O-1
Impact of a Dedicated Pulmonary Vein Stenosis Program on Survival
Presenter
Jay D Patel, MD, Children's Healthcare of Atlanta, Atlanta, GA
Jay D Patel, MD1, Mansi Mandhani, MBBS1, Rosemary Gray, RN1, Yijin Xiang, MPH1, R. Allen Ligon Jr., MD, FSCAI1, Holly Bauser-Heaton, M.D., Ph.D., FSCAI1, Dennis W. Kim, M.D., Ph.D., FSCAI1, James Kuo, M.D., FSCAI2 and Christopher J. Petit, M.D., FSCAI3, (1)Children's Healthcare of Atlanta, Atlanta, GA, (2)Children's Healthcare of Atlanta - Egleston, Decatur, GA, (3)NewYork-Presbyterian Morgan Stanley Children's Hospital, New York, NY
Keywords: Congenital Heart Disease (CHD), Drug-eluting Stent (DES) and Pharmacotherapy
Background:
Pulmonary vein stenosis (PVS) is a progressive disease associated with a high rate of mortality in infants and children. In recent years, there has been a trend to develop dedicated programs to improve outcomes for complex and progressive diseases like PVS. Our institution, is one of the first to have developed a dedicated PVS program. We sought to measure the impact of a dedicated PVS program on somatic growth and survival in infants and children diagnosed with PVS at our institution.
Methods:
In this single-center study, all patients with diagnosis of PVS between 2013-2020 were reviewed. Patients were divided into the pre-PVS program era (diagnosis between 2013-2015) and PVS program era (diagnosis between 2016-2020). The outcome measure of somatic growth rate was determined by plotting patient weight over time, and growth noted by change-in-weight-for-age Z-score (WAZ). Kaplan-Meier survival analysis was performed comparing patient survival in the pre-PVS program era versus PVS program era.
Results:
Between 2013-2020, our institution diagnosed and treated 131 patients with PVS. There were 50 patients treated in the pre-PVS program era and 80 patients treated in the PVS program era. Patients in the PVS program era were more likely to be on systemic sirolimus therapy (15 versus 1; p=0.005). Patients in the PVS program era also had a higher rate of cath interventions at 2.5 (Q1-Q3; 2.25-2.83) versus 1.6 (Q1-Q3; 1.4-1.86) and imaging surveillance at 7.24 (Q1-Q3; 6.76-7.74) versus 4.07 (Q1-Q3; 3.73-4.44). Weight gain in the PVS program era (9.05 g/day) outpaced the growth rate in the pre-PVS program era (6.95 g/day). Patients in the PVS program era had improved survival at 1 year follow-up (77% versus 61%) and 3 year follow-up (64% versus 49%; log-rank p=0.018).
Conclusions:
Patients treated in the PVS program era demonstrated improved somatic growth rate and survival. This improvement in somatic growth rate and survival correlated with more aggressive medical and anatomic therapy approaches offered during the PVS program era.