Pulmonary vein stenosis in heart transplant patients

BACKGROUND: Pulmonary vein stenosis (PVS) is a rare pediatric condition associated with significant mortality and morbidity. PVS in patients following heart transplant (HT) has not yet been described. METHODS: Patients who had clinically significant PVS following a heart transplant during the time period of April 1, 2013 to April 30, 2023, at Seattle Children's Hospital were identified. Clinically significant PVS was defined as an atretic vein or a vein with a gradient of ≥4 mmHg across at least one vein by echocardiogram or during cardiac catheterization. Patients who had a diagnosis of PVS prior to their transplant were excluded. A total of six patients were identified. We collected clinical data on these patients from their pre-transplant course to their most recent status. RESULTS: The median age at HT was 7.5 months (range 2-13 months). The median time from HT to diagnosis of PVS was 3.5 months (range 0.3-13 months). At the last follow-up, the patients had had two to five pulmonary vein interventions, and there were no mortalities. The donor-to-recipient weight and total cardiac volume (TCV) ratios were less than 2.0 in five of six of the patients. CONCLUSIONS: PVS is a rare complication that is associated with patients who undergo HT during infancy. PVS develops soon after HT and screening should occur accordingly. Interestingly, high donor-to-recipient weight and TCV ratios are not necessarily associated with the development of PVS. Further work will need to be performed in order to determine the significance of PVS in post-HT patients.