Beyond transplant: Roles of atrial septostomy and Potts shunt in pediatric pulmonary hypertension

Despite significant successes in treating children with pulmonary hypertension, many still die from their disease. Death most often results from right ventricular failure. Bilateral lung transplantation has historically been the ultimate palliation for children who develop heart failure despite maximal medical therapy. Creating an atrial level shunt has been advocated as a pre-transplant intervention to help reduce symptoms and perhaps serve as a bridge to transplant. More recently, the utilization of a pulmonary-to-systemic anastomosis, that is, Potts shunt, has demonstrated efficacy in ameliorating symptoms in children with severe, progressive disease. This improvement is often coincident with a reduction in expensive and onerous pulmonary hypertension specific medications. Improvement has been sustained over the years in some children, delaying the need for transplant consideration. Research is ongoing to determine more specific risks/benefits of using the Potts shunt in children with pulmonary hypertension both in the short term and long.