Orthotopic heart transplantation for failing single ventricle physiology☆

Abstract

OHT for structural CHD with single ventricle physiology entails substantial early mortality while BDG enables the best transition to heart transplant. OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.

Changing Indications for Pediatric Heart Transplantation

“…32 Congenital heart disease itself has consistently been identified as a risk factor for early and midterm mortality after transplantation compared with patients with cardiomyopathy. 19,29,32,35,39,40,42,[92][93][94][95][96][97][98][99] The increased mortality occurs soon after transplantation and is likely the result of a combination of factors, including the debilitated state of the recipient, longer donor ischemic times, baseline renal insufficiency, a higher incidence of HLA-sensitized patients, and immune system dysfunction. In the Fontan patient, chronic hepatic congestion has been found to lead to fibrosis and ultimately cirrhosis.…”

“…Patients who have undergone the Fontan procedure have a higher posttransplantation mortality than patients with other types of congenital heart disease, with one series reporting a hazard ratio of 8 for early mortality. 29,35,37,98,105 In a report from 2006, 1-year posttransplantation survival was 77% in patients with a Fontan procedure, 85% in patients with other types of congenital heart disease, and 91% in those with cardiomyopathy. 32 One-year posttransplantation survival in patients who have undergone repair of corrected transposition of the great arteries, an atrial switch procedure, or an aortopulmonary shunt ranges between 80% and 100% in several small series.…”

Outcome of Listing for Cardiac Transplantation for Failed Fontan

“…Previous series have been limited to small numbers of patients and have reported significantly higher risks compared with patients with cardiomyopathies. 7,[13][14][15] The present study represents the first large, multi-institutional evaluation of the results of listing for transplantation in children and adolescents who had previously undergone a Fontan procedure. The purpose of this study was to determine the risk factors for death while awaiting transplantation and to examine the results after transplantation in Fontan patients compared with other pediatric heart transplant recipients.…”

Management of the Fontan circulation in the adult patient

“…(153,154) Early mortality is high (155)(156)(157) but later survival for those transplanted for impaired ventricular function is good. PLE prior to transplantation is associated with a poor long term outcome.…”