Annotated References Freedom RM Pulmonary Atresia With Intact Ventricular Septum Futura: Mount Kisco, NY; 1989 A superb monograph of all facets of this disease Although the outcome data are dated, this is an excellent resource for those wishing further insight into this disease Freedom RM, Anderson RH, Perrin D The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum Cardiol Young 2005;15:447–468 An encyclopedic review ostensibly of the role of fistulas in this condition but containing a wideranging review of historical perspective, morphology, pathology, fetal diagnosis, and outcome Freedom RM, Jaeggi E, Perrin D, et al The “wallto-wall” heart in the patient with pulmonary atresia and intact ventricular septum Cardiol Young 2006;16:18–29 A further in-depth review focusing on the dilated RV in this condition A definitive review containing historical perspective, morphology, fetal diagnosis, and outcome Hanley FL, Sade RM, Blackstone EH, et al Outcomes in neonatal pulmonary atresia with intact ventricular septum J Thorac Cardiovasc Surg 1993;105:406–427 Ashburn MD, Blackstone EH, Wells WJ, et al Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum J Thorac Cardiovasc Surg 2004;127:1000–1008 The early and midterm results from the Congenital Heart Surgeons Study of pulmonary atresia with intact ventricular septum in North America This is the largest study of the disease to date with 408 patients There is excellent statistical analysis (parametric modelling of time-related death) particularly of optimal initial procedure relating to the size of the tricuspid valve, and factors influencing achievement of definitive repair Daubeney PEF, Sharland GK, Cook AC, et al Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome Circulation 1998;98:562–566 Daubeney PE, Delany DJ, Anderson RH, et al Pulmonary atresia with intact ventricular septum: range of morphology in a populationbased study J Am Coll Cardiol 2002;39:1670– 1679 Daubeney PEF, Wang D, Delany DJ, et al Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study J Thorac Cardiovasc Surg 2005;130:1071 The results from the collaborative study performed in the United Kingdom and Ireland This is the largest population-based study of this disease containing all 183 patients born in a fixed geographical area in a fixed time frame There are detailed data about fetal diagnosis and its effects The range of morphology is described in a population-based setting, which can serve as a reference to ensure future interventional studies reflect the population at large Outcome data are given and risk factors influencing poor outcome There were a high proportion of primary catheter interventions in this study Dyamenahalli U, McCrindle BW, McDonald C, et al Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients Cardiol Young 2004;14:299–308 The results from Hospital for Sick Children,