992, pp 589–608.Google Scholar
11.Uemura, H, Ho, SY, Devine, WA, Kilpatrick, LL, Anderson, RH. Atrial appendages and venoatrial connections in hearts with patients with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561–569.CrossRefGoogle ScholarPubMed
12.Uemura, H, Ho, SY, Devine, WA, Anderson, RH. Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. Am J Cardiol 1995; 76: 846–849.Google Scholar
13.Jacobs, JP, Anderson, RH, Weinberg, PM, et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 2007; 17 (Suppl 2): 1–28.Google ScholarPubMed
14.Kay, JH, Egerton, W. The repair of mitral insufficiency associated with ruptured chordae tendinease. Ann Surg 1963; 157: 351.CrossRefGoogle Scholar
15.Rowlatt, UF, Rimoldi, HJ, Lev, M. The quantitative anatomy of the normal child’s heart. Pediatr Clin North Am 1963; 10: 499–588.CrossRefGoogle Scholar
16.Stamm, C, Friehs, I, Duebener, LF, et al. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 2002; 74: 1967–1977.Google Scholar
17.Moak, JP, Gersony, WM. Progressive atrioventricular valvular regurgitation in single ventricle. Am J Cardiol 1987; 59: 656–658.CrossRefGoogle ScholarPubMed
18.Imai, Y, Takanashi, Y, Hoshino, S, Terada, M, Aoki, M, Ohta, J. Modified Fontan procedure in ninety-nine cases of atrioventricular valve regurgitation. J Thorac Cardiovasc Surg 1997; 113: 262–269.Google Scholar