Japanese Journal of Cardiovascular Surgery Vol44,No2

New Staged Repair of Neonatal Tetralogy of Fallot with Severe Absent Pulmonary Valve Syndrome

Hiroo Kinami Kiyozo Morita Yoshihiro Ko
Gen Shinohara and Kazuhiro Hashimoto

(Department of Cardiac Surgery, The Jikei University School of Medicine, Tokyo, Japan)

Primary repair of the tetralogy of Fallot with absent pulmonary valve syndrome(TOF/APV)is associated with high mortality rates of 17-33%, especially in neonates. Our standard strategy involves a staged repair with a first palliation, performed during the neonatal period, that includes main pulmonary septation with an ePTFE patch, pulmonary arterioplasty for reduction of vascular dilation, and a modified Blalock-Taussig shunt. We performed successful repairs on two neonates with TOF/APV, one symptomatic and the other non-symptomatic, with this strategy. Case 1:A 7-day-old boy had TOF/APV, with progressively worsening respiratory distress. His left bronchi, superior vena cava and left atrium were compressed by a dilated pulmonary artery, which was repaired by emergency surgery. Decreasing the diameter of the pulmonary artery(PA index from 2,550 to 525)relieved the compressed organs. Case 2:A 16-day-old boy with TOF/APV with a main pulmonary artery that increased in diameter from 8 to 17mm in the course of a single day. He was treated in the same fashion as Case 1. At 1 year of age, an intracardiac repair with tricuspid anuuloplasty was performed successfully. This strategy is much safer than a primary repair and is a good choice for neonatal repair of TOF/APV. 


Jpn. J. Cardiovasc. Surg. 44:97-102(2015)

Keywords:tetralogy of Fallot;absent pulmonary valve syndrome;neonatal;staged repair

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