Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort

Abstract

Pulmonary vein stenosis of ex-premature infants is a complex problem with poor survival, delayed diagnosis, and unsatisfactory treatment. The lack of concordance in twins suggests epigenetic or environmental factors may play a role in the development of pulmonary vein stenosis. In ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia a focused echocardiographic assessment of the pulmonary veins is required with further imaging if the echocardiogram is inconclusive.

Mortality in infants with bronchopulmonary dysplasia: Data from cardiac catheterization

“…However, the role and accuracy of CT angiogram and MRI to diagnose PVS are unclear. Thus cardiac catheterization remains the gold standard . Taken together with prior anatomic and histologic observations, our findings of the relationship of PVS and possibly higher PVR as a predictor of mortality, suggest the pulmonary vascular disease is an important influence in the outcome of former infants with BPD and PH, although the underlying mechanisms remain speculative.…”

“…Our study highlights the importance of evaluating the pulmonary veins in this patient cohort during cardiac catheterization. A prior cohort of former preterm infants with PVS demonstrated increased mortality with bilateral disease or of greater than or equal to three vessels affected and a high rate of missed PVS diagnosis by echocardiography . In this cohort of 39 preterm infants with BPD and PVS, the first definitive diagnosis of PVS was made using echocardiography in 22 of 39 (56%) patients, CT angiography in 8 of 39 (21%) patients, cardiac magnetic resonance imaging in 3 of 39 (8%) patients, and cardiac catheterization 6 of 39 (15%) patients.…”

“…The pathophysiology of acquired PVS in the preterm lung remains a matter of speculation, along with the factors promoting abnormal vascular connections in BPD (such as aorta‐pulmonary collaterals and intrapulmonary arterial‐venous anastomoses) . While the clinical status of infants and children with BPD and the severity of associated PH can both improve due to lung growth during the first year of life after premature birth, PVS does not resolve and may progress, explaining the high mortality of infants with PVS in our cohort. Our study highlights the importance of evaluating the pulmonary veins in this patient cohort during cardiac catheterization.…”

“…This is a single-center, retrospective cohort study. Patients were included if they were born at a GA of less than 32 weeks, had a diagnosis of BPD defined as oxygen requirement at 36 The transpulmonary gradient was calculated by subtracting the left atrial pressure from the mean PAP (in two infants, wedge pressure was used due to the inability to readily access the LA, none of these two infants had pulmonary vein stenosis). Indexed PVR (PVRi) was calculated as the transpulmonary artery gradient divided by the pulmonary blood flow indexed to body surface area.…”

2017 pediatric pulmonology year in review part 2—neonatology

“…The contribution of pulmonary venous abnormalities to PH that complicates BPD is relatively under‐appreciated. Mahgoub and colleagues report describe a multi‐center cohort experience with the relatively uncommon but almost certainly under‐recognized entity of pulmonary vein stenosis (PVS) in 39 patients following preterm birth. The contribution of PVS to pulmonary hypertension often requires cardiac angiography (catheterization or contrast CT scan) to diagnose and is relatively easy to miss using echocardiography, since flow velocity in the affected veins is necessarily low.…”