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

Heterotaxy, Asplenia: Differential Diagnoses & Workup

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 23, 2009

Differential Diagnoses

Acidosis, Metabolic
Dextrocardia
Heterotaxy, Polysplenia
Transposition of the Great Arteries

Other Problems to Be Considered

Kartagener syndrome

Workup

Laboratory Studies

  • Useful laboratory studies in asplenia include a CBC count with peripheral smear to assess for Howell-Jolly bodies and evidence of impaired splenic function.
  • ABG to assess for cyanotic heart disease may also be indicated.

Imaging Studies

  • Complete echocardiography is indicated in any patient with suspected asplenia to rule out associated congenital heart disease.
  • Routine chest radiography is indicated to determine the cardiac size and location, to assess the bronchial anatomy, and to assess abdominal situs.
  • In addition, an upper GI study has been proposed as a routine study in asplenic patients because of the high incidence of intestinal malrotation and the risk of volvulus.

Other Tests

  • Liver-spleen scanning is indicated to confirm the presence of functional splenic tissue.
  • A 12-lead ECG is helpful in assessing patients with asplenia because an abnormal P wave axis is common, and conduction system abnormalities, including complete heart block, sick sinus syndrome, and supraventricular tachycardia (SVT), may occur, although they are less common than in polysplenia.
  • Additional studies of cardiac conduction may be indicated based on the clinical setting and ECG findings; in particular, 24-hour Holter monitoring may be recommended.

Procedures

  • Patients presenting with cardiac malformations often require cardiac catheterization on one or more occasions. In the newborn period, cardiac catheterization may be indicated to assess systemic and pulmonary venous connections, if this information cannot be obtained from echocardiography. Access can often be achieved through the femoral vein or the umbilical vein, although umbilical venous cannulation is more difficult in patients with asplenia than in those with most other congenital malformations. Venous access from the umbilicus or femoral vein is usually sufficient to perform both a right-heart and left-heart catheterization.
  • Later, cardiac catheterization may be indicated to determine individual candidacy for surgical intervention. Indications for catheterization may include assessment of pulmonary vascular resistance before palliation with a cavopulmonary connection. Assess pulmonary venous drainage in patients with unobstructed anomalous pulmonary venous return. In such cases, ruling out mild obstruction to pulmonary venous return, as is often observed on anomalous return to a confluence behind the right atrium or to the proximal superior vena cava (SVC), is important. Other indications for cardiac catheterization include assessment of ventricular size and visualization and potential embolization of aortopulmonary collateral vessels. Finally, electrophysiological testing may be indicated based on the clinical rhythm presentation.

More on Heterotaxy, Asplenia

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

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Keywords

asplenia, right atrial isomerism, laterality defects, cyanotic congenital heart disease, intestinal malrotation, anomalous pulmonary venous return, common atrium, endocardial cushion defects, double outlet right ventricle, treatment, diagnosis, biliary tract abnormalities, transverse liver, congestive heart failure, volvulus

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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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