Elsevier

Current Problems in Diagnostic Radiology

Volume 35, Issue 6, November–December 2006, Pages 219-223
Current Problems in Diagnostic Radiology

Original article
High-Resolution Computed Tomographic Imaging and Pathologic Features of Pulmonary Veno-Occlusive Disease: A Review of Three Patients

https://doi.org/10.1067/j.cpradiol.2006.07.004Get rights and content

Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare and severe form of pulmonary hypertension that is often difficult to differentiate from primary pulmonary hypertension. Differentiating these two entities before medical treatment is critical, as therapy commonly indicated for patients with primary pulmonary hypertension can be harmful and even fatal in patients with PVOD. In the setting of known pulmonary hypertension, computed tomography findings that are highly suggestive of PVOD include extensive, patchy centrilobular ground-glass opacities, ill-defined nodular densities, and interlobular septal thickening. Definitive diagnosis requires lung biopsy, demonstrating fibrous obliteration of the pulmonary venules and small veins of the lobular septa, with secondary medial hypertrophy of the pulmonary arteries. The purpose of this article is to review reported radiographic clues to the diagnosis of PVOD, as well as to illustrate these high-resolution computed tomography findings along with pathologic correlation.

Section snippets

Methods

The radiologic and pathologic images included in this pictorial essay were obtained from five patients who presented to our institution (Cleveland Clinic Foundation) between November 1997 and September 2005. All pathology samples were obtained via open lung biopsy.

All computed tomography (CT) images were reviewed by one of four fellowship-trained, board-certified chest radiologists. Chest CT scans (0.75-second scanning time per section, 120 to 140 kVp, 200 to 240 mA), each with the patient in

Clinical Features

Patients with PVOD often present in a clinically similar fashion to patients with PPH, with progressive dyspnea and signs of right-sided heart failure. Bibasilar rales on chest auscultation, digital clubbing, and >50% reduction in the diffusing capacity of the lung for carbon monoxide on pulmonary function testing may also be present.3 Multiple mismatched segmental perfusion defects on ventilation and perfusion scintigraphy despite negative angiogram has also been described.8

Differentiating

Pathology

Currently, surgical lung biopsy with histologic confirmation remains the only definitive means of diagnosing PVOD. The pathologic hallmark of PVOD is diffuse, eccentric intimal thickening, and, ultimately, fibrous obliteration of the pulmonary venules and small veins of the lobular septa (Fig 1). As the venous media thicken and undergo arterialization to form multiple elastic laminae, the muscular pulmonary arteries undergo secondary medial hypertrophy (Fig 2).3, 4 In addition to pulmonary

Imaging Findings

Due to the fragile hemodynamic status of most patients with longstanding pulmonary hypertension, surgical biopsy for definitive diagnosis of PVOD is most often contraindicated. In recent years, CT has provided a promising, noninvasive means of pretreatment screening to help identify patients at risk for adverse outcomes with vasodilator therapy.14, 17

In a case series of eight patients by Swensen and coworkers,6 the most commonly reported CT findings associated with PVOD included smooth

Conclusion

In the setting of pulmonary hypertension, HRCT findings of patchy centrilobular ground-glass attenuation, septal thickening, and associated reticulonodular densities should raise suspicion toward a possible diagnosis of PVOD. These findings may help to distinguish PVOD from PPH before standard medical treatment that might otherwise lead to near fatal complications. Definitive diagnosis requires lung biopsy demonstrating diffuse fibrous sclerosis throughout the pulmonary veins and venules with

References (17)

There are more references available in the full text version of this article.

Cited by (8)

  • Pulmonary venoocclusive disease in childhood

    2014, Chest
    Citation Excerpt :

    Especially if medical treatment of PH fails, PVOD should be strongly considered,7 as it is often misdiagnosed as idiopathic arterial hypertension.8 As epidemiologic data suggest, a proportion of 5% to 25% of primary PH will fulfill criteria for PVOD if fully investigated; the prevalence of only six pediatric patients (1.7%) in a large current registry37 suggests underdiagnosis of the disease. The use of pulmonary vasodilators (inhaled nitric oxide, epoprostenol, sildenafil) leads to improvement in some patients.

  • Pulmonary Hypertension

    2019, Imaging in Pediatric Pulmonology, Second Edition
  • Assessment of pulmonary vascular disease

    2016, Cardiac CT Imaging: Diagnosis of Cardiovascular Disease
  • Pulmonary hypertension in infants and children

    2012, Imaging in Pediatric Pulmonology
View all citing articles on Scopus
View full text