Outcome after repair of Tetralogy of Fallot with absent pulmonary valve

Bryan E. McDonnell, Gary W Raff, J. William Gaynor, Jack Rychik, Rudolfo I. Godinez, William M. DeCampli, Thomas L. Spray

Research output: Contribution to journalArticlepeer-review

43 Scopus citations


Background. Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression. Methods. Since January 1, 1984, 28 patients with TOF/APV have undergone complete repair (median age 11 days, range 1 day to 16 years). Results. Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04). Conclusions. Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.

Original languageEnglish (US)
Pages (from-to)1391-1396
Number of pages6
JournalAnnals of Thoracic Surgery
Issue number5
StatePublished - May 1 1999
Externally publishedYes

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery


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