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Functional state of patients with heterotaxy syndrome following the Fontan operation

Published online by Cambridge University Press:  26 November 2007

Andrew M. Atz*
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America
Meryl S. Cohen
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Lynn A. Sleeper
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Brian W. McCrindle
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Canada
Minmin Lu
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Ashwin Prakash
Affiliation:
Department of Pediatrics, Division of Cardiology, Columbia University Medical Center, New York, New York, United States of America
Roger E. Breitbart
Affiliation:
Department of Cardiology, Children’s Hospital Boston, Boston, Massachusetts, United States of America
Richard V. Williams
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Utah, Salt Lake City, Utah, United States of America
Charlie J. Sang
Affiliation:
Department of Pediatrics, Division of Cardiology, Duke University Medical Center, Durham, North Carolina, United States of America
Gil Wernovsky
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: Andrew M Atz, Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, Box 250915, Room 601 Children’s Hospital, Charleston, South Carolina 29425, USA. Tel: 843 792 3292; Fax: 843 792 3284; E-mail: atzam@musc.edu

Abstract

Background

Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation.

Methods

In the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy.

Results

Patients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p = 0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p = 0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p = 0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p = 0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy.

Conclusions

The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

Type
Original Article
Copyright
Copyright © Cambridge University Press 2007

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References

1. Fontan, F, Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240248.CrossRefGoogle ScholarPubMed
2. Bartz, PJ, Driscoll, DJ, Dearani, JA, et al. . Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol 2006; 48: 23012305.CrossRefGoogle ScholarPubMed
3. Sleeper, LA, Anderson, P, Hsu, DT, et al. . Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation. Am Heart J 2006; 152: 427433.CrossRefGoogle ScholarPubMed
4. Mahony, L, Sleeper, LA, Anderson, PA, et al. . The Pediatric Heart Network: a primer for the conduct of multicenter studies in children with congenital and acquired heart disease. Pediatr Cardiol 2006; 27: 191198.Google Scholar
5. Jacobs, ML, JrMayer, JE. Congenital Heart Surgery Nomenclature and Database Project: single ventricle. Ann Thorac Surg 2000; 69: S197S204.CrossRefGoogle ScholarPubMed
6. Landgraf, JM. Measuring health-related quality of life in pediatric oncology patients: a brief commentary on the state of the art of measurement and application (discussion). Int J Cancer Suppl 1999; 12: 147150.3.0.CO;2-O>CrossRefGoogle ScholarPubMed
7. Azakie, A, Merklinger, SL, Williams, WG, Van Arsdell, GS, Coles, JG, Adatia, I. Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann Thorac Surg 2001; 72: 16361640.CrossRefGoogle ScholarPubMed
8. Van Praagh, S. Cardiac malpositions and the heterotaxy syndromes. In: Keane, JF, Lock, JE, Fyler, DC (eds). Elsevier Publishers, Philadelphia, PA, Nadas’ Pediatric Cardiology, 2006, pp 589608.Google Scholar
9. Mahle, WT, Cohen, MS, Spray, TL, Rychik, J. Atrioventricular valve regurgitation in patients with single ventricle: impact of the bidirectional cavopulmonary anastomosis. Ann Thorac Surg 2001; 72: 831835.CrossRefGoogle ScholarPubMed
10. Smith, A, Ho, SY, Anderson, RH, et al. . The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialised conduction system. Cardiol Young 2006; 16: 437454.CrossRefGoogle Scholar
11. Wu, MH, Wang, JK, Lin, JL, et al. . Supraventricular tachycardia in patients with right atrial isomerism. J Am Coll Cardiol 1998; 32: 773779.CrossRefGoogle ScholarPubMed
12. Kim, SJ, Kim, WH, Lim, HG, Lee, CH, Lee, JY. Improving results of the Fontan procedure in patients with heterotaxy syndrome. Ann Thorac Surg 2006; 82: 12451251.CrossRefGoogle ScholarPubMed
13. McCrindle, BW, Williams, RV, Mitchell, PD, et al. . Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation 2006; 113: 11231129.CrossRefGoogle ScholarPubMed
14. 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: 908916.CrossRefGoogle Scholar
15. Hashmi, A, bu-Sulaiman, R, McCrindle, BW, Smallhorn, JF, Williams, WG, Freedom, RM. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998; 31: 11201126.CrossRefGoogle ScholarPubMed
16. Gentles, TL, Gauvreau, K, JrMayer, JE, et al. . Functional outcome after the Fontan operation: factors influencing late morbidity. J Thorac Cardiovasc Surg 1997; 114: 392403.Google Scholar
17. 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: 19671977.CrossRefGoogle ScholarPubMed
18. Nishimura, RA, Tajik, AJ. Evaluation of diastolic filling of left ventricle in health and disease: Doppler echocardiography is the clinician’s Rosetta Stone. J Am Coll Cardiol 1997; 30 (1): 818.CrossRefGoogle ScholarPubMed