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J Thorac Cardiovasc Surg 2003;126:1427-1433
© 2003 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Staged repair of tetralogy of Fallot and diminutive pulmonary arteries with a fenestrated ventricular septal defect patch

^a Department of Cardiology, Children's Hospital, Boston, Mass, USA
^b Department of Cardiovascular Surgery, Children's Hospital, Boston, Mass, USA

Received for publication December 11, 2002; revisions received February 10, 2003; revisions received February 24, 2003; accepted for publication April 11, 2003.

^* Address for reprints: Audrey C. Marshall, MD, Department of Cardiology, Children's Hospital, 300 Longwood Ave, Boston, MA 02115, USA.
Audrey.marshall{at}cardio.chboston.org

OBJECTIVES: 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.

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