Pulmonary vein stenosis: expression of receptor tyrosine kinases by lesional cells

Pulmonary vein stenosis and necrotising enterocolitis: Is there a possible link with necrotising enterocolitis?

“…While the exact pathophysiology of NEC is not well known, it is hypothesised that due to intestinal tract immaturity there is an inappropriate response to any intestinal injury. Recent evidence suggests that it may result from an exaggerated inflammatory response mounted by intestinal epithelial cells and mediated by VEGF, which has also been suggested as an associated factor in acquired PVS 2 4 5 9. We did note that infants who had NEC tended to have worse PVS with a higher gradient across the veins, possibly due to a heightened inflammatory response.…”

“…A study published by Riedlinger et al 4 focused on the histology of acquired PVS. Their findings suggested that intimal lesions are the result of a myofibroblast-like proliferation, with this process being mediated in part by expression of receptor tyrosine kinases, such as VEGF.…”

Surgical repair for primary pulmonary vein stenosis: Single-institution, midterm follow-up

“…5 From the histopathologic perspective, neointimal proliferation and medial hypertrophy have been described as main causative pathogenetic processes leading to occlusion of the luminal contour; proliferation of myofibroblast-like cells accounts for this progression. [6][7][8] Treatments that include percutaneous and surgical intervention are, unfortunately, followed by progression of the disease, and restenosis within weeks to months, owing to the malignant obliterative disease biology, which is refractory to current therapeutic methods. 9,10 Sutureless pericardial marsupialization (SPM) has been the preferred approach, in many centers, to relieve obstruction and reduce the restenosis rate; its indication has been extended from postrepair PVS to primary PVS.…”

Pulmonary vein stenosis in patients with Smith-Lemli-Opitz syndrome

“…1,3 Pathologic specimens demonstrate neo-intimal proliferation of myofibroblasts as the primary etiology for the progressive luminal stenosis and obliteration of the vessels, 4 with immunohistochemistry demonstrating strong receptor tyrosine kinase expression, such as VEGF. 5 In some children, obstruction to pulmonary venous flow can be improved with surgical procedures such as sutureless marsupialization repair or catheter-based interventions such as balloon dilation and stenting. 6–8 More recently, drug therapies have been attempted to delay disease progression and to treat the intimal proliferation within the vessels.…”