Hostname: page-component-78c5997874-xbtfd Total loading time: 0 Render date: 2024-11-05T03:21:34.415Z Has data issue: false hasContentIssue false

Surgery for the functionally univentricular heart in patients with visceral heterotaxy

Published online by Cambridge University Press:  10 January 2006

Christo I. Tchervenkov
Affiliation:
Division of Cardiovascular Surgery, Montreal Children's Hospital of the McGill University Health Center, McGill University, Montréal, Québec, Canada
Marshall L. Jacobs
Affiliation:
Section of Pediatric Cardiac Surgery, St Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, Pennsylvania, United States of America
Danny Del Duca
Affiliation:
Division of Cardiovascular Surgery, Montreal Children's Hospital of the McGill University Health Center, McGill University, Montréal, Québec, Canada

Extract

The heterotaxy syndromes are characterized by a high incidence of cardiac anomalies of extreme heterogeneity. Due to this, the surgical management is varied and challenging. Although a minority of patients can undergo biventricular repair, the complexity of the cardiac defects, and the high incidence of ventricular hypoplasia, mandate a surgical approach in the majority of patients depending on the creation of a functionally univentricular heart. Traditionally, the functionally univentricular approach was associated with a high mortality. More recently, the results have been improving as a result of better understanding of these malformations, and logical improvements in surgical technique. In this review, we will make brief comments on the nomenclature and classification of the heterotaxy syndromes, and the range of cardiac anomalies, before concentrating on the surgical treatment for those patients having functionally univentricular hearts in the setting of heterotaxy. More specifically, we will review initial palliation in early life, creation of the cavopulmonary anastomoses, including the Kawashima procedure and the Fontan circulation, and the role of transplantation.

Type
Research Article
Copyright
© 2006 Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Macartney FJ, Zuberbuhler JR, Anderson RH. Morphological considerations pertaining to recognition of atrial isomerism. Br Heart J 1980; 44: 657667.Google Scholar
Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995; 60: 561569.Google Scholar
Sharma S, Devine W, Anderson RH, Zuberbuhler JR. The determination of atrial arrangement by examination of appendage morphology in 1842 heart specimens. Br Heart J 1988; 60: 227231.Google Scholar
Van Praagh R, Van Praagh S. Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia, or normally formed spleen: an erroneous concept. Am J Cardiol 1990; 66: 15041506.Google Scholar
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: 846849.Google Scholar
Van Praagh S, Santini F, Sanders SP. Cardiac malpositions with special emphasis on visceral heterotaxy (asplenia and polysplenia syndromes). In: Fyler DC (ed.). Nadas' Pediatric Cardiology. Hanley & Belfus, Philadelphia, PA, 1992, pp 589608.
Smith A, Ho SY, Anderson RH, et al. The disposition of the specialised conduction system in relation to the diverse cardiac morphology seen in hearts with isomerism of the atrial appendages. Cardiol Young 2006; in press.Google Scholar
Hashmi A, Abu-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.Google Scholar
Pacifico AD, Ricchi A, Bargeron Jr LM, Colvin EC, Kirklin JW, Kirklin JK. Corrective repair of complete atrioventricular canal defects and major associated cardiac anomalies. Ann Thorac Surg 1988; 46: 645651.Google Scholar
Tchervenkov CI, Korkola SJ, Béland MJ. Single-stage anatomical repair of complete atrioventricular canal, double-outlet right ventricle, and cortriatriatum using ventricular septal defect translocation. Ann Thorac Surg 2002; 73: 13171320.Google Scholar
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.Google Scholar
Hirooka K, Yagihara T, Kishimoto H, et al. Biventricular repair in cardiac isomerism. Report of seventeen cases. J Thorac Cardiovasc Surg 1995; 109: 530535.Google Scholar
Sinzobahamvya N, Arenz C, Brecher AM, Urban AE. Atrial isomerism: a surgical experience. Cariovasc Surg 1999; 7: 436442.Google Scholar
Stellin G, Vida VL, Milanesi O, et al. Surgical treatment of complex cardiac anomalies: the “one and one half ventricle repair”. Eur J Cardiothorac Surg 2002; 22: 10431049.Google Scholar
Jacobs ML, Rychik J, Murphy JD, Nicolson SC, Steven JM, Norwood WI. Results of Norwood's operation for lesions other than hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1995; 110: 15551561.Google Scholar
Heinemann MK, Hanley FL, Van Praagh S, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. Ann Thorac Surg 1994; 57: 8891.Google Scholar
Sadiq M, Stumper O, De Giovanni JV, et al. Management and outcome of infants and children with right atrial isomerism. Heart 1996; 75: 314319.Google Scholar
Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1999; 117: 506513.Google Scholar
Alejos JC, Williams RG, Jarmakani JM, et al. Factors influencing survival in patients undergoing the bidirectional Glenn anastomosis. Am J Cardiol 1995; 75: 10481050.Google Scholar
McElhinney DB, Reddy VM, Moore P, Hanley FL. Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage. Ann Thorac Surg 1997; 63: 16761684.Google Scholar
Kawashima Y, Kitamura S, Matsuda H, Shimazaki Y, Nakano S, Hirose H. Total cavopulmonary shunt operation in complex cardiac anomalies. A new operation. J Thorac Cardiovasc Surg 1984; 87: 7481.Google Scholar
Knight WB, Mee RB. A cure for pulmonary arteriovenous fistulas? Ann Thorac Surg 1995; 59: 9991001.Google Scholar
Shah MJ, Rychik J, Fogel MA, Murphy JD, Jacobs ML. Pulmonary AV malformations after superior cavopulmonary connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg 1997; 63: 960963.Google Scholar
Uemura H, Yagihara T, Hattori R, Kawahira Y, Tsukano S, Watanabe K. Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism. Ann Thorac Surg 1999; 68: 17311735.Google Scholar
Cetta F, Feldt RH, O'Leary PW, et al. Improved early morbidity and mortality after Fontan operation: the Mayo Clinic experience, 1987 to 1992. J Am Coll Cardiol 1996; 28: 480486.Google Scholar
Gentles TL, Mayer Jr JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg 1997; 114: 376391.Google Scholar
Mayer Jr JE, Bridges ND, Lock JE, Hanley FL, Jonas RA, Castaneda AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle. J Thorac Cardiovasc Surg 1992; 103: 444452.Google Scholar
Bridges ND, Mayer Jr JE, Lock JE, et al. Effect of baffle fenestration on outcome of the modified Fontan operation. Circulation 1992; 86: 17621769.Google Scholar
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.Google Scholar
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.Google Scholar
Jacobs ML. Complications associated with heterotaxy syndrome in Fontan patients. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 2535.Google Scholar
Larsen RL, Eguchi JH, Mulla NF, et al. Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy. Am J Cardiol 2002; 89: 12751279.Google Scholar