A Devised Option of Neonatal Palliation for Compromised Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

• Published in: Annals of Thoracic and Cardiovascular Surgery Vol. 25; no. 5; pp. 274 - 277
• Main Authors: Kinami, Hiroo, Morita, Kiyozo, Ko, Yoshihiro, Shinohara, Gen, Hashimoto, Kazuhiro
• Format: Journal Article
• Language: English
• Published: Japan The Editorial Committee of Annals of Thoracic and Cardiovascular Surgery 01.01.2019
• Subjects: Blalock-Taussig Procedure; Blood Vessel Prosthesis; Blood Vessel Prosthesis Implantation - instrumentation; Case Report; Humans; Infant, Newborn; Male; neonatal palliation; Palliative Care; Polytetrafluoroethylene; Prosthesis Design; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - physiopathology; Pulmonary Artery - surgery; Pulmonary Valve - abnormalities; Pulmonary Valve - diagnostic imaging; Pulmonary Valve - physiopathology; small left ventricle; Tetralogy of Fallot - diagnostic imaging; Tetralogy of Fallot - physiopathology; Tetralogy of Fallot - surgery; tetralogy of Fallot with absent pulmonary valve syndrome; Treatment Outcome; small left ventricle; tetralogy of Fallot with absent pulmonary valve syndrome; neonatal palliation
• ISSN: 1341-1098; 2186-1005
• DOI: 10.5761/atcs.cr.17-00085

Neonatal primary repair of tetralogy of Fallot (TOF) with absent pulmonary valve (APV) syndrome is associated with high mortality rates. Our plan involves a staged repair that avoids one-stage intracardiac repair (ICR), with a first palliation that closes the main pulmonary orifice using an expanded polytetrafluoroethylene (ePTFE) patch, pulmonary arterioplication, and an adjustable Blalock-Taussig (BT) shunt. This strategy was used for a neonatal case with TOF/APV syndrome with hypoplastic left ventricle (LV). There was evidence of subsequent progressive increase in the LV size, and bronchial compression was relieved and an ICR was performed successfully at 9 months of age.