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Polysplenia with pulmonary arteriovenous malformations

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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.

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References

  1. Anabtawi IN, Ellison RG, Ellison LT (1965) Pulmonary arteriovenous aneurysms and fistulas: Anatomic variations, embryology, and classification.Ann Thorac Surg 1:277–285

    Google Scholar 

  2. Burke CM, Safai C, Nelson DP, Ruffin TA (1986) Pulmonary arteriovenous malformations: A critical update.Am Rev Respir Dis 134:334–339

    Google Scholar 

  3. Debich, DE, Devine WA, Anderson RH, Patt FRC (1980) Polysplenia with normally structured hearts.Am J Cardiol 65:1274–1275

    Google Scholar 

  4. Groniowski J (1960) Morphological investigations on pulmonary circulation in the neonatal period.J Dis Children 99:146–153

    Google Scholar 

  5. Hansotti RC, Sharma S (1989) Cirrhosis of the liver simulating congenital cyanotic heart disease.Chest 96:843–848

    Google Scholar 

  6. Moller JH, Amplatz K, Wolfson J (1971) Malrotation of the bowel in patients with congenital heart disease associated with splenic anomalies.Radiology 99:393–398

    Google Scholar 

  7. Peoples W, Moller JH, Edwards JE (1983) Polysplenia A review of 146 cases.Pediatr Cardiol 4:129–137

    Google Scholar 

  8. Peery WH (1987) Clinical spectrum of hereditary hemorrhagic telangectasia (Olser, Weber, Rendu disease).Am J Med 82:989–997

    Google Scholar 

  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 37:840–852

    Google Scholar 

  10. Rydell R, Haffbauer FW (1956) Multiple pulmonary arteriovenous fistulas in juvenile cirrhosis.Am J Med 21:450–459

    Google Scholar 

  11. Van Mierop LHS, Gessner IH, Schiebler GL (1972) Asplenia and polysplenia syndromes.Birth Defects: Original Article Series, VIII:36–44

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Division of, Duke University Medical Center, P.O. Box 3195, 27710, Durham, North Carolina, USA

    John Papagiannis, Ronald J. Kanter & Brenda E. Armstrong

  2. Division of Pediatric Pulmonary Medicine, Duke University Medical Center, P.O. Box 3195, 27710, Durham, North Carolina, USA

    Roxanne Marcille & Iley B. Browning III

  3. Division of Pediatric Radiology, Duke University Medical Center, P.O. Box 3195, 27710, Durham, North Carolina, USA

    Eric L. Effman

  4. Department of Pathology, Duke University Medical Center, P.O. Box 3195, 27710, Durham, North Carolina, USA

    Philip C. Pratt

Authors
  1. John Papagiannis
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  2. Ronald J. Kanter
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  3. Eric L. Effman
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  4. Philip C. Pratt
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  5. Roxanne Marcille
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  6. Iley B. Browning III
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  7. Brenda E. Armstrong
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Papagiannis, J., Kanter, R.J., Effman, E.L. et al. Polysplenia with pulmonary arteriovenous malformations. Pediatr Cardiol 14, 127–129 (1993). https://doi.org/10.1007/BF00796995

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  • DOI: https://doi.org/10.1007/BF00796995

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