Hiding in Plain Sight: Pulmonary Vein Stenosis Following Pediatric Heart Transplantation

Pulmonary vein stenosis (PVS) after pediatric heart transplantation (PHT) is an observed phenomenon with previously unknown incidence, risk factors, treatment, and outcome.

This is a review of three recent publications describing PVS after PHT.

In total, 712 PHT recipients from four centers, over a combined 43 years, are reviewed. Thirty-one new cases of PVS after PHT, in addition to six patients with preexisting PVS, are described. PVS diagnosis occurred in the first year after PHT for most patients. Left-sided PVS were more than twice as common as right-sided PVS. Nearly half (43%) experienced multivessel PVS. Major risk factors of PVS after PHT included younger age, history of congenital heart disease (CHD), and history of anomalous pulmonary venous return. The treatment of PVS after PHT varied, reflecting uncertainty in the management of PVS generally. With a median follow-up of less than 3 years, 19% of patients with PVS after PHT died.

PVS after PHT complicated approximately 4.4% of cases in these reports. PVS is more common after PHT in younger patients with a history of CHD. PVS is generally diagnosed in the first year after PHT. We recommend careful evaluation for PVS in the first year after PHT in patients with known risk factors.