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Life-saving stepwise management of pulmonary hypertension in an infant with Down syndrome presenting with a large patent ductus arteriosus and giant hepatic arteriovenous malformation

Published online by Cambridge University Press:  18 August 2025

Emine Gulsah Torun Open the ORCID record for Emine Gulsah Torun [Opens in a new window] ,
Nevin Özdemiroğlu ,
Velihan Çayhan  and
İbrahim Ece
Emine Gulsah Torun*
Affiliation:
Department of Pediatric Cardiology, Ankara Bilkent City Hospital, Ankara, Turkey
Nevin Özdemiroğlu
Affiliation:
Department of Pediatric Cardiology, Gaziantep City Hospital, Gaziantep, Turkey
Velihan Çayhan
Affiliation:
Department of Radiology, Ankara Bilkent City Hospital, Ankara, Turkey
İbrahim Ece
Affiliation:
Department of Pediatric Cardiology, Ankara Bilkent City Hospital, Ankara, Turkey
*
Corresponding author: Emine Gulsah Torun; Email: drgtorun@gmail.com
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Abstract

We report a rare case of a 3-month-old male infant with Down syndrome, severe pulmonary arterial hypertension (a large patent ductus arteriosus, an atrial septal defect, and a giant hepatic arteriovenous malformation). Initial treatment involved hepatic arteriovenous malformation embolisation using 17 Azur® CX Peripheral Coils, resulting in clinical improvement. However, persistent pulmonary arterial hypertension led to patent ductus arteriosus closure using an 8-mm Lifetech Cera™ vascular plug after a successful balloon occlusion test. Post-intervention, the patient showed symptomatic relief, reduced right ventricular overload, and atrial septal defect shunting shifted to left to right. This is the first reported paediatric case combining these features successfully managed through a multidisciplinary and stepwise treatment approach, eliminating the need for liver transplantation.

Keywords

Coil embolisationhepatic arteriovenous malformationpatent ductus arteriosuspulmonary arterial hypertension

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 8 , August 2025 , pp. 1730 - 1733
Copyright
© The Author(s), 2025. Published by Cambridge University Press

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References

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