Listing and Transplanting Adults With Congenital Heart Disease

Abstract

Background-An increasing number of patients with congenital heart disease (CHD) are reaching adulthood and may require heart transplantation. The survival of these patients after listing and transplantation has not been evaluated. Methods and Results-A total of 41 849 patients (aged Ͼ18 years) were listed for primary transplantation during [1995][1996][1997][1998][1999][2000][2001][2002][2003][2004][2005][2006][2007][2008][2009]. Patients with a history of CHD (nϭ1035; 2.5%) were compared with those with other causes (non-CHD group) (nϭ40 814; 97.5%); 26 055 (62.3%) reached transplantation and were subdivided into those with (reoperation group; nϭ10 484; 40.2%) and without (nonreoperation group; nϭ15 571; 59.8%) a previous sternotomy. Survival on the waiting list was similar between groups, but mechanical ventricular assistance was not associated with superior survival to transplantation among CHD patients. CHD patients were more likely to have body mass index Ͻ18.5 at transplantation (PϽ0.0001), were younger, and had fewer comorbidities. Early mortality among patients with CHD was high (reoperation, 18.9% versus 9.6%; PϽ0.0001; nonreoperation, 16.6% versus 6.3%; PϽ0.0001), but by 10 years, overall survival was equivalent (53.8% versus 53.6%). Analysis was limited by the lack of specific information regarding the CHD diagnosis in most patients. Conclusions-Adults with CHD have high 30-day mortality but better late survival after heart transplantation. Mechanical circulatory assistance does not improve waiting list survival in these patients. This may be due to a combination of highly complex reoperative surgery and often poor preoperative systemic health. (Circulation. 2011;123:759-767.)

Transplantation in the single ventricle population

“…Early complications, relating to transplant complexity, contribute substantially to the mortality of both adults and children and have been reported across the literature (24,28,38). Survivors have comparable outcomes to all CHD recipients.…”

Management of advanced adult congenital heart disease

“…Davies, et al identified 1 035 adult patients with CHD who were listed for primary cardiac transplantation (2.5% of the total number). (78) They found that patients with CHD were on the waiting list for a longer time and, again demonstrated a higher early mortality in those with CHD (18.9% vs 9.6%) but an equivalent 10 year mortality (52.8% vs 53.6%). Similar results were also found by Patel, et al who further reported that patients with CHD who underwent transplantation had significantly longer intraoperative ischaemic times.…”

“…Two larger studies from the US examined all transplants recorded in a nationwide registry and database. (74,78) However, unlike Irving, et al, the type of CHD was not recorded. Davies, et al identified 1 035 adult patients with CHD who were listed for primary cardiac transplantation (2.5% of the total number).…”

“…(74) Transplantation in patients with ACHD, however, has previously been perceived to be associated with poor outcome, when compared to other indications (75,76) with Taylor, et al stating "the diagnosis of ACHD is a powerful risk factor for death at one year after transplant". (77) Problems specific to this population include complex anatomy often with multiple previous operations (74,76,78) and an elevated pulmonary vascular resistance (PVR), a known risk factor for early donor organ failure. (79) Further to these, HLA sensitisation is common, especially in those patients who have undergone previous surgery with a homograft.…”

“…(81) Despite these problems, the numbers of patients with CHD being referred for transplantation has increased over the past 15 years. (74,78) However, the overall proportion of transplants for underlying CHD remains small and account for just 2% of all transplants performed. (82) Thus, many studies are small, single centre studies.…”