7. Outcome Following Repair of Tetralogy of Fallot with Absent Pulmonary Valve
Bryan E. McDonnell, J. William Gaynor, Jack Rychik, Rodolfo I. Godinez, William M. DeCampli, Thomas L. Spray
The Childrens Hospital of Philadelphia
Philadelphia, Pa.
Background: Tetralogy of Fallot with Absent Pulmonary Valve (TOP/APV) is associated with airway anomalies.
Methods: Since January 1, 1984, 26 patients with TOF/APV have undergone complete correction (median age 28 days, rage one day to 16 years). A retrospective analysis was performed.
Results: Eleven patients were ventilated for respiratory failure preoperatively and three underwent ECMO. Pulmonary arterioplasty was performed in 24 (anterior to 11, anterior/posterior in 13). The RV outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (four), or monocusp valve (three). Two patients underwent tracheotomy. Early mortality was 19.2 percent (5/26) with one late death. All early deaths occurred in patients who were incubate preoperatively. Survival was 84.1±7.2 percent at one year and 79.5±8.2 percent at 10 years. Three patients underwent RVOT revision for persistent respiratory symptoms which resolved after repeat application and placement of a valved conduit. Freedom from death or re-operation was 79.9±8.0 percent at one year and 70.1±9.6 percent at 10 years. By univariate analysis, only preoperative respiratory failure was a predictor of mortality. At follow-up 70 percent (14/20) had no respiratory symptoms.
Conclusions: Long-term outcome for most patients with TOF/APV is good. In patients with persistent respiratory symptoms, replication and revision of RVOT may improve outcome.