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Heterotaxy Syndrome: Defining Contemporary Disease Trends

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Abstract

The purpose of this study was to define a population of visceral heterotaxy and to investigate the incidence of bacterial sepsis in the current era of universal pediatric pneumococcal immunization. Pediatric echocardiography and radiology databases, along with electronic medical records, were searched for patients followed-up since birth between 1999 and 2009 with either asplenia or polysplenia and cardiac anatomy consistent with heterotaxy syndrome. A total of 29 patients were identified. Seven patients (24%) had a total of 8 sepsis events, and 6 patients (86%) developed sepsis while taking appropriately prescribed antibiotic prophylaxis. Of the patients with sepsis, 5 had polysplenia and 2 had asplenia. Sixty-two percent of sepsis events were nosocomially acquired. No cases of pneumococcal sepsis occurred after the introduction of the conjugated pneumococcal vaccination to the pediatric vaccination schedule. Bacterial sepsis was associated with a 44% mortality rate. An unexpected finding in 3 patients with visceral heterotaxy, asplenia, and an interrupted inferior vena cava (IVC) as the only anomaly on echocardiography was associated intestinal malrotation. Children with visceral heterotaxy remain at significant risk of life-threatening bacterial infection. In addition, the finding of interrupted IVC on echocardiography should prompt screening for intestinal malrotation, even in the absence of additional structural heart disease.

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References

  1. Abou Elella R, Najm HK, Balkhy H, Bullard L, Kabbani MS (2010) Impact of bloodstream infection on the outcome of children undergoing cardiac surgery. Pediatr Cardiol 31:483–489

    Article  PubMed  Google Scholar 

  2. Anagnostopoulos PV, Pearl JM, Octave C, Cohen M, Gruessner A, Wintering E et al (2009) Improved current era outcomes in patients with heterotaxy syndromes. Eur J Cardiothorac Surg 35:871–877

    Article  PubMed  Google Scholar 

  3. Azakie A, Merklinger SL, Williams WG, Van Arsdell GS, Coles JG, Adatia I (2001) Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann Thorac Surg 72:1636–16640

    Article  CAS  PubMed  Google Scholar 

  4. Barker GM, O’Brien SM, Welke KF, Jacobs ML, Benjamin DK Jr, Peterson ED et al (2010) Major infection after pediatric cardiac surgery: a risk estimation model. Ann Thorac Surg 89:843–850

    Article  PubMed  Google Scholar 

  5. Bartz PJ, Driscoll DJ, Dearani JA, Puga FJ, Danielson GK, O’Leary PW et al (2006) Early and late results of the modified Fontan operation for heterotaxy syndrome: 30 years of experience in 142 patients. J Am Coll Cardiol 48:2301–2305

    Article  PubMed  Google Scholar 

  6. Biggar WD, Ramirez RA, Rose V (1981) Congenital asplenia: immunologic assessment and a clinical review of eight surviving patients. Pediatrics 67:548–551

    CAS  PubMed  Google Scholar 

  7. Bone RC, Balk RA, Cerra FB, Dellinger RP, Fein AM, Knaus WA et al (1992) Definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis. The ACCP/SCCM Consensus Conference Committee. American College of Chest Physicians/Society of Critical Care Medicine. Chest 101:1644–1655

    Article  CAS  PubMed  Google Scholar 

  8. Bratzler DW, Houck PM (2005) Antimicrobial prophylaxis for surgery: an advisory statement from the National Surgical Infection Prevention Project. Am J Surg 189:395–404

    Article  PubMed  Google Scholar 

  9. Brendolan A, Rosado MM, Carsetti R, Selleri L, Dear TN (2007) Development and function of the mammalian spleen. Bioessays 29:166–177

    Article  CAS  PubMed  Google Scholar 

  10. Brueckner M (2007) Heterotaxia, congenital heart disease, and primary ciliary dyskinesia. Circulation 115:2793–2795

    Article  PubMed  Google Scholar 

  11. Cheung YF, Cheng VY, Chau AK, Chiu CS, Yung TC, Leung MP (2002) Outcome of infants with right atrial isomerism: is prognosis better with normal pulmonary venous drainage? Heart 87:146–152

    Article  CAS  PubMed  Google Scholar 

  12. Collignon PJ (1994) Intravascular catheter associated sepsis: a common problem. The Australian study in intravascular catheter associated sepsis. Med J Aust 161:374–378

    CAS  PubMed  Google Scholar 

  13. Ditchfield MR, Hutson JM (1998) Intestinal rotational abnormalities in polysplenia and asplenia syndromes. Pediatr Radiol 28:303–306

    Article  CAS  PubMed  Google Scholar 

  14. Dyke MP, Martin RP, Berry PJ (1991) Septicaemia and adrenal haemorrhage in congenital asplenia. Arch Dis Child 66:636–637

    Article  CAS  PubMed  Google Scholar 

  15. Gaston MH, Verter JI, Woods G, Pegelow C, Kelleher J, Presbury G et al (1986) Prophylaxis with oral penicillin in children with sickle cell anemia: a randomized trial. N Engl J Med 314:1593–1599

    Article  CAS  PubMed  Google Scholar 

  16. Grisaru-Soen G, Paret G, Yahav D, Boyko V, Lerner-Geva L (2009) Nosocomial infections in pediatric cardiovascular surgery patients: a 4-year survey. Pediatr Crit Care Med 10:202–206

    Article  PubMed  Google Scholar 

  17. Huang SS, Hinrichsen VL, Stevenson AE, Rifas-Shiman SL, Kleinman K, Pelton SI et al (2009) Continued impact of pneumococcal conjugate vaccine on carriage in young children. Pediatrics 124:e1–e11

