Page 25 - Journal of Structural Heart Disease Volume 5, Issue 2

Case Report
Journal of Structural Heart Disease, April 2019, Volume 5, Issue 2:38-42
DOI: https://doi.org/10.12945/j.jshd.2019.014.18
Received: April 27, 2018 Accepted: May 26, 2018 Published online: April 2019
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
Abstract
Aortopulmonary artery fistula represents a rare anomalous communication between aorta and pulmo- nary artery. The treatment of these communications is well established and involves either surgical or percu- taneous approach. We present a 15-year-old male with history of hypoplastic left ventricle, hypoplastic aorta and ventricular septal defect with Damus-Kaye-Stan- sel surgery in the past, who developed acute Fontan circuit failure secondary to the development of aor- topulmonary fistula of unknown etiology. Fistula was successfully closed percutaneously, using Amplatzer duct occluder.
Copyright © 2019 Science International Corp.
Key Words
Aortopulmonary artery fistula • Congenital heart disease • Duct occluder
Introduction
Aortopulmonary artery fistula (APF) represents an anomalous communication between aorta and pul- monary artery. It is a rare occurrence. There are two types of APFs described in the literature: congenital andacquired.CongenitalAPFsareextremelyrareand have only been described in case reports [1]. On the
other hand, acquired APFs are relatively more com- mon and usually associated with pseudo-aneurysm that breaks into the pulmonary artery [2-4]. They may or may not be associated with trauma [5]. Treatment for these abnormal communications is well estab- lished and is either surgical or percutaneous [2, 6]. We present an interesting case of Fontan circuit failure due to development of aortopulmonary artery fistula in a 15-year-old male with history of complex congen- ital heart disease including hypoplastic left ventricle, hypoplastic aorta and ventricular septal defect (VSD) with Damus-Kaye-Stansel surgery in the past followed by Fontan palliation with fenestration and transcath- eter fenestration closure years after primary surgery. He presented to emergency department with increas- ing abdominal pain in the setting of recent bicycle ac- cident followed by dyspnea on exertion.
Case presentation
15-year-old male with history of complex congen- ital heart disease, status post Fontan palliation pre- sented to an outside hospital (OSH) ER two weeks after a fall while riding his bike when flipped over the handle bars. Over the two-week period following the accident, patient developed increasing abdominal discomfort and dyspnea on exertion and finally pre-
* Corresponding Author:
Sanja Dzelebdzic, MD
Department of Pediatrics
MedStar Georgetown University Hospital
3800 Reservoir Road, NW, Washington, DC 20007, USA
Tel. +1 202 431 3509; E-Mail: Sanja.Dzelebdzic@gunet.georgetown.edu
Fax +1 203 785 3346
E-Mail: jshd@scienceinternational.org http://structuralheartdisease.org/
© 2019 Journal of Structural Heart Disease Published by Science International Corp. ISSN 2326-4004
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