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Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia*

Published online by Cambridge University Press:  16 December 2008

Ahmed Farouk ,
Kenneth Zahka ,
Ernest Siwik ,
Francine Erenberg ,
Yasser Al-Khatib ,
Alex Golden ,
Mohsen Karimi ,
Minhaz Uddin  and
Hani A. Hennein
Ahmed Farouk
Affiliation:
Department of Pediatric Cardiothoracic Surgery, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Kenneth Zahka
Affiliation:
Department of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Ernest Siwik
Affiliation:
Department of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Francine Erenberg
Affiliation:
Department of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Yasser Al-Khatib
Affiliation:
Department of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Alex Golden
Affiliation:
Department of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Mohsen Karimi
Affiliation:
Department of Pediatric Cardiothoracic Surgery, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Minhaz Uddin
Affiliation:
Department of Pediatric Cardiothoracic Surgery, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
Hani A. Hennein*
Affiliation:
Department of Pediatric Cardiothoracic Surgery, Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, Ohio, United States of America
*
Correspondence to: Hani A. Hennein, MD, Chief, Pediatric Cardiothoracic Surgery, Rainbow Babies and Children’s Hospital, Associate Professor, Case Western Reserve University, 11100 Euclid Avenue, Suite 380, Cleveland, Ohio 44106, United States of America. Tel: 216-844-3058; Fax: 216-844-3517; E-mail: [email protected]
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Abstract

Background

Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the lungs on the native intrapericardial pulmonary arterial circulation whenever possible. We describe an individualized approach to this group of patients that optimizes these variables.

Methods

Over a consecutive 10-year period, we treated 66 patients presenting with tetralogy of Fallot and pulmonary atresia according to the source of the pulmonary arterial flow. Patients were grouped according to whether the flow of blood to the lungs was derived exclusively from the intrapericardial pulmonary arteries, as seen in 29 patients, exclusively from systemic-to-pulmonary collateral arteries, as in 5 patients, or from both the intrapericardial pulmonary and collateral arteries, as in the remaining 32 patients. We divided the latter group into 9 patients deemed simple, and 23 considered complex, according to whether the pulmonary arterial index was greater than or less than 90 millimetres squared per metre squared, and whether the number of collateral arteries was less than or greater than 2, respectively.

Results

We achieved complete biventricular repair in 58 patients (88%), with an overall mortality of 3%. Repair was accomplished in a single stage in all patients without systemic-to-pulmonary collateral arteries, but was staged, with unifocalization, in the patients lacking intrapericardial pulmonary arteries. Complete repair without unifocalization was achieved in all patients with the simple variant of the mixed morphology, and in 56% of patients with the complex variant. The average number of procedures per patient to achieve complete repair was 1, 2.2, 3.8, and 2.6 in patients with exclusively native intrapericardial, simple and mixed, complex and mixed and exclusively collateral pulmonary arterial flow, respectively.

Conclusions

An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.

Keywords

Systemic-to-pulmonary collateral arteriesunifocalizationpulmonary arteriespulmonary atresia with ventricular septal defectpulmonary arterial index
Type
Original Article
Information
Cardiology in the Young , Volume 19 , Issue 1 , February 2009 , pp. 76 - 85
Copyright
Copyright © Cambridge University Press 2008

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Footnotes

*

The presentation on which this work is based was given at the Inaugural Meeting of the World Society for Pediatric and Congenital Heart Surgery, held in Washington, District of Columbia, May 3 and 4, 2007.

References

1. Tchervenkov, CI, Roy, N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg 2000; 69: S97–105.Google Scholar
2. Nakata, S, Imai, Y, Takanashi, Y, et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984; 88: 610–619.CrossRefGoogle ScholarPubMed
3. Reddy, VM, Liddicoat, JR, Hanley, FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1995; 109: 832–844; discussion 844–845.CrossRefGoogle ScholarPubMed
4. Castaneda, AR Jr, Mayer, JE Jr, Hanley, FL. Cardiac surgery of the neonate and infant. WB Saunders Co, Philadelphia, 1994.Google Scholar
5. Hennein, HA, Mosca, RS, Urcelay, G, Crowley, DC, Bove, EL. Intermediate results after complete repair of tetralogy of Fallot in neonates. J Thorac Cardiovasc Surg 1995; 109: 332–342, 344; discussion 342–343.Google Scholar
6. Duncan, BW, Mee, RB, Prieto, LR, et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003; 126: 694–702.Google Scholar
7. Reddy, VM, McElhinney, DB, Amin, Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation 2000; 101: 1826–1832.Google Scholar
8. Carotti, A, Albanese, SB, Minniti, G, Guccione, P, Di Donato, RM. Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Eur J Cardiothorac Surg 2003; 23: 719–726; discussion 726–727.CrossRefGoogle ScholarPubMed
9. Somerville, J. Management of pulmonary atresia. Br Heart J 1970; 32: 641–651.CrossRefGoogle ScholarPubMed
10. Reddy, VM, Petrossian, E, McElhinney, DB, Moore, P, Teitel, DF, Hanley, FL. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed? J Thorac Cardiovasc Surg 1997; 113: 858–866; discussion 866–868.Google Scholar
11. Tchervenkov, CI, Salasidis, G, Cecere, R, et al. One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals. J Thorac Cardiovasc Surg 1997; 114: 727–735; discussion 735–737.Google Scholar
12. Abella, RF, De La Torre, T, Mastropietro, G, Morici, N, Cipriani, A, Marcelletti, C. Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach. J Thorac Cardiovasc Surg 2004; 127: 193–202.Google Scholar
13. Gupta, A, Odim, J, Levi, D, Chang, RK, Laks, H. Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 104 patients. J Thorac Cardiovasc Surg 2003; 126: 1746–1752.Google Scholar
14. Cho, JM, Puga, FJ, Danielson, GK, et al. Early and long-term results of the surgical treatment of tetralogy of Fallot with pulmonary atresia, with or without major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2002; 124: 70–81.Google Scholar
15. Pagani, FD, Cheatham, JP, 3rdBeekman, RH, Lloyd, TR, Mosca, RS, Bove, EL. The management of tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries. J Thorac Cardiovasc Surg 1995; 110: 1521–1532; discussion 1532–1533.Google Scholar
16. Yagihara, T, Yamamoto, F, Nishigaki, K, et al. Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 1996; 112: 392–402.CrossRefGoogle ScholarPubMed
17. Metras, D, Chetaille, P, Kreitmann, B, Fraisse, A, Ghez, O, Riberi, A. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg 2001; 20: 590–596; discussion 596–597.CrossRefGoogle ScholarPubMed
18. d’Udekem, Y, Alphonso, N, Norgaard, MA, et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits. J Thorac Cardiovasc Surg 2005; 130: 1496–1502.Google Scholar
19. Amark, KM, Karamlou, T, O’Carroll, A, et al. Independent factors associated with mortality, reintervention, and achievement of complete repair in children with pulmonary atresia with ventricular septal defect. J Am Coll Cardiol 2006; 47: 1448–1456.Google Scholar
20. Norgaard, MA, Alphonso, N, Cochrane, AD, Menahem, S, Brizard, CP, d’Udekem, Y. Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries. Eur J Cardiothorac Surg 2006; 29: 653–658.CrossRefGoogle ScholarPubMed
21. Iyer, KS, Mee, RB. Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals. Ann Thorac Surg 1991; 51: 65–72.Google Scholar
22. Mee, RB. Presentation and attrition in complex pulmonary atresia. J Am Coll Cardiol 1996; 28: 539–540.CrossRefGoogle ScholarPubMed