Korean J Pediatr.  2014 Jan;57(1):19-25. 10.3345/kjp.2014.57.1.19.

Primary repair of symptomatic neonates with tetralogy of Fallot with or without pulmonary atresia

Affiliations
  • 1Department of Thoracic Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea. leechha@sejongh.co.kr

Abstract

Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.

Keyword

Tetralogy of Fallot; Neonate; Early primary repair; Palliation

MeSH Terms

Anoxia
Cardiopulmonary Bypass
Fibrosis
Humans
Hypertrophy
Infant
Infant, Newborn*
Lung
Pathology
Pulmonary Atresia*
Pulmonary Valve
Pulmonary Valve Stenosis
Tetralogy of Fallot*
Thoracic Surgery
Ventricular Function, Right
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