Pulmonary atresia (PA) with intact ventricular septum (IVS)
PA with ventricular septal defect (VSD) is considered within tetralogy of Fallot (ToF)
Definitions
Obstruction to right ventricular outflow at level of pulmonary valve (PV)
IMAGING
General Features
Echocardiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Gottschalk I et al: Severe pulmonary stenosis or atresia with intact ventricular septum in the fetus: the natural history. Fetal Diagn Ther. 47(5):420-28, 2020
Hogan WJ et al: Fetal cardiac intervention for pulmonary atresia with intact ventricular septum: International Fetal Cardiac Intervention Registry. Fetal Diagn Ther. 1-9, 2020
Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. 105(13):1007-13, 2019
Cao L et al: Prenatal echocardiographic predictors of postnatal management strategy in the fetus with right ventricle hypoplasia and pulmonary atresia or stenosis. Pediatr Cardiol. 38(8):1562-8, 2017
Carrillo SA et al: Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals with absent intrapericardial pulmonary arteries. Ann Thorac Surg. 100(2):606-14, 2015
Lowenthal A et al: Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum. Fetal Diagn Ther. 35(2):101-7, 2014
Schneider AW et al: More than 25 years of experience in managing pulmonary atresia with intact ventricular septum. Ann Thorac Surg. 98(5):1680-6, 2014
Moon-Grady AJ et al: Effect of selective fetoscopic laser photocoagulation therapy for twin-twin transfusion syndrome on pulmonary valve pathology in recipient twins. Ultrasound Obstet Gynecol. 37(1):27-33, 2011
Tworetzky W et al: In Utero Valvuloplasty for Pulmonary Atresia With Hypoplastic Right Ventricle: Techniques and Outcomes. Pediatrics. Epub ahead of print, 2009
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Pulmonary stenosis (PS)
Pulmonary atresia (PA) with intact ventricular septum (IVS)
PA with ventricular septal defect (VSD) is considered within tetralogy of Fallot (ToF)
Definitions
Obstruction to right ventricular outflow at level of pulmonary valve (PV)
IMAGING
General Features
Echocardiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Gottschalk I et al: Severe pulmonary stenosis or atresia with intact ventricular septum in the fetus: the natural history. Fetal Diagn Ther. 47(5):420-28, 2020
Hogan WJ et al: Fetal cardiac intervention for pulmonary atresia with intact ventricular septum: International Fetal Cardiac Intervention Registry. Fetal Diagn Ther. 1-9, 2020
Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. 105(13):1007-13, 2019
Cao L et al: Prenatal echocardiographic predictors of postnatal management strategy in the fetus with right ventricle hypoplasia and pulmonary atresia or stenosis. Pediatr Cardiol. 38(8):1562-8, 2017
Carrillo SA et al: Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals with absent intrapericardial pulmonary arteries. Ann Thorac Surg. 100(2):606-14, 2015
Lowenthal A et al: Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum. Fetal Diagn Ther. 35(2):101-7, 2014
Schneider AW et al: More than 25 years of experience in managing pulmonary atresia with intact ventricular septum. Ann Thorac Surg. 98(5):1680-6, 2014
Moon-Grady AJ et al: Effect of selective fetoscopic laser photocoagulation therapy for twin-twin transfusion syndrome on pulmonary valve pathology in recipient twins. Ultrasound Obstet Gynecol. 37(1):27-33, 2011
Tworetzky W et al: In Utero Valvuloplasty for Pulmonary Atresia With Hypoplastic Right Ventricle: Techniques and Outcomes. Pediatrics. Epub ahead of print, 2009
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