Hostname: page-component-848d4c4894-2pzkn Total loading time: 0 Render date: 2024-06-03T06:04:51.169Z Has data issue: false hasContentIssue false
  • English
  • Français

Prognosis in asplenia syndrome—a population-based review

Published online by Cambridge University Press:  19 August 2008

Steven A. Webber ,
George G.S. Sandor ,
Michael W.H. Patterson ,
Glenn P. Taylor ,
Louis D. Wadsworth  and
Jacques G. LeBlanc
Steven A. Webber*
Affiliation:
From the Division of Cardiology, Department of Pediatrics, the Department of Cardiothoracic Surgery and the Department of Pathology, University of British ColumbiaBritish Columbia's Children's Hospital, Vancouver
George G.S. Sandor
Affiliation:
From the Division of Cardiology, Department of Pediatrics, the Department of Cardiothoracic Surgery and the Department of Pathology, University of British ColumbiaBritish Columbia's Children's Hospital, Vancouver
Michael W.H. Patterson
Affiliation:
From the Division of Cardiology, Department of Pediatrics, the Department of Cardiothoracic Surgery and the Department of Pathology, University of British ColumbiaBritish Columbia's Children's Hospital, Vancouver
Glenn P. Taylor
Affiliation:
From the Division of Cardiology, Department of Pediatrics, the Department of Cardiothoracic Surgery and the Department of Pathology, University of British ColumbiaBritish Columbia's Children's Hospital, Vancouver
Louis D. Wadsworth
Affiliation:
From the Division of Cardiology, Department of Pediatrics, the Department of Cardiothoracic Surgery and the Department of Pathology, University of British ColumbiaBritish Columbia's Children's Hospital, Vancouver
Jacques G. LeBlanc
Affiliation:
From the Division of Cardiology, Department of Pediatrics, the Department of Cardiothoracic Surgery and the Department of Pathology, University of British ColumbiaBritish Columbia's Children's Hospital, Vancouver
*
Division of Pediatric Cardiology, Children's Hospital of Pittsburgh3705 Fifth Avenue at DeSoto Street, Pittsburgh, PA 152 13-2583, USA. Tel. 412-692-5540; Fax. 412-692-5138.
Get access Rights & Permissions [Opens in a new window]

Summary

The prognosis for the fetus or neonate with asplenia syndrome is unknown as population-based data are unavailable. Factors influencing survival in this syndrome were, therefore, assessed for the province of British Columbia by retrospective review of all cases diagnosed between 1970 and 1990. Particular attention was paid to the prognostic influence of palliative surgery, sepsis, extracardiac malformations, gastrointestinal disease, arrhythmias and sudden death. We identified 43 cases of asplenia syndrome, of which 40 were live-born (incidence 1/22,000 live‐births). Only one spontaneous death was noted during fetal life amongst approximately 5,000 fetal autopsies. Five cases had been diagnosed by fetal echocardiography, two of these pregnancies being terminated. Of the live-born infants, 28 (70%) underwent active management of their cardiac anomalies and of these, 14 are alive. Since 1980, 26 systemic-to‐pulmonary shunts have been constructed in 17 children with two operative deaths. Three children have had successful Fontan or bidirectional Glenn procedures. Systemic sepsis occurred in three children with no deaths. Three neonates underwent surgery for obstruction of the upper gastrointestinal tract without complication. Late sudden death occurred in four children with systemic-to-pulmonary shunts, none of whom had documented arrhythmias. Major extracardiac malformations were present in nine cases (21%), these contributing to death in two. Overall survival for all live-born infants is 35%. Sepsis and palliative surgery are no longer the major causes of death in asplenia syndrome, the current challenges being prevention of sudden death and the complexity of surgical repair of the cardiac malformations.

Keywords

Aspleniaisomerism of right atrial appendagesprognosis
Type
Original Articles
Information
Cardiology in the Young , Volume 2 , Issue 2 , April 1992 , pp. 129 - 135
Copyright
Copyright © Cambridge University Press 1992

