Segmental pulmonary veno-occlusive disease secondary to lung cancer

Ibrahim, N. B. N.; Burnley, Holly; Gaber, Khalid; Ormerod, L J; Addis, B. J.; Capron, Frédérique; McGavin, C R

doi:10.1136/jcp.2004.020735

Cited by 13 publications

(7 citation statements)

References 7 publications

(3 reference statements)

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“…Genetic risk factors have been proposed due to the development of several cases of PVOD in siblings [18][19][20]. PVOD has also been reported in cancer patients following radiation and chemotherapy and after peripheral blood stem cell and bone marrow transplantation [21,22]. Overall, many of the etiological questions remain unanswered.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Veno-occlusive Disease: A Case Report and Review of Literature

El-Gammal¹,

Alsudairi²,

Alwohaibi³

et al. 2016

Clin Case Rep Rev

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An 18-year-old male was referred from a local hospital with history of progressive shortness of breath for six months. He also complained of a syncopal attack on exertion lasting for few seconds. The patient had no significant past medical history except mild intermittent bronchial asthma controlled on inhaled albuterol as required. He denied use of any medication and there was no family history of respiratory or cardiac illness. On examination, he was found to have regular heart rate of 90 beats per minute, respiratory rate of 22 breaths per minute, blood pressure of 117/74 mmHg and an oxygen saturation of 91% on room air. Cardiopulmonary examination revealed raised jugular venous pressure, positive left parasternal heave, loud pulmonary component of 2 nd heart sound, tricuspid regurgitation murmur, minimal basal crackled on chest auscultation and mild pedal edema. Abdominal examination showed soft, non-tender, lax abdomen with no organ enlargement and neurological examination was normal.Laboratory including complete blood count, liver and kidney functions were within normal range. Pro-B type natriuretic peptide was elevated at 654 pg/mL (normal <100 pg/mL). Screening for autoantibodies was negative.Chest X-ray (Figure 1) revealed enlarged pulmonary arteries and increased bronchovascular markings. ECG showed sinus tachycardia, right axis deviation and prominent P wave.A 2-dimensional transthoracic cardiac echocardiogram showed a severely dilated right ventricle, moderate tricuspid regurgitation with estimated systolic pulmonary artery pressure of 50 mmHg. Left ventricle was normal with preserved systolic function. Right heart catheterization result revealed mean pulmonary artery pressure >60 mmHg, Right atrial pressure 6 mmHg, pulmonary capillary wedge pressure 8 mmHg, pulmonary vascular resistance 13.78 Wood units and cardiac index 1.98 L/m/min.A computed tomographic angiogram (CTA) of chest (Figure 2) did not show any evidence of pulmonary embolism but there were dilated main pulmonary arteries, enlarged right atrium and ventricle, septal thickening with nodularity, centrilobular ground glass densities

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Section: Discussionmentioning

confidence: 99%

Pulmonary Veno-occlusive Disease: A Case Report and Review of Literature

El-Gammal¹,

Alsudairi²,

Alwohaibi³

et al. 2016

Clin Case Rep Rev

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“…This may explain the poor responses to PAH therapy observed in some patients with connective tissue disease associated PAH and complicates diagnostic efforts [20]. Several malignancies are associated with development of PVOD, including Hodgkin's lymphoma [21,22], primary lung cancers (squamous cell carcinoma) [23], and leiomyosarcoma [26]. Pulmonary veno-occlusive disease may develop after bone marrow transplantation [24,25] and renal transplantation [26] and has been linked to HIV infection [27].…”

Section: Risk Factorsmentioning

confidence: 99%

Pulmonary veno-occlusive disease: An important consideration in patients with pulmonary hypertension

Balko

Edriss

Nugent

et al. 2017

Respiratory Medicine

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Pulmonary veno-occlusive disease is a rare subcategory of pulmonary arterial hypertension (WHO Group 1). The disease is poorly understood and difficult to diagnose; it has no definitive cure to date. These patients present with nonspecific symptoms, including dyspnea, exercise intolerance, and weakness. Chest x-rays sometimes differ from idiopathic pulmonary arterial hypertension and may demonstrate alveolar infiltrates and pleural effusions. High resolution computed tomography scans reveal ground glass opacities, interlobular septal thickening, and lymphadenopathy. Echocardiography can estimate the level of pulmonary artery pressures; right heart catheterization is needed for complete hemodynamic characterization of these patients. Lung biopsies demonstrate remodeling of the venules and small veins with intimal and adventitial fibrosis. This can result in total venous occlusion and subsequent recanalization. Similar changes occur in the small arteries and arterioles but are less pronounced than the venous changes. There is no effective medical therapy for these patients, and treatment with the pulmonary arterial hypertension specific medications often causes acute deterioration with pulmonary edema. The recent discovery of the biallelic mutations of the EIF2AK4 gene as an etiology for heritable form of pulmonary veno-occlusive disease increases our understanding of the disease pathogenesis and potentially identifies a future approach to treatment. Without definitive treatment, the prognosis is very poor, and the life expectancy of these patients is much shorter than patients with pulmonary arterial hypertension. These patients need early referral to transplantation centers.

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“…Nonetheless, an exaggerated response to endothelial injury is believed an early inciting event, leading to activation of aberrant repair mechanisms and ultimately a widespread fibrosis of the lung microvasculature [28]. A number of anecdotal reports have linked development of PVOD to different forms of malignancy or exposure to various cytotoxic agents [29][30][31][32][33][34]. Peripheral blood stem cell transplantation [28], autologous and allogenic bone marrow transplantation [35,36], solid organ transplantation [37] and radiotherapy [32] have additionally been described as potential risk factors.…”

Section: Pathogenesismentioning

confidence: 99%