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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
- Classified as group 1.6: PAH with overt features of venous/capillary (PVOD/PCH) involvement
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
- Foley RW et al: Computed tomography appearances of the lung parenchyma in pulmonary hypertension. Br J Radiol. 94(1117):20200830, 2021
- Remy-Jardin M et al: Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society. Radiology. 203108, 2021
- 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
- Simonneau G et al: Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 53(1), 2019
- 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
- Chaisson NF et al: Pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease. Clin Chest Med. 37(3):523-34, 2016
- 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
- Mineo G et al: Pulmonary veno-occlusive disease: the role of CT. Radiol Med. 119(9):667-73, 2014
- Frazier AA et al: From the archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 27(3):867-82, 2007
- Resten A et al: Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 183(1):65-70, 2004
- Swensen SJ et al: Pulmonary venoocclusive disease: CT findings in eight patients. AJR Am J Roentgenol. 167(4):937-40, 1996
- Scheibel RL et al: Radiographic and angiographic characteristics of pulmonary veno-occlusive disease. Radiology. 103(1):47-51, 1972
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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
- Classified as group 1.6: PAH with overt features of venous/capillary (PVOD/PCH) involvement
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
- Foley RW et al: Computed tomography appearances of the lung parenchyma in pulmonary hypertension. Br J Radiol. 94(1117):20200830, 2021
- Remy-Jardin M et al: Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society. Radiology. 203108, 2021
- 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
- Simonneau G et al: Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 53(1), 2019
- 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
- Chaisson NF et al: Pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease. Clin Chest Med. 37(3):523-34, 2016
- 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
- Mineo G et al: Pulmonary veno-occlusive disease: the role of CT. Radiol Med. 119(9):667-73, 2014
- Frazier AA et al: From the archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 27(3):867-82, 2007
- Resten A et al: Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 183(1):65-70, 2004
- Swensen SJ et al: Pulmonary venoocclusive disease: CT findings in eight patients. AJR Am J Roentgenol. 167(4):937-40, 1996
- Scheibel RL et al: Radiographic and angiographic characteristics of pulmonary veno-occlusive disease. Radiology. 103(1):47-51, 1972