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

1.Ivemark, BI. Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood. An analysis of the heart malformations in the splenic agenesis syndrome, with 14 new cases. Acta Paediatr Scand 1956; 44(Suppl 104): 1–110.Google Scholar
2.Ruttenberg, HD, Neufeld, HN, Lucas, RV, Carey, LS, Adams, P JrAnderson, RC, Edwards, JE. Syndrome ofcongenital cardiac disease with asplenia. Distinction from other forms of con genital cyanotic cardiac disease. Am J Cardiol 1964; 13: 387–406.Google Scholar
3.Anderson, C, Devine, WA, Anderson, RH, Debich, DE, Zuberbuhler, JR. Abnormalities of the spleen in relation to congenital malformations of the heart: a survey of necropsy findings in children. Br Heart J 1990; 63: 122–128.Google Scholar
4.Ho, SY, Cook, A, Anderson, RH, Allan, LD, Fagg, N. Isomerism of the atrial appendages in the fetus. Pediatr Pathol 1991; 11: 589–608.Google Scholar
5.Huhta, JC, Smallhorn, JF, Macartney, FJ. Two dimensional echocardiographic diagnosis of situs. Br Heart J 1982; 48: 97–108.Google Scholar
6.Putschar, WGJ, Manion, WC. Congenital absence of the spleen and associated anomalies. Am J Clin Pathol 1956; 26: 429–470.CrossRefGoogle ScholarPubMed
7.DeLeon, SY, Gidding, SS, Ilbawi, MN, Idriss, FS, Muster, AJ, Cole, RB, Paul, MH. Surgical management of infants with complex cardiac anomalies associated with reduced pulmonary blood flow and total anomalous pulmonary venous drainage. Ann Thorac Surg 1987; 43: 207–211.Google Scholar
8.Donato, Di R, Carlo, di D, Squitieri, C, Rossi, E, Ammirati, A, Marino, B, Marcelletti, C. Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in fancy. Ann Thorac Surg 1987; 44: 35–39.Google Scholar
9.Humes, 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
10.Rose, V, Izukawa, T, Moes, CAF. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malforma tions in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975; 37: 840–852.Google Scholar
11.Sapire, DW, Ho, SY, Anderson, RH, Rigby, ML. Diagnosis and significance of atrial isomerism. Am J Cardiol 1986; 58: 342–346.CrossRefGoogle ScholarPubMed
12.Chiu, IS, How, SW, Wang, JK, Wu, MH, Chu, SH, Lue, HC, Hung, CR. Clinical implications of atrial isomerism. Br Heart J 1988; 60: 72–77.CrossRefGoogle ScholarPubMed
13.Mierop, Van Mierop, Gessner, IH, Schiebler, GL. Asplenia and polysplenia syndromes. Birth Defects: Original Article Series 1972; 8: 36–44.Google Scholar
14.Macartney, FJ, Zuberbuhier, JR, Anderson, RH. Morphologi cal considerations pertaining to recognition of atrial isomer ism. Consequences for sequential chamber localization. Br Heart J 1980; 44: 657–667.Google Scholar
15.Waldman, JD, Rosenthal, A, Smith, AL, Shurin, S, Nadas, AS. Sepsis and congenital asplenia. J Pediatr 1977; 90: 555–559.CrossRefGoogle ScholarPubMed
16.Burn, J, Coffey, R, Allan, LD, Robinson, P, Pembrey, ME, Macartney, FJ. Isomerism: a genetic analysis. In: Doyle, EF, Engle, MA, Gersony, WM, Rashkind, WJ, Talner, NS (eds). Pediatric Cardiology; Proceedings of the Second World Con gress. Springer-Verlag, New York, 1986, pp 1126–1128.Google Scholar
17.Biggar, WD, RamIrez, RA, Rose, V. Congenital asplenia: Im munologic assessment and a clinical review of eight surviving patients. Pediatrics 1981; 67: 548–551.CrossRefGoogle Scholar
18.Webber, SA, Sandor, GGS, Patterson, MWH, Mitchell, LA, Scheifele, D, Ochnio, JJ. Short term immunogenicity of Haemophilus influenzae type B PRP-D conjugate vaccine in congenital asplenia. Presented to Western Society for Pediat ric Research, February 1991. [Abstruct]Google Scholar
19.Lamberti, JJ, Waidman, JD, Mathewson, JW, Kirkpatrick, SE. Repair of subdiaphragmatic total anomalous pulmonary ve nous connection without cardiopulmonary bypass. J Thorac Cardiovasc Surg 1984; 88: 627–630.Google Scholar
20.Wolfe, MW, Vacek, JL, Kinard, RE, Bailey, CG. Prolonged and functional survival with the asplenia syndrome. Am J Med 1986; 81: 1089–1091.CrossRefGoogle ScholarPubMed
21.Gutgesell, HP. Cardiac malposition and heterotaxy. In: Garson, A, Bricker, JT, McNemara, DC (eds). The Science and Practice of Pediatric Cardiology. Lea & Febiger, Philadelphia, 1990, p 1295.Google Scholar
22.Bridges, ND, Lock, JE, Castafleda, AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation 1990; 82: 1681–1689.Google Scholar
23.Bridges, ND, Jonas, RA, Mayer, JE, Flanagan, MF, Keane, JF, Castafleda, AR. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates: Early re sults. Circulation 1990; 82(Suppl IV): 1V170–1V176.Google Scholar