Compared with other patients on a waitlist for heart transplantation, adults with congenital heart disease have elevated risk for mortality while on the waitlist, an expert said at Cardiology 2018.
This suggests a separate waitlist for adults with congenital heart disease may be necessary, Victor Morell, MD, chief of the division of pediatric cardiothoracic surgery at Children’s Hospital of Pittsburgh of UPMC, said during a presentation.
“Adults with congenital heart disease are clearly unique because of their anatomy, physiology and previous surgical history; the lack of [ventricular assist devices] to support them so they can make it to transplant; their higher waiting list mortality; and their younger age,” Morell told Cardiology Today. “It is not fair to make them compete for organs on equal terms with other patients that do have more resources available to them such as VADs. This has to be more thoughtfully discussed nationally and internationally as the number of adults with congenital heart disease keeps growing.”
Approximately 10% to 20% of patients with congenital heart disease eventually require heart or heart-lung transplantation, but they “have unique characteristics that can make clinical management and assessment for cardiac transplantation challenging,” Morell said during the presentation.
Compared with other patients listed for heart transplantation, adults with congenital heart disease are younger; more likely to have single-ventricle physiology; have elevated infection and bleeding risks; have increased renal dysfunction; more likely to have had previous cardiac surgery; more likely to have anatomy that confers surgical complexity; and less likely to be on a VAD or inotropic agent when listed, Morell said.
However, he said, outcomes after transplantation are often better for adults with congenital heart disease vs. those without it.
“They have a higher perioperative mortality due to many factors, but they have a better long-term survival,” Morell told Cardiology Today. “They have a higher waiting list mortality, partially attributable to the lack of adequate VADs or the decreased use of VADs to support this complex and varied group of patients.”
There are a number of significant barriers facing this population, Morell said in an interview.
“Adults with congenital heart disease need to be transplanted in centers with expertise on the management of these patients,” he said. “It is possible that because of their younger age and complex anatomy/medical issues, they are not being referred in a timely fashion for transplant evaluation. There is lack of adequate VAD support, and the fact that a significant number of these patients have undergone several cardiac surgeries during their lifetime, have complex anatomy and are likely sensitized due to anti-[human leukocyte antigen] antibodies makes them higher risk.” – by Erik Swain
Morell V. Cardiomyopathy, Heart Failure and Transplantation. Presented at: Cardiology: 21st Annual Update on Pediatric and Congenital Cardiovascular Disease; Feb. 21-25, 2018; Scottsdale, Ariz.
Disclosure: Morell reports no relevant financial disclosures.