title: Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy.
abstract: 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.
authors.forename: Diana E
authors.initials: DE
authors.lastname: Drogalis-Kim
authors.forename: Brian L
authors.initials: BL
authors.lastname: Reemtsen
authors.forename: Leigh Christopher
authors.initials: LC
authors.lastname: Reardon
mesh_terms: D000293:Adolescent; D001705:Bioprosthesis; D018618:Echocardiography, Doppler, Color; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000295:instrumentation; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011474:Prosthesis Design; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D013771:Tetralogy of Fallot / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D016896:Treatment Outcome
keywords: Abnormalities, multiple/diagnosis/surgery; adolescent; heart defects, congenital/diagnosis/surgery; pulmonary artery/pathology; pulmonary valve/abnormalities; tetralogy of Fallot/complications/surgery; time factors; treatment outcome