Original articleHigh-Resolution Computed Tomographic Imaging and Pathologic Features of Pulmonary Veno-Occlusive Disease: A Review of Three Patients
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
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Cited by (8)
Pulmonary venoocclusive disease in childhood
2014, ChestCitation 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 EditionAssessment of pulmonary vascular disease
2016, Cardiac CT Imaging: Diagnosis of Cardiovascular DiseasePulmonary hypertension in infants and children
2012, Imaging in Pediatric PulmonologyPulmonary veno-occlusive disease: A misnomer?
2012, Pediatric Radiology