Bookmarks
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
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
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
