History
The presenting signs and symptoms of pulmonary veno-occlusive disease (PVOD) lack specificity. Most of the symptoms mimic other pulmonary and cardiac entities. The most common presenting symptoms are exertional dyspnea, fatigue, and cough. Sometimes, a respiratory tract infection–like illness may be identifiable preceding the diagnosis. Chronic cough (either productive or nonproductive) is present in some individuals.
In the later stages of PVOD, symptoms attributable to right ventricular failure, including chest pain and dizziness with exertion, abdominal pressure and tenderness secondary to hepatic congestion, and exertional syncope, may be noted. Hemoptysis with diffuse alveolar hemorrhage has been reported as a presentation. [32]
The suggestion of occult alveolar hemorrhage in bronchoalveolar lavage findings, however, is not uncommon. [33] Postural dyspnea or orthopnea may be reported by patients with PVOD but these findings are unusual among patients with primary pulmonary hypertension.
Physical Examination
Physical examination findings may be normal in patients with early pulmonary veno-occlusive disease (PVOD) disease. As the disease progresses, findings attributable to pulmonary hypertension and right-sided heart failure supervene. These findings include jugular venous distention, a right ventricular heave, a loud pulmonic heart sound, pedal edema, and epigastric tenderness. Inspiratory crackles may be heard if pulmonary infiltrates are prominent. [34] Clubbing is an unusual feature, [35] but has been described. Sudden death has also been reported as a presenting feature of PVOD. [36]
PVOD, being a postcapillary process, leads to increased visceral pleural and pulmonary capillary pressures with transudation of fluid into the pleural space resulting in pleural effusions. Pleural effusions are very rare in patients with primary pulmonary hypertension. [37]
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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.
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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.
Tables
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 |
