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Pulmonary Venoocclusive Disease
Lynette Teo, MBChB(UK), FRCR(UK); Carol C. Wu, MD; Jeffrey P. Kanne, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Pulmonary venoocclusive disease (PVOD), pulmonary capillary hemangiomatosis (PCH)
            • Definitions

              • Rare causes of pulmonary artery hypertension (PAH) (WHO Group 1)
              • PVOD: Fibrous intimal proliferation and occlusion of pulmonary venules and small veins
              • PCH: Proliferation of capillaries within alveolar walls
              • PVOD and PCH are considered to be common entity and represent varied expressions of same disease
                • Postcapillary pathologies; behave similar to precapillary PAH

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Echocardiographic Findings

                      • Angiographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Reporting Tips

                                            Selected References

                                            1. Galiè N et al: 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 37(1):67-119, 2016
                                            2. Montani D et al: Pulmonary veno-occlusive disease. Eur Respir J. 47(5):1518-34, 2016
                                            3. Huertas A et al: Pulmonary veno-occlusive disease: advances in clinical management and treatments. Expert Rev Respir Med. 5(2):217-29; quiz 230-1, 2011
                                            4. Montani D et al: Pulmonary veno-occlusive disease: recent progress and current challenges. Respir Med. 104 Suppl 1:S23-32, 2010
                                            5. Iwaki M et al: Idiopathic pulmonary veno-occlusive disease. Intern Med. 48(15):1289-92, 2009
                                            6. Montani D et al: Idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: similarities and differences. Semin Respir Crit Care Med. 30(4):411-20, 2009
                                            7. Montani D et al: Pulmonary veno-occlusive disease. Eur Respir J. 33(1):189-200, 2009
                                            8. Frazier AA et al: From the Archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 27(3):867-82, 2007
                                            9. Resten A et al: CT imaging of peripheral pulmonary vessel disease. Eur Radiol. 15(10):2045-56, 2005
                                            10. Resten A et al: Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 183(1):65-70, 2004
                                            11. Swensen SJ et al: Pulmonary venoocclusive disease: CT findings in eight patients. AJR Am J Roentgenol. 167(4):937-40, 1996
                                            12. Hasleton PS et al: Pulmonary veno-occlusive disease. A report of four cases. Histopathology. 10(9):933-44, 1986
                                            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

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Pulmonary venoocclusive disease (PVOD), pulmonary capillary hemangiomatosis (PCH)
                                                      • Definitions

                                                        • Rare causes of pulmonary artery hypertension (PAH) (WHO Group 1)
                                                        • PVOD: Fibrous intimal proliferation and occlusion of pulmonary venules and small veins
                                                        • PCH: Proliferation of capillaries within alveolar walls
                                                        • PVOD and PCH are considered to be common entity and represent varied expressions of same disease
                                                          • Postcapillary pathologies; behave similar to precapillary PAH

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Echocardiographic Findings

                                                                • Angiographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Reporting Tips

                                                                                      Selected References

                                                                                      1. Galiè N et al: 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 37(1):67-119, 2016
                                                                                      2. Montani D et al: Pulmonary veno-occlusive disease. Eur Respir J. 47(5):1518-34, 2016
                                                                                      3. Huertas A et al: Pulmonary veno-occlusive disease: advances in clinical management and treatments. Expert Rev Respir Med. 5(2):217-29; quiz 230-1, 2011
                                                                                      4. Montani D et al: Pulmonary veno-occlusive disease: recent progress and current challenges. Respir Med. 104 Suppl 1:S23-32, 2010
                                                                                      5. Iwaki M et al: Idiopathic pulmonary veno-occlusive disease. Intern Med. 48(15):1289-92, 2009
                                                                                      6. Montani D et al: Idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: similarities and differences. Semin Respir Crit Care Med. 30(4):411-20, 2009
                                                                                      7. Montani D et al: Pulmonary veno-occlusive disease. Eur Respir J. 33(1):189-200, 2009
                                                                                      8. Frazier AA et al: From the Archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 27(3):867-82, 2007
                                                                                      9. Resten A et al: CT imaging of peripheral pulmonary vessel disease. Eur Radiol. 15(10):2045-56, 2005
                                                                                      10. Resten A et al: Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 183(1):65-70, 2004
                                                                                      11. Swensen SJ et al: Pulmonary venoocclusive disease: CT findings in eight patients. AJR Am J Roentgenol. 167(4):937-40, 1996
                                                                                      12. Hasleton PS et al: Pulmonary veno-occlusive disease. A report of four cases. Histopathology. 10(9):933-44, 1986