title: Absent pulmonary valve, tricuspid atresia, and congenital heart block.
abstract: We describe management of a patient with a prenatal diagnosis of absent pulmonary valve, tricuspid atresia, ventricular septal defect, and congenital heart block. Initial treatment consisted of temporary pacemaker implantation, and subsequent palliation included a central shunt during the neonatal period and placement of a permanent pacemaker. At seven months of age, a bidirectional Glenn anastomosis was performed. Cardiac catheterization revealed high cavopulmonary pressures and ventricular dysfunction precluding Fontan completion. Heart transplantation was performed at 3.75 years of age. The patient is alive and well 26 months posttransplantation.
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, PA, USA ignaciojuaneda@hotmail.com.
authors.forename: Ignacio
authors.initials: I
authors.lastname: Juaneda
authors.affiliation: Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Fuller
authors.affiliation: Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Paul M
authors.initials: PM
authors.lastname: Weinberg
authors.affiliation: Division of Pediatric Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Jonathan J
authors.initials: JJ
authors.lastname: Rome
authors.affiliation: Division of Pediatric Cardiology, Emory University, Atlanta, GA, USA.
authors.forename: William T
authors.initials: WT
authors.lastname: Mahle
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, PA, USA.
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery* / Q000628:therapy; D006328:Cardiac Catheterization; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D018729:Fontan Procedure; D006327:Heart Block / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery / Q000628:therapy; D006345:Heart Septal Defects, Ventricular / Q000175:diagnosis / Q000601:surgery* / Q000628:therapy; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010138:Pacemaker, Artificial; D011247:Pregnancy; D011296:Prenatal Diagnosis; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery; D018785:Tricuspid Atresia / Q000175:diagnosis / Q000601:surgery* / Q000628:therapy
keywords: CHD; heart; pacemaker; pulmonary valve; transplantation; tricuspid atresia; univentricular heart