Introduction: The management of small infants with tetralogy of Fallot (TOF) with pulmonary atresia (PA) and hypoplastic pulmonary arteries can be very challenging.
Methods: In three small infants (weight range 2,200-3,600 g, pulmonary trunk 2.0-3.2 mm), initial palliation consisted of sternotomy, transventricular puncture of the right ventricular outflow tract and atretic pulmonary valve, followed by balloon dilation (n = 1) or stent deployment (n = 2) from the right ventricle into the pulmonary trunk (stent diameter 5-6 mm, length 16 mm).
Results: The procedure resulted in adequate palliation with good anterograde flow to the pulmonary arteries and near normal saturations in all three patients (>92%); there was no associated morbidity. Additional transvenous stenting was required in all patients because of progressive muscular infundibular stenosis after a median of 3 months. Two patients evolved to full repair at the age of 5 months and one patient with multiple hilar stenoses requires additional percutaneous procedures through the stented RV outflow tract.
Conclusion: Transventricular balloon dilation and stenting of the RVOT through medial sternotomy as initial palliation strategy appears a safe and well tolerated alternative treatment in small infants with TOF with PA and a hypoplastic pulmonary trunk.
Keywords: congenital heart disease; hybrid procedure; palliation; perventricular; premature infant; pulmonary atresia; small infant; stenting; tetralogy of Fallot; transventricular.
© 2013 Wiley Periodicals, Inc.