How is pulmonary veno-occlusive disease (PVOD) differentiated from pulmonary edema?

Updated: Oct 16, 2018
  • Author: Hakim Azfar Ali, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Pulmonary veno-occlusive disease (PVOD) should be suspected in patients who have been diagnosed with pulmonary arterial hypertension (PAH) but who have radiographic findings suggestive of left-sided heart failure. The diagnosis is based on clinical and radiographic findings. Surgical or transbronchial biopsy should not be performed secondary to the very high rate of complications, including death.

Most patients with PVOD present with progressive dyspnea upon exertion. [38] Often, right-sided heart failure is initially suspected (owing to edema, jugular venous distention, a loud P2 sound, and hypoxemia) or left-sided heart failure is considered (secondary to radiographic findings of bilateral pulmonary infiltrates and Kerley B lines).

When these patients are evaluated using echocardiography or right-sided heart catheterization, the diagnosis of pulmonary hypertension is confirmed but their wedge pressure (if the pulmonary artery catheter is properly wedged) is within normal limits. In summary, PVOD is currently recognized based on one of two sets of findings, as follows (see the Table below):

  • The patient is diagnosed with pulmonary arterial hypertension (PAH) but a review of the chest radiograph and CT scan raises the suggestion of pulmonary edema.

  • The patient is diagnosed with suspected pulmonary edema but echocardiography or right-sided heart catheterization reveals pulmonary hypertension.

Table. Distinguishing Pulmonary Edema From PVOD Based on Radiographic, Echocardiographic, and Heart Catheterization Data (Open Table in a new window)

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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