    Article  PubMed  Google Scholar 

  18. Jacobs JP, Anderson RH, Weinberg PM, Walters HL 3rd, Tchervenkov CI, Del Duca D, et al (2007) The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 17(Suppl)2:1–28

    Google Scholar 

  19. Kim SJ, Kim WH, Lim HG, Lee CH, Lee JY (2006) Improving results of the Fontan procedure in patients with heterotaxy syndrome. Ann Thorac Surg 82:1245–1251

    Article  PubMed  Google Scholar 

  20. Maher KO, VanDerElzen K, Bove EL, Mosca RS, Chenoweth CE, Kulik TJ (2002) A retrospective review of three antibiotic prophylaxis regimens for pediatric cardiac surgical patients. Ann Thorac Surg 74:1195–1200

    Article  PubMed  Google Scholar 

  21. Melles DC, de Marie S (2004) Prevention of infections in hyposplenic and asplenic patients: an update. Neth J Med 62:45–52

    CAS  PubMed  Google Scholar 

  22. Mikoluc B, Kayhty H, Bernatowska E, Motowski R (2008) Immune response to the 7-valent pneumococcal conjugate vaccine in 30 asplenic children. Eur J Clin Microbiol Infect Dis 27:923–928

    Article  CAS  PubMed  Google Scholar 

  23. Nagel BH, Williams H, Stewart L, Paul J, Stumper O (2005) Splenic state in surviving patients with visceral heterotaxy. Cardiol Young 15:469–473

    Article  PubMed  Google Scholar 

  24. Overturf GD (2000) American Academy of Pediatrics. Committee on Infectious Diseases. Technical report: prevention of pneumococcal infections, including the use of pneumococcal conjugate and polysaccharide vaccines and antibiotic prophylaxis. Pediatrics 106:367–376

    Google Scholar 

  25. Pickering LK, Baker CJ, Kimberlin DW, Long SS (2009) Red Book: 2009 Report of the Committee on Infectious Diseases, 28th ed. p 84

  26. Price VE, Blanchette VS, Ford-Jones EL (2007) The prevention and management of infections in children with asplenia or hyposplenia. Infect Dis Clin North Am 21:697–710

    Article  PubMed  Google Scholar 

  27. Rose V, Izukawa T, Moes CA (1975) Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 37:840–852

    Article  CAS  PubMed  Google Scholar 

  28. Ruscazio M, Van Praagh S, Marrass AR, Catani G, Iliceto S, Van Praagh R (1998) Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol 81:111–1116

    Article  CAS  PubMed  Google Scholar 

  29. Schutze GE, Mason EO Jr, Barson WJ, Kim KS, Wald ER, Givner LB et al (2002) Invasive pneumococcal infections in children with asplenia. Pediatr Infect Dis J 21:278–282

    Article  PubMed  Google Scholar 

  30. Singer DB (1973) Postsplenectomy sepsis. Perspect Paediatr Pathol 1:285–311

    CAS  Google Scholar 

  31. Smets F, Bourgois A, Vermylen C, Brichard B, Slacmuylders P, Leyman S et al (2007) Randomized revaccination with pneumococcal polysaccharide or conjugate vaccine in asplenic children previously vaccinated with polysaccharide vaccine. Vaccine 25:5278–5282

    Article  CAS  PubMed  Google Scholar 

  32. Sox C (2003) Health supervision for children with sickle cell disease. Pediatrics 111:710–711

    Article  PubMed  Google Scholar 

  33. Spelman D, Buttery J, Daley A, Isaacs D, Jennens I, Kakakios A et al (2008) Guidelines for the prevention of sepsis in asplenic and hyposplenic patients. Intern Med J 38:349–356

    Article  CAS  PubMed  Google Scholar 

  34. Stamm C, Friehs I, Duebener LF, Zurakowski D, Mayer JE Jr, Jonas RA et al (2002) Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 74:1967–1977

    Article  PubMed  Google Scholar 

  35. Tashjian DB, Weeks B, Brueckner M, Touloukian RJ (2007) Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia. J Pediatr Surg 42:528–531

    Article  PubMed  Google Scholar 

  36. Ticho BS, Goldstein AM, Van Praagh R (2000) Extracardiac anomalies in the heterotaxy syndromes with focus on anomalies of midline-associated structures. Am J Cardiol 85:729–734

    Article  CAS  PubMed  Google Scholar 

  37. Vincent JL, Bihari DJ, Suter PM, Bruining HA, White J, Nicolas-Chanoin MH et al (1995) The prevalence of nosocomial infection in intensive care units in Europe. Results of the European prevalence of infection in intensive care (EPIC) study. EPIC international advisory committee. JAMA 274:639–644

    Article  CAS  PubMed  Google Scholar 

  38. Waldman JD, Rosenthal A, Smith AL, Shurin S, Nadas AS (1977) Sepsis and congenital asplenia. J Pediatr 90:555–559

    Article  CAS  PubMed  Google Scholar 

  39. Wu MH, Wang JK, Lue HC (2002) Sudden death in patients with right atrial isomerism (asplenism) after palliation. J Pediatr 140:93–96

    Article  PubMed  Google Scholar 

Download references

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The authors have no financial relationships relevant to this article to disclose or conflicts of interest to declare. The submission is with the full knowledge and approval of all listed coauthors.

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Correspondence to Terence W. Prendiville.

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Prendiville, T.W., Barton, L.L., Thompson, W.R. et al. Heterotaxy Syndrome: Defining Contemporary Disease Trends. Pediatr Cardiol 31, 1052–1058 (2010). https://doi.org/10.1007/s00246-010-9764-z

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