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Pulmonary Atresia
Brett W. Carter, MD; Prabhakar Rajiah, MBBS, MD, FRCR, FSCMR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Pulmonary atresia with ventricular septal defect (PA-VSD)
            • Pulmonary atresia with intact ventricular septum (PA-IVS)
          • Definitions

            • Absence of communication between right ventricle (RV) and main pulmonary artery (MPA)
            • 2 distinct types based on interventricular septum
              • PA-IVS (intact ventricular septum)
                • Complete atresia of pulmonary valve
                • RV hypoplasia (single ventricle physiology) or dilation
                • Normal-sized PAs
                • Lungs supplied by PDA and PFO
                • RV to coronary connections in 50% at birth
                • Tricuspid valve hyopplasia/dysplasia
              • PA-VSD (ventricular septal defect)
                • Ranges from imperforate pulmonary valve (extreme variant of TOF, seen in 10%) to hypoplastic/absent pulmonary arteries and branches (pseudotruncus, truncus arteriosus type 4)
                • Major aortopulmonary collateral arteries (MAPCAs) supply 1 or both lungs
                • 2 functional ventricles exist
                • RV blood exits through overriding aorta and supplies systemic and pulmonary circuit
            • Category: Cyanotic, cardiomegaly, decreased ± irregular pulmonary vascularity

          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. Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. ePub, 2019
                                          2. Presnell LB et al: An overview of pulmonary atresia and major aortopulmonary collateral arteries. World J Pediatr Congenit Heart Surg. 6(4):630-9, 2015
                                          3. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                          4. Rajeshkannan R et al: Role of 64-MDCT in evaluation of pulmonary atresia with ventricular septal defect. AJR Am J Roentgenol. 194(1):110-8, 2010
                                          5. François CJ et al: Unenhanced MR angiography of the thoracic aorta: initial clinical evaluation. AJR Am J Roentgenol. 190(4):902-6, 2008
                                          6. Gaca AM et al: Repair of congenital heart disease: a primer--Part 2. Radiology. 248(1):44-60, 2008
                                          7. Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
                                          8. Dorfman AL et al: Magnetic resonance imaging evaluation of congenital heart disease: conotruncal anomalies. J Cardiovasc Magn Reson. 8(4):645-59, 2006
                                          9. Greil GF et al: Imaging of aortopulmonary collateral arteries with high-resolution multidetector CT. Pediatr Radiol. 36(6):502-9, 2006
                                          10. Boechat MI et al: Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 25(6):1535-46, 2005
                                          11. Roche KJ et al: Assessment of vasculature using combined MRI and MR angiography. AJR Am J Roentgenol. 182(4):861-6, 2004
                                          12. Baque J et al: Evaluation of pulmonary atresia with magnetic resonance imaging. Heart. 87(2):159, 2002
                                          13. Okada M et al: Modified Blalock-Taussig shunt patency for pulmonary atresia: assessment with electron beam CT. J Comput Assist Tomogr. 26(3):368-72, 2002
                                          14. Holmqvist C et al: Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect. Acta Radiol. 42(1):63-9, 2001
                                          15. Doyle TP et al: Tetralogy of Fallot and pulmonary atresia with ventricular septal defect. In: Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 391-408, 2000
                                          16. Freedom RM: Pulmonary atresia and intact ventricular septum. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 442-60, 2000
                                          17. Powell AJ et al: Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia. Int J Card Imaging. 16(3):169-74, 2000
                                          18. Ichida F et al: Evaluation of pulmonary blood supply by multiplanar cine magnetic resonance imaging in patients with pulmonary atresia and severe pulmonary stenosis. Int J Card Imaging. 15(6):473-81, 1999
                                          19. Westra SJ et al: Cardiac electron-beam CT in children undergoing surgical repair for pulmonary atresia. Radiology. 213(2):502-12, 1999
                                          20. Choe YH et al: MR imaging of non-visualized pulmonary arteries at angiography in patients with congenital heart disease. J Korean Med Sci. 13(6):597-602, 1998
                                          21. Frank H et al: Magnetic resonance imaging of absent pulmonary valve syndrome. Pediatr Cardiol. 17(1):35-9, 1996
                                          22. Taneja K et al: Comparison of computed tomography and cineangiography in the demonstration of central pulmonary arteries in cyanotic congenital heart disease. Cardiovasc Intervent Radiol. 19(2):97-100, 1996
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Pulmonary atresia with ventricular septal defect (PA-VSD)
                                                    • Pulmonary atresia with intact ventricular septum (PA-IVS)
                                                  • Definitions

