title: Tetralogy of Fallot with absent pulmonary valve syndrome; appropriate surgical strategies.
abstract: OBJECTIVE
  To evaluate patients presenting with Tetralogy of Fallot with absent pulmonary valve syndrome to a tertiary care hospital and their surgical management.
  
  
  METHODS
  The retrospective study was conducted at Congenital Cardiac Services, Aga Khan University Hospital, Karachi, Pakistan, and comprised data of Tetralogy of Fallot patients between April 2007 and June 2012. Data was analysed together with follow-up echocardiography. Variables assessed included demographics, imaging, operative technique, complications, post-operative recovery and follow-up echocardiography. SPSS 17 was used for statistical analysis.
  
  
  RESULTS
  Of the 204 patients, 6 (3%) had undergone surgical correction for Tetralogy of Fallot with absent pulmonary valve syndrome. All 6(100%) patients underwent complete repair. Median age for surgery was 8.5 years (range: 0.5-29 years). Of the different surgical strategies used, Contegra and Bioprosthetic valve placement had satisfactory outcome with minimal gradient at Right Ventricular Outflow Tract, good ventricular function and mild valvular regurgitation. One (16.6%) patient with Trans Annular Patch developed post-operative Right Ventricle Outflow Tract gradient of 80mmHg with moderate pulmonary regurgitation. One (16.6%) patient with monocusp valve developed free pulmonary regurgitation at 6 months. The other 4(66.6%) patients are currently free from any complications or re-intervention.
  
  
  CONCLUSIONS
  Early surgery is preferred in symptomatic patients. The repair depends upon achieving integrity of pulmonary circulation which is best achieved by using right ventricle to pulmonary artery conduit or inserting a pulmonary valve.
authors.affiliation: Medical graduate, MBBS, Agha Khan University, Karachi.
authors.forename: Fatima
authors.initials: F
authors.lastname: Shahid
authors.affiliation: Liaquat National Hospital and Medical College, University of Karachi.-
authors.forename: Maria Tariq
authors.initials: MT
authors.lastname: Siddiqui
authors.affiliation: Congenital Cardiothoracic Surgery, Department of Surgery, Aga Khan University, Karachi.
authors.forename: Muhammad Muneer
authors.initials: MM
authors.lastname: Amanullah
mesh_terms: D000293:Adolescent; D000328:Adult; D001705:Bioprosthesis; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D061345:Early Medical Intervention; D005260:Female; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D007223:Infant; D008297:Male; D010154:Pakistan; D011664:Pulmonary Valve / Q000002:abnormalities*; D011665:Pulmonary Valve Insufficiency / Q000150:complications / Q000601:surgery*; D012189:Retrospective Studies; D013577:Syndrome; D013771:Tetralogy of Fallot / Q000150:complications / Q000601:surgery*; D055815:Young Adult
keywords: Absent pulmonary valve syndrome, Contegra valved conduit, Pulmonary valve replacement, Trans-annular patch.