title: Staged repair of tetralogy of Fallot and diminutive pulmonary arteries with a fenestrated ventricular septal defect patch.
abstract: OBJECTIVE
  Patients with tetralogy of Fallot and diminutive pulmonary arteries are at risk for suprasystemic right ventricular pressure and right ventricular failure after complete repair. We report the short-term outcome and medium-term follow-up after using a fenestrated ventricular septal defect patch as a component of staged repair in selected patients.
  
  
  METHODS
  We reviewed 47 patients with tetralogy of Fallot and diminutive pulmonary arteries whose ventricular septal defect patch was fenestrated, either electively or as a rescue technique, at a single institution between 1984 and 2001.
  
  
  RESULTS
  Early mortality was 10.6% and occurred only in patients who underwent rescue fenestration. Review of medium-term follow-up (median, 39 months) revealed 4 late deaths; an additional 4 patients experienced right ventricular failure despite fenestration. Most (7/8) of these late events occurred in patients who underwent planned fenestration. Excessive left-to-right shunt through the fenestration developed in only 2 patients.
  
  
  CONCLUSIONS
  Fenestrated patch closure of the ventricular septal defect in patients with tetralogy of Fallot and diminutive pulmonary arteries resulted in 10.6% early mortality. Used preemptively in selected patients, this technique is associated with no surgical mortality and a low incidence of excessive left-to-right shunt (4%). Early survivors remain at risk for late death and right ventricular failure despite fenestration.
authors.affiliation: Department of Cardiology, Children's Hospital, Boston, MA 02115, USA. Audrey.marshall@cardi.chboston.org
authors.forename: Audrey C
authors.initials: AC
authors.lastname: Marshall
authors.forename: Barry A
authors.initials: BA
authors.lastname: Love
authors.forename: Peter
authors.initials: P
authors.lastname: Lang
authors.forename: Richard A
authors.initials: RA
authors.lastname: Jonas
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: del Nido
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.forename: James E
authors.initials: JE
authors.lastname: Lock
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000792:Angiography; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006344:Heart Septal Defects, Atrial / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis / Q000401:mortality; D011336:Probability; D018633:Pulmonary Atresia; D012189:Retrospective Studies; D018570:Risk Assessment; D013524:Surgical Flaps; D016019:Survival Analysis; D013771:Tetralogy of Fallot / Q000175:diagnosis / Q000401:mortality* / Q000601:surgery*; D016896:Treatment Outcome