Case Reports

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Journal of Structural Heart Disease, April 2019, Volume 5, Issue 2:38-42
DOI: 10.12945/j.jshd.2019.014.18

Diagnosis and Management of Fontan Failure Secondary to Aortopulmonary Artery Fistula

Sanja Dzelebdzic, MD1*, Sherif Tawfik, MD2, Lucas Collazo, MD3, Michael Shorofsky, MD1,4, Alan Benheim, MD2, Annette Ansong, MD2, Sharon Karr, MD2

1 Department of Pediatrics, MedStar Georgetown University Hospital, Washington, DC, USA
2 Division of Pediatric Cardiology, INOVA Children's Hospital, Falls Church, Virginia, USA
3 Division of Pediatric and Congenital Cardiac Surgery, INOVA Children's Hospital, Falls Church, Virginia, USA
4 Division of Pediatrics, INOVA Children's Hospital, Falls Church, Virginia, USA


Aortopulmonary artery fistula represents a rare anomalous communication between aorta and pulmonary artery. The treatment of these communications is well established and involves either surgical or percutaneous approach. We present a 15-year-old male with history of hypoplastic left ventricle, hypoplastic aorta and ventricular septal defect with Damus-Kaye-Stansel surgery in the past, who developed acute Fontan circuit failure secondary to the development of aortopulmonary fistula of unknown etiology. Fistula was successfully closed percutaneously, using Amplatzer duct occluder.

Supplemental Media

  • Video 1

    The reversal of flow in descending aorta in diastole without aortic regurgitation.

  • Video 2

    Shunt-like flow coming from aorta to pulmonary artery (transthoracic echocardiogram).

  • Video 3

    APF between the posterior right aspect of the aorta and the pulmonary artery (transesophageal echocardiogram).

  • Video 4

    Contrast washout in pulmonary artery suggesting additional flow coming from high pressure setting (pulmonary angiogram).

  • Video 5

    Aortopulmonary artery fistula.

  • Video 6

    Device position with trivial residual shunting.


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Cite this article as: Dzelebdzic S, Tawfik S, Collazo L, Shorofsky M, Benheim A, Ansong A, Karr S. Diagnosis and Management of Fontan Failure Secondary to Aortopulmonary Artery Fistula. Structural Heart Disease 2019;5(2):38-42. DOI: 10.12945/j.jshd.2019.014.18

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