Treatment algorithm for Pulmonary Atresia with Intact Ventricular Septum John E. Foker a, ⁎, James M. Berry b , Lee A. Pyles b a Division of Cardiothoracic Surgery, University of Minnesota, Minneapolis, MN, USA b Division of Cardiothoracic Pediatric Cardiology, University of Minnesota, Minneapolis, MN, USA abstract article info Available online 27 March 2010 Keywords: Pulmonary atresia with intact ventricular septum Hypoplasia SVR 2VR The articles in this issue reveal the complexity of the PAIVS spectrum and the methods of treatment that are currently being used. Despite the complexity, the articles provide greater understanding of the developmental events leading to these lesions and the methods that could be used to reverse them. Taken together, the evidence certainly favors embarking on a 2VR track whenever possible. The number of completed biventricular repairs will be increased and also the adverse later consequences of a hypertensive RV and persisting significant RV to coronary artery connections will be eliminated when they are not. The algorithm, therefore, is heavily weighted to 2VRs. © 2010 Elsevier Ireland Ltd. All rights reserved. Progress in the treatment of PAIVS patients is the goal of this special issue. The articles have provided important information which suggests that the patients should be placed on a 2VR track initially and, even if they eventually fall short, the situation will also be improved for those who end up with a SVR. A reasonable expectation is that a substantial increase in 2VRs should result and produce a similar improvement in long-term outcomes for the group. As opposed to beginning with the goal of a 2VR, a number of centers have attempted to improve the results by deciding at the initial evaluation whether a SVR or a 2VR track would be most appropriate. Usually, the degree of right heart hypoplasia is the deciding factor in choosing the treatment track. By sharpening the criteria for this stratification, some centers have achieved a reduction in overall mortality although not in SVR rates. The initial assignment into a SVR track, moreover, disregards the considerable growth potential and essentially eliminates the likelihood of having two functioning ventricles. The algorithm presented here, in contrast, begins with the assumption that a 2VR or something close to it is possible in these infants. The enlistment of the growth potential, wherever possible, should provide the best physiological solution with the greatest long- term benefit. Futhermore, leaving a hypertensive RV and persisting RV- CACs predictably have adverse consequences into adulthood, as presented in this issue by Tanoue et al and Ekman-Joelsson et al. Consequently, even if a 2VR is not completely realized, eliminating these lesions will still improve the long-term outlook. The algorithm portrays, in bolder type, the evaluations needed and the basic components required to achieve a 2VR outcome (Fig. 1). The algorithm also includes, in lighter type, the other procedures that may be needed to correct residual lesions, when present, to improve the 2VR result. Because of the spectrum of the lesions and the difficulties in achieving complete repair initially, some patients may require addi- tional procedures for optimum catch-up growth and function. For those patients who are only suitable for a palliative repair, the various possibilities of shunts, transplantation, and a Fontan proce- dure (SVR) are indicated with dashed lines. Diagnosis often begins by fetal echo and someday, fetal treatment may also be commonplace. For now, however, in utero valvotomies are done only at a small handful of centers. More time and experience will be required to accurately list the indications, procedures and likely results. Certainly, providing forward flow will encourage RV and TV growth and should limit the degree of hypoplasia although additional procedures to relieve residual RV outflow track obstruction will be needed. Nevertheless, when applicable, a satisfactory 2VR should be the outcome and in utero treatment will likely be used more in the future. The initial newborn evaluation will usually establish the details of PAIVS. Among these, the presence or absence of significant RV-CACs and associated coronary artery lesions, as well as the size and quality of the tricuspid valve are important issues. If there are significant RV-CACs, the evaluation should attempt to confirm that the coronary arteries are connected to the aorta. This is an important developmental event that rarely does not take place. Even a brief antegrade signal in diastole establishes continuity through to the aorta. If no continuity between aorta and coronary arteries exists transplantation seems to be the best option. The evaluation should then proceed to determine the location of the connections and identify, with the aid of angiography, significant stenoses resulting from the associated arteritis. From these studies, a plan for the treatment of the RV-CACs should emerge. The size of the TV is important and, in addition, about 25% of the valves have some degree of abnormality, principally stenosis, over and above the small size. Most often the TV abnormality does not need to be addressed early, in part because right heart function does not have to be fully normal for adequate pulmonary blood flow and also because some Progress in Pediatric Cardiology 29 (2010) 61–63 ⁎ Corresponding author. University of Minnesota, 420 Delaware St. SE MMC 495, Minneapolis, MN 55455, United States. Tel.: +1 612 625 0910; fax: +1 612 625 4106. E-mail address: foker001@umn.edu (J.E. Foker). 1058-9813/$ – see front matter © 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ppedcard.2010.02.005 Contents lists available at ScienceDirect Progress in Pediatric Cardiology journal homepage: www.elsevier.com/locate/ppedcard