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Bryan E. McDonnell
Gary W. Raff
William M. DeCampli
Thomas L. Spray
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Ann Thorac Surg 1999;67:1391-1395
© 1999 The Society of Thoracic Surgeons


Original Articles

Outcome after repair of tetralogy of Fallot with absent pulmonary valve

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

a Division of Pediatric Cardiothoracic Surgery, the Cardiac Center, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
b Division of Cardiology, the Cardiac Center, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
c Department of Anesthesia and Critical Care, the Cardiac Center, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

Address reprint requests to Dr Gaynor, Department of Cardiac Surgery, Children’s Hospital of Philadelphia, Suite 8527, 34th St and Civic Center Blvd, Philadelphia, PA 19104-4399
e-mail: gaynor{at}email.chop.edu

Presented at the Forty-fifth Annual Meeting of the Southern Thoracic Surgical Association, Orlando, FL, Nov 12–14, 1998.

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.




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