Transplantation
Pediatric lung transplantation has not seen the overall improvement in post-transplant survival seen in pediatric heart transplant cohorts. Overall, pediatric lung transplant has median survival of 5.4 years (ISHLT 2016 slides). In children with PVS, there is a high incidence of congenital heart disease and so heart lung transplant (HLTx) is often the only option. Median survival for HLTx due to congenital heart disease is 2.4 years, while transplantation due to pulmonary hypertension is 4.8 years (ISHLT 2016 slides). Waitlist mortality is a serious consideration due to scarcity of donor organs and lack of long term mechanical pulmonary support options in children typically less than 15kg.
Lung transplant outcomes for 20 children with PVS have been reported from one institution ( Bharat A, et al, 2014). They reported an overall 5 year survival of 67.3% for congenital PVS and 50.7% for those children with acquired PVS. Small numbers of patients at single institutions limits the ability to understand transplant practices, outcomes and risk factors associated with transplantation for pulmonary venous disease. Published data suggests lung transplant may be a viable option for certain children with pulmonary venous disease and studies examining larger cohorts are required.
Lung transplant outcomes for 20 children with PVS have been reported from one institution ( Bharat A, et al, 2014). They reported an overall 5 year survival of 67.3% for congenital PVS and 50.7% for those children with acquired PVS. Small numbers of patients at single institutions limits the ability to understand transplant practices, outcomes and risk factors associated with transplantation for pulmonary venous disease. Published data suggests lung transplant may be a viable option for certain children with pulmonary venous disease and studies examining larger cohorts are required.