                                                    • Absence of communication between right ventricle (RV) and main pulmonary artery (MPA)
                                                    • 2 distinct types based on interventricular septum
                                                      • PA-IVS (intact ventricular septum)
                                                        • Complete atresia of pulmonary valve
                                                        • RV hypoplasia (single ventricle physiology) or dilation
                                                        • Normal-sized PAs
                                                        • Lungs supplied by PDA and PFO
                                                        • RV to coronary connections in 50% at birth
                                                        • Tricuspid valve hyopplasia/dysplasia
                                                      • PA-VSD (ventricular septal defect)
                                                        • Ranges from imperforate pulmonary valve (extreme variant of TOF, seen in 10%) to hypoplastic/absent pulmonary arteries and branches (pseudotruncus, truncus arteriosus type 4)
                                                        • Major aortopulmonary collateral arteries (MAPCAs) supply 1 or both lungs
                                                        • 2 functional ventricles exist
                                                        • RV blood exits through overriding aorta and supplies systemic and pulmonary circuit
                                                    • Category: Cyanotic, cardiomegaly, decreased ± irregular pulmonary vascularity

                                                  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. Wright LK et al: Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart. ePub, 2019
                                                                                  2. Presnell LB et al: An overview of pulmonary atresia and major aortopulmonary collateral arteries. World J Pediatr Congenit Heart Surg. 6(4):630-9, 2015
                                                                                  3. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                                                                  4. Rajeshkannan R et al: Role of 64-MDCT in evaluation of pulmonary atresia with ventricular septal defect. AJR Am J Roentgenol. 194(1):110-8, 2010
                                                                                  5. François CJ et al: Unenhanced MR angiography of the thoracic aorta: initial clinical evaluation. AJR Am J Roentgenol. 190(4):902-6, 2008
                                                                                  6. Gaca AM et al: Repair of congenital heart disease: a primer--Part 2. Radiology. 248(1):44-60, 2008
                                                                                  7. Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
                                                                                  8. Dorfman AL et al: Magnetic resonance imaging evaluation of congenital heart disease: conotruncal anomalies. J Cardiovasc Magn Reson. 8(4):645-59, 2006
                                                                                  9. Greil GF et al: Imaging of aortopulmonary collateral arteries with high-resolution multidetector CT. Pediatr Radiol. 36(6):502-9, 2006
                                                                                  10. Boechat MI et al: Cardiac MR imaging and MR angiography for assessment of complex tetralogy of Fallot and pulmonary atresia. Radiographics. 25(6):1535-46, 2005
                                                                                  11. Roche KJ et al: Assessment of vasculature using combined MRI and MR angiography. AJR Am J Roentgenol. 182(4):861-6, 2004
                                                                                  12. Baque J et al: Evaluation of pulmonary atresia with magnetic resonance imaging. Heart. 87(2):159, 2002
                                                                                  13. Okada M et al: Modified Blalock-Taussig shunt patency for pulmonary atresia: assessment with electron beam CT. J Comput Assist Tomogr. 26(3):368-72, 2002
                                                                                  14. Holmqvist C et al: Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect. Acta Radiol. 42(1):63-9, 2001
                                                                                  15. Doyle TP et al: Tetralogy of Fallot and pulmonary atresia with ventricular septal defect. In: Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 391-408, 2000
                                                                                  16. Freedom RM: Pulmonary atresia and intact ventricular septum. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 442-60, 2000
                                                                                  17. Powell AJ et al: Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia. Int J Card Imaging. 16(3):169-74, 2000
                                                                                  18. Ichida F et al: Evaluation of pulmonary blood supply by multiplanar cine magnetic resonance imaging in patients with pulmonary atresia and severe pulmonary stenosis. Int J Card Imaging. 15(6):473-81, 1999
                                                                                  19. Westra SJ et al: Cardiac electron-beam CT in children undergoing surgical repair for pulmonary atresia. Radiology. 213(2):502-12, 1999
                                                                                  20. Choe YH et al: MR imaging of non-visualized pulmonary arteries at angiography in patients with congenital heart disease. J Korean Med Sci. 13(6):597-602, 1998
                                                                                  21. Frank H et al: Magnetic resonance imaging of absent pulmonary valve syndrome. Pediatr Cardiol. 17(1):35-9, 1996
                                                                                  22. Taneja K et al: Comparison of computed tomography and cineangiography in the demonstration of central pulmonary arteries in cyanotic congenital heart disease. Cardiovasc Intervent Radiol. 19(2):97-100, 1996