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Pulmonary Stenosis, Atresia
Michael D. Puchalski, MD; Whitnee Hogan, MD
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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

                                    1. 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
                                    2. 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
                                    3. Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. 105(13):1007-13, 2019
                                    4. 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
                                    5. 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
                                    6. 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
                                    7. 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
                                    8. 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
                                    9. 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

                                                                      1. 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
                                                                      2. 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
                                                                      3. Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. 105(13):1007-13, 2019
                                                                      4. 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
                                                                      5. 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
                                                                      6. 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
                                                                      7. 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
                                                                      8. 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
                                                                      9. Tworetzky W et al: In Utero Valvuloplasty for Pulmonary Atresia With Hypoplastic Right Ventricle: Techniques and Outcomes. Pediatrics. Epub ahead of print, 2009