Diagnostic Considerations

In a patient with significant pulmonary hypertension, the possibility of chronic pulmonary thromboembolic disease must be considered because it is one of the common and treatable causes of pulmonary hypertension. More common causes of pulmonary venous hypertension (eg, systolic or diastolic left-sided heart failure) and valvular heart disease (eg, mitral stenosis) should be considered as a part of the differential diagnosis before a diagnosis of pulmonary veno-occlusive disease (PVOD) is established.

Vascular remodeling similar to PVOD occurs in both pulmonary veins and arteries in conditions such as mitral stenosis and fibrosing mediastinitis. However, the structural changes in the veins are more prominent in PVOD than in these other two conditions and may therefore provide a morphologic approach to differentiation of these diseases.^[25]

In some cases of PVOD, advanced parenchymal lung diseases (eg, sarcoidosis, interstitial lung disease, pneumoconioses) may be considered as diagnostic possibilities because of prominent interstitial changes seen on chest radiographs. However, the distinction between most forms of advanced interstitial lung disease from PVOD is relatively straightforward for most experts upon review of chest CT scans. Pulmonary capillary hemangiomatosis should also be considered in the differential diagnosis of PVOD.

Differential Diagnoses

Atrial Myxoma
Cardiogenic Pulmonary Edema
Cor Triatriatum
Drug-Induced Pulmonary Toxicity
Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD)
Mitral Stenosis
Idiopathic Pulmonary Arterial Hypertension
Pulmonary Capillary Hemagiomatosis
Pulmonary Embolism (PE)
Sarcoidosis
Pulmonary Arterial Hypertension

Workup

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Media Gallery

Medium-power photomicrograph (original magnification, X10; hematoxylin and eosin stain) demonstrates a fibrotic interlobular septum containing a vein with an occluded lumen (arrowhead). The occlusion is composed of dense, collagen-rich, fibrous tissue. Image courtesy of Thoracic Imaging Section, Department of Radiologic Pathology, Armed Forces Institute of Pathology.
Pulmonary veno-occlusive disease in a 43-year-old man. An axial computed tomography (CT) scan (lung window level) shows multiple septal lines (arrowhead) and a dilated central pulmonary artery (arrow). Image courtesy of Thoracic Imaging Section, Department of Radiologic Pathology, Armed Forces Institute of Pathology.

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Table. Distinguishing Pulmonary Edema From PVOD Based on Radiographic, Echocardiographic, and Heart Catheterization Data

Table. Distinguishing Pulmonary Edema From PVOD Based on Radiographic, Echocardiographic, and Heart Catheterization Data

Features	Pulmonary Edema	PVOD
Chest radiograph
Kerley B lines	Present	Present
Pleural effusion	Usually present	May be present
Enlarged cardiac silhouette	Present	Less prominent
Enlarged pulmonary artery	Present	Present
Chest CT scan
Thickened septae	Present	Present
Pleural effusion	Usually present	May be present
Enlarged heart	Present	Less prominent
Enlarged pulmonary artery	Present	Present
Septal concavity into left ventricle	Absent	Present
Echocardiogram
Pulmonary artery systolic pressure	Elevated (usually not >80 mm Hg)	Elevated (may be >80 mm Hg)
Left atrial enlargement	Present	Absent
Right atrial enlargement	Present	Present
Right ventricular hypertrophy	Absent	Present
Paradoxical septal motion	Absent	Present
Large pericardial effusion	Absent	May be present
Right-sided heart catheterization
Pulmonary vascular resistance	Below 3.0 Wood units	Above 3.0 Wood units
Pulmonary capillary wedge pressure	Above 18 mm Hg	Usually below 15 mm Hg (if catheter is properly wedged)
Mean pulmonary artery pressure	Elevated (almost never >50 mm Hg)	Elevated (may be >50 mm Hg)
Cardiac output	May be normal, low, or high	Mostly decreased, may be low normal
Oxyhemoglobin step-up	Absent	Absent

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Author

Hakim Azfar Ali, MD Consultant in Pulmonary and Critical Care Medicine, Christiana Care Hospital

Hakim Azfar Ali, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Shoaib Alam, MD Staff Clinician, Pulmonary and Vascular Medicine, National Heart, Lung, and Blood Institute, National Institutes of Health

Shoaib Alam, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, Society of Critical Care Medicine, International Society for Magnetic Resonance in Medicine, European Respiratory Society, Pennsylvania Thoracic Society

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, FACP, FCCP Geri and Richard Brawerman Chair in Pulmonary and Critical Care Medicine, Professor and Executive Vice Chairman, Department of Medicine, Medical Director, Women's Guild Lung Institute, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgements

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP Professor of Genomics and Personalized Medicine Research, Internal Medicine, and Pediatrics, Associate Director, Center for Genomics and Personalized Medicine Research, Director of Research, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Wake Forest University School of Medicine

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, and Sigma Xi

Disclosure: See below for list of all activities None None

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment