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Creating a Fontan fenestration in a child with dextrocardia and interrupted inferior vena cava


Department of Pediatrics, Division of Cardiology, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA

Correspondence Address:
Pradeepkumar Charlagorla
Riley Hospital for Children, Indiana University School of Medicine, 705 Riley Hospital Drive RR127, Indianapolis - 46202, Indiana
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.173547

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Year : 2016  |  Volume : 9  |  Issue : 2  |  Page : 179-182

 

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Plastic bronchitis is a rare life-threatening complication of the Fontan operation. Transcatheter Fontan fenestration can ameliorate symptoms by decompressing elevated venous pressures. Transcatheter creation of a fenestration can be technically challenging in cases with complex venous anatomy. We report a case of a 5-year-old boy with heterotaxy, dextrocardia with unbalanced atrioventricular canal (AVC), atrial and visceral situs inversus, left-sided superior vena cava (SVC), and left-sided interrupted inferior vena cava (IVC) with azygos continuation. With few modifications to the equipment, a successful Fontan fenestration with stent implantation was performed via transjugular approach. At 2-year follow-up, his symptoms of plastic bronchitis improved significantly.






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Department of Pediatrics, Division of Cardiology, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA

Correspondence Address:
Pradeepkumar Charlagorla
Riley Hospital for Children, Indiana University School of Medicine, 705 Riley Hospital Drive RR127, Indianapolis - 46202, Indiana
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.173547

Rights and Permissions

Plastic bronchitis is a rare life-threatening complication of the Fontan operation. Transcatheter Fontan fenestration can ameliorate symptoms by decompressing elevated venous pressures. Transcatheter creation of a fenestration can be technically challenging in cases with complex venous anatomy. We report a case of a 5-year-old boy with heterotaxy, dextrocardia with unbalanced atrioventricular canal (AVC), atrial and visceral situs inversus, left-sided superior vena cava (SVC), and left-sided interrupted inferior vena cava (IVC) with azygos continuation. With few modifications to the equipment, a successful Fontan fenestration with stent implantation was performed via transjugular approach. At 2-year follow-up, his symptoms of plastic bronchitis improved significantly.






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