eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Heterotaxy, Polysplenia: Follow-up

Author: Kevin M Shannon, MD, Associate Professor, Division of Pediatric Cardiology, Director of Pediatric Electrophysiology Program, UCLA School of Medicine; Consulting Staff, Pediatric Cardiology Clinic, Olive View-UCLA Medical Center
Contributor Information and Disclosures

Updated: Apr 22, 2009

Follow-up

Further Inpatient Care

  • Follow-up care in polysplenia is dictated by the type of cardiac defect and/or associated noncardiac manifestations. Patients with univentricular physiology typically require staged palliation, repeated hospitalization for preoperative evaluation, and perioperative care. Other inpatient care may be dictated by associated noncardiac lesions.

Further Outpatient Care

  • Patients with polysplenia syndrome often have univentricular physiology and/or very complex anatomy requiring staged palliative procedures and lifelong cardiac follow-up.
  • Patients with functional asplenia require careful evaluation of fever. They are at risk for overwhelming sepsis. Fevers without obvious cause should be evaluated with a blood culture, and admission for intravenous antibiotics, until the culture results are available, should be considered.

Inpatient & Outpatient Medications

  • Anticongestive therapy is required in most patients, along with anticoagulation and vasodilator therapy. No consensus exists on the most appropriate medical regimen for these patients, and the broad range of manifestations makes studies regarding the safety and efficacy of drug regimens difficult.
  • Many patients with palliated polysplenia syndrome develop arrhythmia, requiring medication. Consensus on the most appropriate antiarrhythmic medications or interventional therapy also is lacking.
  • Patients with functional asplenia require antibiotic prophylaxis. The usual regimen is amoxicillin once daily.

Complications

  • Complications from polysplenia syndrome can arise from the cardiac defects, the intestinal defects, or the immunodeficiency. The most common cardiac complications are congestive heart failure and arrhythmia. Careful prospective management of pulmonary blood flow, atrioventricular valve regurgitation, and cyanosis can help prevent the early onset of ventricular dysfunction and congestive heart failure; however, this complication is nearly universal in these patients. Arrhythmia can result from an absent or poorly functioning sinus node or from intra-atrial reentry around surgical scars. Patients with a history of pulmonary vein repair appear to be particularly prone to this complication. Antiarrhythmic therapy and pacemaker implantation often are required in these cases.
  • GI complications usually arise from malrotation with volvulus but can also result from annular pancreas, esophageal or intestinal atresia, and biliary tree abnormalities.
  • Complications arising from functional asplenia can include overwhelming sepsis, often leading to loss of extremities or death.

Prognosis

  • The prognosis for polysplenia syndrome is closely related to the cardiac manifestations. In a 26-year study period, the overall survival rate in the large series of 91 patients from Toronto was 31%; the 5-year survival rate was less than 40%. Risk factors for poor outcome included unobstructed pulmonary blood flow, obstructed pulmonary venous return, and major AV valve anomalies.

Patient Education

  • Patient education usually starts with educating parents on the care of infants with heart disease. The importance of routine follow-up, adherence to the medical regimen, and risk of infection should be stressed. In addition, early discussions about the relatively poor prognosis, potential need for orthotopic heart transplant, and need for multiple palliative procedures often help parents to cope with this chronic condition.

Miscellaneous

Medicolegal Pitfalls

  • The most common medicolegal pitfall in this patients with polysplenia syndrome seems to be failure to recognize sepsis in a patient with polysplenia who is functionally asplenic. Rapidly fatal infections are common in asplenic patients and often occur shortly after the onset of fever in an otherwise healthy appearing child. The lack of an ill appearance in these children should not be reassuring. All asplenic patients should be evaluated carefully, and inpatient observation with prophylactic antibiotics should be considered.

Special Concerns

  • Patients who survive to childbearing age are generally discouraged from carrying a pregnancy to term because the likelihood of carrying a pregnancy to term is low, and the maternal risk is high. However, patients with polysplenia have survived pregnancies and delivered term healthy babies without congenital heart disease.
 


More on Heterotaxy, Polysplenia

Overview: Heterotaxy, Polysplenia
Differential Diagnoses & Workup: Heterotaxy, Polysplenia
Treatment & Medication: Heterotaxy, Polysplenia
Follow-up: Heterotaxy, Polysplenia
Multimedia: Heterotaxy, Polysplenia
References
Further Reading
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References

  1. Bartram U, Fischer G, Kramer HH. Congenitally interrupted inferior vena cava without other features of the heterotaxy syndrome: report of five cases and characterization of a rare entity. Pediatr Dev Pathol. Jul-Aug 2008;11(4):266-73. [Medline].

