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Proximal Interruption of Pulmonary Artery
Emily Tsai, MD; Carol C. Wu, MD; Tyler H. Ternes, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Proximal interruption of pulmonary artery (PIPA)
            • Synonyms

              • Unilateral absence (agenesis) of pulmonary artery (PA)
                • "Interruption" preferred over "absence" since intrapulmonary portion of PA is intact
            • Definitions

              • Failed development of proximal PA

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Williams EA et al: Proximal interruption of the pulmonary artery. J Thorac Imaging. 34(1):56-64, 2019
                                      2. Walker CM et al: Bronchial arteries: anatomy, function, hypertrophy, and anomalies. Radiographics. 35(1):32-49, 2015
                                      3. Dillman JR et al: Expanding upon the unilateral hyperlucent hemithorax in children. Radiographics. 31(3):723-41, 2011
                                      4. Lee EY et al: Multidetector CT evaluation of congenital lung anomalies. Radiology. 247(3):632-48, 2008
                                      5. CastaƱer E et al: Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics. 26(2):349-71, 2006
                                      6. Newman B: Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol. 36(8):773-91, 2006
                                      7. Ryu DS et al: HRCT findings of proximal interruption of the right pulmonary artery. J Thorac Imaging. 19(3):171-5, 2004
                                      8. Ten Harkel AD et al: Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 122(4):1471-7, 2002
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Proximal interruption of pulmonary artery (PIPA)
                                                • Synonyms

                                                  • Unilateral absence (agenesis) of pulmonary artery (PA)
                                                    • "Interruption" preferred over "absence" since intrapulmonary portion of PA is intact
                                                • Definitions

                                                  • Failed development of proximal PA

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Angiographic Findings

                                                          • Nuclear Medicine Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Williams EA et al: Proximal interruption of the pulmonary artery. J Thorac Imaging. 34(1):56-64, 2019
                                                                          2. Walker CM et al: Bronchial arteries: anatomy, function, hypertrophy, and anomalies. Radiographics. 35(1):32-49, 2015
                                                                          3. Dillman JR et al: Expanding upon the unilateral hyperlucent hemithorax in children. Radiographics. 31(3):723-41, 2011
                                                                          4. Lee EY et al: Multidetector CT evaluation of congenital lung anomalies. Radiology. 247(3):632-48, 2008
                                                                          5. CastaƱer E et al: Congenital and acquired pulmonary artery anomalies in the adult: radiologic overview. Radiographics. 26(2):349-71, 2006
                                                                          6. Newman B: Congenital bronchopulmonary foregut malformations: concepts and controversies. Pediatr Radiol. 36(8):773-91, 2006
                                                                          7. Ryu DS et al: HRCT findings of proximal interruption of the right pulmonary artery. J Thorac Imaging. 19(3):171-5, 2004
                                                                          8. Ten Harkel AD et al: Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 122(4):1471-7, 2002