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Absent pulmonary valve, tricuspid atresia, and congenital heart block. 2015
Ignacio Juaneda, and
Jack Rychik, and
Stephanie Fuller, and
Paul M Weinberg, and
Jonathan J Rome, and
William T Mahle, and
J William Gaynor
Division of Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, PA, USA ignaciojuaneda@hotmail.com.
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.
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