Pulmonary vein stenosis and the pathophysiology of “upstream” pulmonary veins

Abstract

Neonatal pulmonary vein banding in piglets recapitulates critical aspects of clinical PVS and highlights a pathologic profile consistent with EndMT, supporting the rationale for evaluating therapeutic strategies designed to exploit reversibility of upstream pulmonary vein pathology.

Integrins αvβ5 and αvβ3 promote latent TGF-β1 activation by human cardiac fibroblast contraction

“…To induce fibrosis in the right ventricle, 7-day-old Yorkshire piglets (3.9 + 0.8 kg) underwent staged bilateral pulmonary vein banding (banded, n ¼ 3) or sham operations (sham, n ¼ 3), as described previously. 22 First, the banding of left pulmonary and common pulmonary veins was performed via a left fifth intercostal space thoracotomy. One week later, banding of the right upper and middle pulmonary veins was carried out via a right fourth intercostal space thoracotomy.…”

“…Pulmonary vein stenosis in Yorkshire pigs leads to pressure overload and hypertrophy of the right ventricle as we demonstrated recently. 22 To control for the development of fibrosis after banding the pulmonary veins for 7 weeks, tissue sections of the right ventricular myocardium were stained with H&E to visualize cardiomyocytes and Masson's trichrome for collagen accumulation ( Figure 1A). Figure 1A and see Supplementary material online, Figure S1A).…”

Pulmonary vein stenosis: Plea for a multi-institutional registry

“…2,3,4 This paper continues that contribution by reinforcing the value of MRI in imaging the PVs before intervention, and by providing a valuable "hint" that pre-operative PV size measurements relate to outcome. The task of definitively demonstrating this relationship is daunting for any single institution, however, because (1) PVS is relatively rare, (2) MRI/CT are relatively recently-employed diagnostic modalities, and (3) MRI is not easily employed in an important subset of the cohort, i.e., the small infant .…”

Repair Type Influences Mode of Pulmonary Vein Stenosis in Total Anomalous Pulmonary Venous Drainage

“…The cross-sectional image analysis also demonstrated that approximately 40% of the affected PVs in the SR group had diffuse PVO. A recent animal study from our group demonstrated that downstream PVO causes intimal thickening of the upstream individual PVs as a result of endothelial-mesenchymal transition [5]. The affected upstream PVs have loss of endothelial markers and increased mesenchymal marker expression, which was partially reversible by relief of the downstream PVO.…”

“…The location of PVO associated with standard repair (SR) is typically at the anastomotic site, probably owing to the combination of inflammation, fibrosis, and direct purse string effect. However, anastomotic obstruction could result in diffuse PVO through retrograde extension of the fibrotic process into the upstream PVs [5].…”