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Pediatric Cardiology

, Volume 14, Issue 2, pp 127–129 | Cite as

Polysplenia with pulmonary arteriovenous malformations

  • John Papagiannis
  • Ronald J. Kanter
  • Eric L. Effman
  • Philip C. Pratt
  • Roxanne Marcille
  • Iley B. BrowningIII
  • Brenda E. Armstrong
Case Reports

Summary

A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.

Key Words

Polysplenia Pulmonary arteriovenous malformations Portosystemic anastomosis 

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References

  1. 1.
    Anabtawi IN, Ellison RG, Ellison LT (1965) Pulmonary arteriovenous aneurysms and fistulas: Anatomic variations, embryology, and classification.Ann Thorac Surg 1:277–285Google Scholar
  2. 2.
    Burke CM, Safai C, Nelson DP, Ruffin TA (1986) Pulmonary arteriovenous malformations: A critical update.Am Rev Respir Dis 134:334–339Google Scholar
  3. 3.
    Debich, DE, Devine WA, Anderson RH, Patt FRC (1980) Polysplenia with normally structured hearts.Am J Cardiol 65:1274–1275Google Scholar
  4. 4.
    Groniowski J (1960) Morphological investigations on pulmonary circulation in the neonatal period.J Dis Children 99:146–153Google Scholar
  5. 5.
    Hansotti RC, Sharma S (1989) Cirrhosis of the liver simulating congenital cyanotic heart disease.Chest 96:843–848Google Scholar
  6. 6.
    Moller JH, Amplatz K, Wolfson J (1971) Malrotation of the bowel in patients with congenital heart disease associated with splenic anomalies.Radiology 99:393–398Google Scholar
  7. 7.
    Peoples W, Moller JH, Edwards JE (1983) Polysplenia A review of 146 cases.Pediatr Cardiol 4:129–137Google Scholar
  8. 8.
    Peery WH (1987) Clinical spectrum of hereditary hemorrhagic telangectasia (Olser, Weber, Rendu disease).Am J Med 82:989–997Google Scholar
  9. 9.
    Rose V, Izukawa T, Moses CAF (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 37:840–852Google Scholar
  10. 10.
    Rydell R, Haffbauer FW (1956) Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis.Am J Med 21:450–459Google Scholar
  11. 11.
    Van Mierop LHS, Gessner IH, Schiebler GL (1972) Asplenia and polysplenia syndromes.Birth Defects: Original Article Series, VIII:36–44Google Scholar

Copyright information

© Springer-Verlag New York Inc. 1993

Authors and Affiliations

  • John Papagiannis
    • 1
  • Ronald J. Kanter
    • 1
  • Eric L. Effman
    • 3
  • Philip C. Pratt
    • 4
  • Roxanne Marcille
    • 2
  • Iley B. BrowningIII
    • 2
  • Brenda E. Armstrong
    • 1
  1. 1.Department of Pediatrics, Division ofDuke University Medical CenterDurhamUSA
  2. 2.Division of Pediatric Pulmonary MedicineDuke University Medical CenterDurhamUSA
  3. 3.Division of Pediatric RadiologyDuke University Medical CenterDurhamUSA
  4. 4.Department of PathologyDuke University Medical CenterDurhamUSA

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