Pediatric Cardiology and Cardiac Surgery
Vol.27 No.2 2011 (62-68)

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Satoshi Yazaki

Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan


Pulmonary atresia with intact ventricular septum is a disease with various clinical courses derived from its morphological diversity. Management strategies are established with consideration principally of the tricuspid valve size and morphology, the right ventricular cavity, and the sinusoidal communications. With advances in catheter-based interventions in last decade, balloon pulmonary valvuloplasty (BPV) following perforation of the atretic valve has been becoming a treatment of choice in patients with good RV morphology. General agreement might be obtained about its indication as tricuspid valve Z-score > -1.5, RVEDV > 70% of the predicted value, and the absence of massive sinusoids. Because the course of patients with intermediate RV morphology is widespread, we should steer carefully along the serial changes of clinical manifestations. Referring to the technical aspect of BPV, use of a micro catheter is effective in order to squeeze through a small, perforated hole in the pulmonary valve.