Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy

Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thriv...

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Bibliographic Details
Published in:Texas Heart Institute journal 2016-12, Vol.43 (6), p.517-519
Main Authors: Drogalis-Kim, Diana E, Reemtsen, Brian L, Reardon, Leigh Christopher
Format: Article
Language:English
Subjects:
Adolescent
Bioprosthesis
Case Reports
Echocardiography, Doppler, Color
Heart Valve Prosthesis
Heart Valve Prosthesis Implantation - instrumentation
Humans
Magnetic Resonance Imaging
Male
Prosthesis Design
Pulmonary Valve - abnormalities
Pulmonary Valve - diagnostic imaging
Pulmonary Valve - surgery
Tetralogy of Fallot - complications
Tetralogy of Fallot - diagnostic imaging
Tetralogy of Fallot - surgery
Treatment Outcome
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Description
Summary:Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. We describe the unusual case of a mildly symptomatic 16-year-old boy with these conditions who underwent successful primary repair. Our search of the medical literature yielded fewer than 5 cases of tetralogy of Fallot with absent pulmonary valve (or variants with an absent left pulmonary artery) and survival without repair into later adolescence or adulthood.
ISSN:0730-2347
1526-6702
DOI:10.14503/THIJ-15-5385