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Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome
  1. Shinichiro Sakaki,
  2. Taiyu Hayashi and
  3. Hiroshi Ono
  1. Division of Pediatric Cardiology, National Center for Child Health and Development, Tokyo, Japan
  1. Correspondence to Dr Taiyu Hayashi, taiyuhayashi{at}


A 5-month-old girl with single ventricle, interrupted inferior vena cava and polysplenia syndrome palliated with bilateral Blalock-Taussig shunts developed severe cyanosis despite apparently increased pulmonary blood flow. Angiography revealed diffuse pulmonary arteriolar capillary dilatation and early pulmonary venous filling, suggesting the presence of pulmonary arteriovenous malformations. Abdominal angiography at 6 months demonstrated a large extrahepatic portosystemic shunt, which was percutaneously closed with a vascular plug. Kawashima operation was performed 2 weeks after portosystemic shunt closure. Although cyanosis improved temporarily, the patient suffered from deteriorating cyanosis at 9 months of age and underwent Fontan completion. Thereafter, her oxygen saturation gradually improved to 95% over the course of 3 weeks. Both the congenital extrahepatic portosystemic shunt and Kawashima operation contributed to the development of pulmonary arteriovenous malformations.

  • congenital disorders
  • portal vein
  • radiology
  • cardiovascular medicine
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  • Contributors SS drafted the manuscript. TH contributed to the conception of the work, wrote and revised the manuscript. HO critically reviewed the manuscript.

  • Funding This work was supported by Japan Society for the Promotion of Science,Grant-in-Aid(KAKENHI) No. 18K07835.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Parental/guardian consent obtained.

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