Dismal Lung Transplant Outcomes in Children With Tetralogy of Fallot With Pulmonary Atresia Compared to Eisenmenger Syndrome or Pulmonary Vein Stenosis

Lung transplantation in children with idiopathic pulmonary arterial hypertension: An 18-year experience

“…6 Children who underwent lung transplantation at our institution with associated congenital heart disease, such as pulmonary vein stenosis or Eisenmenger syndrome, fared slightly better than children with IPAH, with a median survival of 6.1 and 6.4 years, respectively. 8 Our median survival rate of 5.8 years is remarkably similar to that seen in adults who undergo lung transplantation for IPAH, who have a median survival of 5.6 years. 9 We assessed a number or pre-transplant variables for their impact on post-transplant outcomes.…”

“…This finding is consistent with our previous observation that showed a similar survival post-lung transplant in children with pulmonary vein stenosis (mainly infants) vs those with Eisenmenger syndrome (mainly teenagers). 8 Thus, age or size of a child should not be a barrier to consideration of lung transplantation for IPAH.…”

A Prospective Phase II Trial of Vinblastine and Methotrexate in Multivessel Intraluminal Pulmonary Vein Stenosis in Infants and Children

“…14 Although occasional survivors have been noted after aggressive surgical techniques, 16 only lung or heart-lung transplantation is considered effective. 17 We used chemotherapeutic agents as adjunct to surgery and catheter interventions to halt or slow the abnormal proliferation of myofibroblasts based on previous experience with the use of vinblastine and methotrexate in desmoid tumors of infancy, a benign tumor of myofibroblastic proliferation in infants. Patients in this trial were divided into two categories: patients with PVS occurring either as a complicating feature of complex CHD 18 or in isolation in infants with otherwise normal hearts.…”

Pediatric Lung Transplantation

“…Multiple arteriovenous collaterals, such as those seen in absent pulmonary artery syndromes, coupled with multiple prior thoracotomies have been associated with poor outcome and are a strong relative contraindication in many centers. 6,23 Significant allosensitization (related to prior thoracic surgeries or implantation of homograft valves or vessels) is also a relative contraindication because of the risk of hyperacute rejection or subsequent antibody-mediated allograft injury.…”