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Native atrial septal restriction after Fontan palliation successfully treated with transcatheter Diabolo stent


Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children's Hospital, Iowa City, Iowa, USA

Correspondence Address:
Osamah Aldoss
Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children's Hospital, 200 Hawkins Dr., Iowa City - 52242, Iowa
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.171411

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Year : 2016  |  Volume : 9  |  Issue : 1  |  Page : 49-52

 

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A 6-year-old male child born with hypoplastic left heart syndrome (HLHS) was palliated with an extracardiac nonfenestrated Fontan procedure (18-mm Gore-Tex tube graft). He developed low-pressure (mean Fontan pressure 10 mmHg) protein-losing enteropathy 6 months after Fontan palliation. After initially responding to medical therapy and transcatheter pulmonary artery stent implantation, he developed medically refractory protein-losing enteropathy. At this time, his transthoracic echocardiogram showed new restriction across his native atrial septum with an 8 mmHg mean gradient. Cardiac catheterization now showed high-pressure (mean Fontan pressure 18-20 mmHg) protein-losing enteropathy and a new 6 mmHg mean gradient across the atrial septum. To avoid cardiopulmonary bypass, he underwent successful transcatheter relief of atrial septal restriction and creation of a fenestration with rapid clinical and biochemical improvement of his protein-losing enteropathy.






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Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children's Hospital, Iowa City, Iowa, USA

Correspondence Address:
Osamah Aldoss
Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children's Hospital, 200 Hawkins Dr., Iowa City - 52242, Iowa
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.171411

Rights and Permissions

A 6-year-old male child born with hypoplastic left heart syndrome (HLHS) was palliated with an extracardiac nonfenestrated Fontan procedure (18-mm Gore-Tex tube graft). He developed low-pressure (mean Fontan pressure 10 mmHg) protein-losing enteropathy 6 months after Fontan palliation. After initially responding to medical therapy and transcatheter pulmonary artery stent implantation, he developed medically refractory protein-losing enteropathy. At this time, his transthoracic echocardiogram showed new restriction across his native atrial septum with an 8 mmHg mean gradient. Cardiac catheterization now showed high-pressure (mean Fontan pressure 18-20 mmHg) protein-losing enteropathy and a new 6 mmHg mean gradient across the atrial septum. To avoid cardiopulmonary bypass, he underwent successful transcatheter relief of atrial septal restriction and creation of a fenestration with rapid clinical and biochemical improvement of his protein-losing enteropathy.






[FULL TEXT] [PDF]*


        
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