Transplant and mechanical circulatory support in patients with adult congenital heart disease

Abstract

Advances in surgery and pediatric care over the past decades have achieved improved survival for children born with congenital heart disease (CHD) and have produced a large, growing population of patients with adult congenital heart disease (ACHD). Heart failure has emerged as the leading cause of death and a major cause of morbidity among the ACHD population, while as little evidence supports the efficacy of guideline-directed medical therapies in this population. It is increasingly important that clinicians caring for these patients understand how to utilize mechanical circulatory support (MCS) in ACHD. In this review, we summarize the data on transplantation and MCS in the ACHD-heart failure population and provide a framework for how ACHD patients may benefit from advanced heart failure therapies like transplantation and MCS.

Increased frequency of heart transplantation, shortened waitlist time and preserved post‐transplant survival in adults with congenital heart disease, on the new heart transplant allocation system

“…25 In fact, the diagnosis of CHD at the time of transplant is considered an independent risk factor for poor outcome at 30 days and 1-year post-transplantation. 5,26 Early mortality is likely related to increased complexity for surgery which could be overcome with surgeries occurring in high volume centers with experienced congenital transplant surgeons. Furthermore, the improved early post-transplant mortality in highly specialized CHD centers is likely due not only to the increased volume of patients but also to the participation of experienced adult congenital surgeons, who would anticipate and prepare for surgical challenges which are unique to the ACHD population.…”

“…Furthermore, the improved early post-transplant mortality in highly specialized CHD centers is likely due not only to the increased volume of patients but also to the participation of experienced adult congenital surgeons, who would anticipate and prepare for surgical challenges which are unique to the ACHD population. 5,7,27 The poor 1 year survival post-transplantation in the ACHD population has also been attributed to co-morbid conditions such as chronic kidney disease and/or liver disease, higher frequency of pulmonary hypertension, increased sensitization, higher proportion of prior sternotomies, and a longer ischemic time. 14,19,28 Thus, the changes in the heart transplant allocation system on the ACHD patients should not be evaluated only based on its effects on the waitlist, but also on the post-transplant outcomes.…”

“…However, approximately 40% of adult CHD (ACHD) patients die due to heart failure (HF) 3,4 . End‐stage heart failure ACHD patients may benefit from heart transplantation (HT) 5 . The proportion of ACHD who receive HT has nearly doubled over the last 20 years 6,7 .…”

“…3,4 End-stage heart failure ACHD patients may benefit from heart transplantation (HT). 5 The proportion of ACHD who receive HT has nearly doubled over the last 20 years. 6,7 Historically, ACHD patients listed for HT have longer waitlist times, increased waitlist mortality, and are less likely to be bridged with…”

“…mechanical circulatory support than patients with other etiologies for end-stage HF. 3,5,[8][9][10] In the absence of ACHD guidelines for appropriate timing for HT referral, it is possible that these patients may be referred for transplant too late. 10 Thus, ACHD patients may be at a disadvantage when compared with other end-stage HF patients.…”