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Pulmonary Atresia
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Pulmonary atresia (PAt)
        • Synonyms

          • Sometimes referred to as truncus arteriosus type 4 or pseudotruncus (misnomer)
        • Definitions

          • 2 distinct entities, differentiated by presence or absence of ventricular septal defect (VSD)
            • PAt, VSD, multiple aortopulmonary collateral arteries (MAPCAs): Hypoplastic/absent pulmonary arteries (PAs); MAPCAs supply 1 or both lungs
              • Type A: Normal-sized PAs with small aortopulmonary (AP) collaterals
              • Type B: Mild to moderate PA hypoplasia with multiple AP collaterals
              • Type C: Markedly hypoplastic PAs with pulmonary flow from numerous AP collaterals
            • PAt, intact ventricular septum (VS): Normal-sized PAs supplied by patent ductus arteriosus (PDA), patent foramen ovale (PFO)
              • Coronary sinusoids or fistulas are often present
          • Both are characterized by underdevelopment of right ventricular outflow tract (RVOT) & pulmonary valve
            • PAt, VSD, MAPCAs: At extreme end of spectrum of RVOT-obstructive (Fallot-type) heart lesions with complex & highly variable PA anatomy
          • Category: Cyanotic, cardiomegaly, ↓ &/or irregular pulmonary vasculature
          • Hemodynamics: Extreme outflow obstruction of RV; (almost) entire cardiac output goes into dilated overriding ascending aorta

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Echocardiographic Findings

                  • Angiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Gindes L et al: Prenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus. J Matern Fetal Neonatal Med. 1-9, 2021
                                        2. Katz JD et al: A 15-year-old with tetralogy of Fallot with pulmonic atresia, multiple aorticopulmonary collateral arteries and bilateral peripheral pulmonary stenosis. Cureus. 12(10):e11151, 2020
                                        3. Sinha M et al: Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography. J Card Surg. 35(11):2979-86, 2020
                                        4. Fishbein GA et al: Tricuspid and pulmonic valve pathology. Curr Cardiol Rep. 21(7):54, 2019
                                        5. Rato J et al: Ebstein's anomaly with 'reversible' functional pulmonary atresia. BMJ Case Rep. 12(12), 2019
                                        6. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                        7. Jonas SN et al: Pulmonary valve anatomy and abnormalities: a pictorial essay of radiography, computed tomography (CT), and magnetic resonance imaging (MRI). J Thorac Imaging. 31(1):W4-12, 2016
                                        8. Byeon JH et al: Coronary artery collateral flow and its effect on myocardial perfusion in a patient with unilateral pulmonary artery atresia. Cardiol Young. 1-4, 2015
                                        9. Ryan JR et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals. JACC Cardiovasc Imaging. 8(1):103-4, 2015
                                        10. Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
                                        11. Morozov AA: [Surgical treatment of pulmonic atresia with defect of interventricular septum and collateral lung circulation.] Vestn Khir Im I I Grek. 172(5):115-9, 2013
                                        12. Séguéla PE et al: Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum. Pediatr Radiol. 41(8):1069-72, 2011
                                        13. Kawel N et al: Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia--usefulness of MR angiography in clinical routine. J Cardiovasc Magn Reson. 12:52, 2010
                                        14. Formigari R et al: Prevalence of bilateral patent ductus arteriosus in patients with pulmonic valve atresia and asplenia syndrome. Am J Cardiol. 70(13):1219-20, 1992
                                        15. Hruda J et al: Diagnostic selective balloon occlusion technique in pulmonic valve atresia and ventricular septal defect. Am J Cardiol. 63(18):1408-9, 1989
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Pulmonary atresia (PAt)
                                              • Synonyms

                                                • Sometimes referred to as truncus arteriosus type 4 or pseudotruncus (misnomer)
                                              • Definitions

                                                • 2 distinct entities, differentiated by presence or absence of ventricular septal defect (VSD)
                                                  • PAt, VSD, multiple aortopulmonary collateral arteries (MAPCAs): Hypoplastic/absent pulmonary arteries (PAs); MAPCAs supply 1 or both lungs
                                                    • Type A: Normal-sized PAs with small aortopulmonary (AP) collaterals
                                                    • Type B: Mild to moderate PA hypoplasia with multiple AP collaterals
                                                    • Type C: Markedly hypoplastic PAs with pulmonary flow from numerous AP collaterals
                                                  • PAt, intact ventricular septum (VS): Normal-sized PAs supplied by patent ductus arteriosus (PDA), patent foramen ovale (PFO)
                                                    • Coronary sinusoids or fistulas are often present
                                                • Both are characterized by underdevelopment of right ventricular outflow tract (RVOT) & pulmonary valve
                                                  • PAt, VSD, MAPCAs: At extreme end of spectrum of RVOT-obstructive (Fallot-type) heart lesions with complex & highly variable PA anatomy
                                                • Category: Cyanotic, cardiomegaly, ↓ &/or irregular pulmonary vasculature
                                                • Hemodynamics: Extreme outflow obstruction of RV; (almost) entire cardiac output goes into dilated overriding ascending aorta

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Echocardiographic Findings

                                                        • Angiographic Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Staging, Grading, & Classification

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Gindes L et al: Prenatal diagnosis of major aortopulmonary collateral arteries (MAPCA) in fetuses with pulmonary atresia with ventricular septal defect and agenesis of ductus arteriosus. J Matern Fetal Neonatal Med. 1-9, 2021
                                                                              2. Katz JD et al: A 15-year-old with tetralogy of Fallot with pulmonic atresia, multiple aorticopulmonary collateral arteries and bilateral peripheral pulmonary stenosis. Cureus. 12(10):e11151, 2020
                                                                              3. Sinha M et al: Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography. J Card Surg. 35(11):2979-86, 2020
                                                                              4. Fishbein GA et al: Tricuspid and pulmonic valve pathology. Curr Cardiol Rep. 21(7):54, 2019
                                                                              5. Rato J et al: Ebstein's anomaly with 'reversible' functional pulmonary atresia. BMJ Case Rep. 12(12), 2019
                                                                              6. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                                                              7. Jonas SN et al: Pulmonary valve anatomy and abnormalities: a pictorial essay of radiography, computed tomography (CT), and magnetic resonance imaging (MRI). J Thorac Imaging. 31(1):W4-12, 2016
                                                                              8. Byeon JH et al: Coronary artery collateral flow and its effect on myocardial perfusion in a patient with unilateral pulmonary artery atresia. Cardiol Young. 1-4, 2015
                                                                              9. Ryan JR et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals. JACC Cardiovasc Imaging. 8(1):103-4, 2015
                                                                              10. Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
                                                                              11. Morozov AA: [Surgical treatment of pulmonic atresia with defect of interventricular septum and collateral lung circulation.] Vestn Khir Im I I Grek. 172(5):115-9, 2013
                                                                              12. Séguéla PE et al: Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum. Pediatr Radiol. 41(8):1069-72, 2011
                                                                              13. Kawel N et al: Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia--usefulness of MR angiography in clinical routine. J Cardiovasc Magn Reson. 12:52, 2010
                                                                              14. Formigari R et al: Prevalence of bilateral patent ductus arteriosus in patients with pulmonic valve atresia and asplenia syndrome. Am J Cardiol. 70(13):1219-20, 1992
                                                                              15. Hruda J et al: Diagnostic selective balloon occlusion technique in pulmonic valve atresia and ventricular septal defect. Am J Cardiol. 63(18):1408-9, 1989