Pulmonary Atresia with Intact Ventricular Septum (PA/IVS)
Because some of the technical details of this procedure are different from those described in the section on critical PS, PA/IVS is described separately. Patients with short-segment (membrane-like) pulmonary atresia are cyanotic at birth and require PGE for maintenance of pulmonary blood flow. This is perhaps the most straightforward example of ductal-dependant pulmonary blood flow. Among those with PA/IVS there is a spectrum, ranging from those with relatively good-sized RV chambers to those with significant RV hypoplasia or RV-dependent coronary circulation (1). Some degree of tricuspid valve hypoplasia is the rule and generally follows RV hypoplasia. Nevertheless, many patients with PA/IVS can achieve a biventricular circulation, and often their first step toward this is a trip as a newborn to the catheterization lab for valve perforation and dilation.
KeywordsTricuspid Valve Pulmonary Valve Pulmonary Blood Flow Pulmonary Atresia Coaxial Catheter
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
Daubeney PE, Delaney DJ, Anderson, RH et al. Pulmonary atresia with intact ventricular septum range of morphology in a population-based study. J Am Coll Cardiol
Giglia TM, Mandell VS, Connor AR et al. Diagnosis and management of right ventricle–dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation
Guleserian KJ, Armsby LB, Thiagarajan RR et al. Natural history of pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg
Agnoletti G, Piechaud JF, Bonhoeffer P et al. Perforation of the atretic pulmonary valve long-term follow-up. J Am Coll Cardiol
Humpl T, Soderberg B, McCrindle BW et al. Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular septum impact on patient care. Circulation
© Springer Science+Business Media, LLC 2009