  2. Kapa S, Gleeson FC, Vege SS. Dorsal pancreas agenesis and polysplenia/heterotaxy syndrome: a novel association with aortic coarctation and a review of the literature. JOP. Jul 9 2007;8(4):433-7. [Medline].

  3. Ferdman B, States L, Gaynor JW, Hedrick HL, Rychik J. Abnormalities of intestinal rotation in patients with congenital heart disease and the heterotaxy syndrome. Congenit Heart Dis. Jan 2007;2(1):12-8. [Medline].

  4. Halac M, Mut SS, Ylmaz S, Ergul N, Sonmezoglu K. Appearance of situs inversus totalis and polysplenia syndrome on FDG PET/CT. Clin Nucl Med. Feb 2008;33(2):142-3. [Medline].

  5. Cohen MS, Anderson RH, Cohen MI, Atz AM, Fogel M, Gruber PJ, et al. Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. Sep 2007;17 Suppl 2:29-43. [Medline].

  6. Atkinson DE, Drant S. Diagnosis of heterotaxy syndrome by fetal echocardiography. Am J Cardiol. Nov 1 1998;82(9):1147-9, A10. [Medline].

  7. Bartram U, Wirbelauer J, Speer CP. Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 2005;88(4):278-90. [Medline].

  8. Belmont JW, Mohapatra B, Towbin JA, Ware SM. Molecular genetics of heterotaxy syndromes. Curr Opin Cardiol. May 2004;19(3):216-20. [Medline]. [Full Text].

  9. Berg C, Geipel A, Kamil D, et al. The syndrome of left isomerism: sonographic findings and outcome in prenatally diagnosed cases. J Ultrasound Med. Jul 2005;24(7):921-31. [Medline]. [Full Text].

  10. Chen SJ, Li YW, Wang JK, et al. Usefulness of electron beam computed tomography in children with heterotaxy syndrome. Am J Cardiol. Jan 15 1998;81(2):188-94. [Medline].

  11. Ditchfield MR, Hutson JM. Intestinal rotational abnormalities in polysplenia and asplenia syndromes. Pediatr Radiol. May 1998;28(5):303-6. [Medline].

  12. Gayer G, Apter S, Jonas T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. Mar-Apr 1999;24(2):178-84. [Medline].

  13. Gilljam T, McCrindle BW, Smallhorn JF, et al. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol. Sep 2000;36(3):908-16. [Medline].

  14. Hofstaetter C, Plath H, Hansmann M. Prenatal diagnosis of abnormalities of the fetal venous system. Ultrasound Obstet Gynecol. Mar 2000;15(3):231-41. [Medline].

  15. Ticho BS, Van Praagh R. Inherited structural heart diseases associated with arrhythmias: Defects in laterality. In: Berul CI, Towbin JA, eds. Molecular Genetics of Cardiac Electrophysiology. Boston, MA: Kluwer Academic Publishers; 2000:317-28.

  16. Uemura H, Ho SY, Anderson RH, Yagihara T. Ventricular morphology and coronary arterial anatomy in hearts with isometric atrial appendages. Ann Thorac Surg. May 1999;67(5):1403-11. [Medline].

  17. Uemura H, Ho SY, Devine WA, Anderson RH. Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. Am J Cardiol. Oct 15 1995;76(11):846-9. [Medline].

  18. Yoo SJ, Kim YM, Choe YH. Magnetic resonance imaging of complex congenital heart disease. Int J Card Imaging. Apr 1999;15(2):151-60. [Medline].

  19. Zissin R, Rathaus V, Oscadchy A, et al. Intestinal malrotation as an incidental finding on CT in adults. Abdom Imaging. Nov-Dec 1999;24(6):550-5. [Medline].

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Keywords

polysplenia, heterotaxy syndrome, left atrial isomerism, polysplenia syndrome, cyanotic congenital heart disease, biliary atresia, intestinal malrotation, functional asplenia, jaundice, congenital heart disease, sepsis, dextrocardia, treatment, diagnosis

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Contributor Information and Disclosures

Author

Kevin M Shannon, MD, Associate Professor, Division of Pediatric Cardiology, Director of Pediatric Electrophysiology Program, UCLA School of Medicine; Consulting Staff, Pediatric Cardiology Clinic, Olive View-UCLA Medical Center
Kevin M Shannon, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Heart Rhythm Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Julian M Stewart, MD, PhD, Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College
Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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