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Pulmonary Venoocclusive Disease/Pulmonary Capillary Hemangiomatosis
Claudio Silva, MD, MSc, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Pulmonary venoocclusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH)
                • Pulmonary arterial hypertension (PAH)
              • Definitions

                • Pulmonary hypertension with significant pulmonary venous &/or capillary wall proliferation (intimal hyperplasia)
              • Classification

                • Classified as group 1.6: PAH with overt features of venous/capillary (PVOD/PCH) involvement
                  • Previously considered separate entities; currently thought to represent continuum

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Nuclear Medicine Findings

                      • Angiographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          • Diagnosis

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              Selected References

                                              1. Foley RW et al: Computed tomography appearances of the lung parenchyma in pulmonary hypertension. Br J Radiol. 94(1117):20200830, 2021
                                              2. Remy-Jardin M et al: Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society. Radiology. 203108, 2021
                                              3. Ali N et al: Computed tomographic and clinical features of pulmonary veno-occlusive disease: raising the radiologist's awareness. Clin Radiol. 74(9):655-62, 2019
                                              4. Simonneau G et al: Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 53(1), 2019
                                              5. Montani D et al: Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study. Lancet Respir Med. 5(2):125-34, 2017
                                              6. Chaisson NF et al: Pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease. Clin Chest Med. 37(3):523-34, 2016
                                              7. Nazzareno G 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). Eur Respir J. 46(6):903-75, 1855-6, 2015
                                              8. Mineo G et al: Pulmonary veno-occlusive disease: the role of CT. Radiol Med. 119(9):667-73, 2014
                                              9. Frazier AA et al: From the archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 27(3):867-82, 2007
                                              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. Scheibel RL et al: Radiographic and angiographic characteristics of pulmonary veno-occlusive disease. Radiology. 103(1):47-51, 1972
                                              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

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Pulmonary venoocclusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH)
                                                            • Pulmonary arterial hypertension (PAH)
                                                          • Definitions

                                                            • Pulmonary hypertension with significant pulmonary venous &/or capillary wall proliferation (intimal hyperplasia)
                                                          • Classification

                                                            • Classified as group 1.6: PAH with overt features of venous/capillary (PVOD/PCH) involvement
                                                              • Previously considered separate entities; currently thought to represent continuum

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • Nuclear Medicine Findings

                                                                  • Angiographic Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      • Diagnosis

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          Selected References

                                                                                          1. Foley RW et al: Computed tomography appearances of the lung parenchyma in pulmonary hypertension. Br J Radiol. 94(1117):20200830, 2021
                                                                                          2. Remy-Jardin M et al: Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society. Radiology. 203108, 2021
                                                                                          3. Ali N et al: Computed tomographic and clinical features of pulmonary veno-occlusive disease: raising the radiologist's awareness. Clin Radiol. 74(9):655-62, 2019
                                                                                          4. Simonneau G et al: Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 53(1), 2019
                                                                                          5. Montani D et al: Clinical phenotypes and outcomes of heritable and sporadic pulmonary veno-occlusive disease: a population-based study. Lancet Respir Med. 5(2):125-34, 2017
                                                                                          6. Chaisson NF et al: Pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease. Clin Chest Med. 37(3):523-34, 2016
                                                                                          7. Nazzareno G 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). Eur Respir J. 46(6):903-75, 1855-6, 2015
                                                                                          8. Mineo G et al: Pulmonary veno-occlusive disease: the role of CT. Radiol Med. 119(9):667-73, 2014
                                                                                          9. Frazier AA et al: From the archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 27(3):867-82, 2007
                                                                                          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. Scheibel RL et al: Radiographic and angiographic characteristics of pulmonary veno-occlusive disease. Radiology. 103(1):47-51, 1972