Pulmonary Hypertension in the Preterm Infant with Chronic Lung Disease can be Caused by Pulmonary Vein Stenosis: A Must-Know Entity

Abstract

Pulmonary hypertension (PHT) in the preterm infant is frequently due to chronic lung disease. Rarely, PHT can be caused by pulmonary vein (PV) stenosis that has been described to be associated with prematurity. This study is a retrospective analysis of all premature infants <37 weeks of gestation with PV stenosis and PHT in two French pediatric congenital cardiac centers from 1998 till 2015. Diagnosis, hemodynamics and outcome are described. Sixteen patients met the inclusion criteria. Median gestational age was 28 weeks (25 + 6-35) with a median birth weight of 842 g (585-1500). The majority of infants (87.5 %) had chronic lung disease and associated cardiac defects. Median age at diagnosis was 6.6 months (1.5-71). Fifty-six percentage (n = 9) had initially unilateral PV stenosis affecting in 89 % the left PV. Median initial invasive mean pulmonary artery pressure was 42 mmHg (25-70). Treatment options included surgical intervention (n = 6), interventional cardiac catheter (n = 3) and/or targeted therapy for pulmonary arterial hypertension (n = 5). In six patients, decision of nonintervention was taken. Global mortality was 44 %. All deaths occurred within 7 months after diagnosis regardless of chosen treatment option. Mean follow-up was 6 years (4.9 months-12 years). At last visit, all eight survivors were in stable clinical condition with five of them receiving targeted therapy for pulmonary arterial hypertension. PV stenosis is an unusual cause of PHT in the premature infant with chronic lung disease. Diagnosis is challenging since initial echocardiography can be normal and the disease is progressive. Treatment options are numerous, but prognosis remains guarded.

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

“…The striking predisposition of the left sided pulmonary veins to stenosis is noted again here . Compression of the left sided veins between the heart or aorta and the spine in the supine position, may account for the tendency of pulmonary vein stenosis to be first manifest on the left side.…”

“…Although pulmonary vein stenosis is a rare condition, it is emerging as a clinically important problem in pediatrics, particularly among ex‐premature infants who develop bronchopulmonary dysplasia . In addition, recent reports of surgical and catheter intervention for pulmonary vein stenosis have included more ex‐premature infants …”

Mortality in infants with bronchopulmonary dysplasia: Data from cardiac catheterization

“…year of life after premature birth, PVS does not resolve and may progress, 35,36 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.…”

“…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.…”

The impact of right ventricular pressure and function on survival in patients with pulmonary vein stenosis

“…It has been known since the 1950s that pulmonary vein stenosis (PVS) is associated with pulmonary hypertension (PH), 1 , 2 and multiple reports documenting pulmonary arterial pressures (PAP) with this condition have been published since. 3 – 7 However, very little information is available with respect to the impact of PAP or the ratio of PAP to systemic blood pressure 8 on right ventricular (RV) systolic function, survival, and clinical course. PVS patients are complex, variably exhibiting not only PH but also abnormal pulmonary parenchyma, abnormal distribution of pulmonary perfusion, and increased lung water.…”