Improving outcomes for transplantation in failing Fontan-what is the next target?

Abstract

Objective: To identify the key contributors to postoperative mortality in patients undergoing orthotopic heart transplantation (OHT) for late Fontan failure.

Methods: This retrospective review of failing Fontan patients who underwent OHT in our tertiary care center between 2007 and 2019 included adult patients with congenital heart disease and single ventricle physiology who were palliated with a Fontan circulation for >1 year. We excluded patients undergoing combined heart-liver transplantation.

Results: The study cohort comprised 31 patients, including 18 males (58.1%), with a mean weight of 58.4 kg, median age at Fontan of 6.9 years (interquartile range [IQR], 2-38 years), and a median age at OHT of 27.1 years (IQR, 16.7-53.3). Almost all (93.5%) of the patients were in New York Heart Association class III-IV, and the majority (74.2%) were in Interagency Registry for Mechanically Assisted Circulatory Support class 3. Overall survival at 30 days, 1 year, and 5 years after OHT was 81%, 71%, and 67%, respectively. Major intraoperative bleeding was associated with increased mortality after OHT (odds ratio, 30; 95% confidence interval, 2.8-322; P = .002). Neither preoperative systemic ventricular function nor the development of primary graft dysfunction (PGD) was significantly associated with postoperative death. Nevertheless, PGD determined significant morbidity of this population.

Conclusions: In our cohort, major intraoperative bleeding was the key factor associated with mortality after OHT for late Fontan failure. Novel strategies for the prevention and management of postoperative bleeding will improve outcomes in this group of patients.

Keywords: CHD, congenital heart disease; CPB, cardiopulmonary bypass; ECMO, extracorporeal membrane oxygenation; EF, ejection fraction; Fontan circulation; ICU, intensive care unit; IVF, impaired ventricular function; LV, left ventricular; MELD-XI, model for end-stage liver disease score excluding the international normalized ratio; OHT, orthotopic heart transplantation; PGD, primary graft dysfunction; PVF, Preserved ventricular function; RV, right ventricular; adult congenital heart disease; heart transplantation.

Graphical abstract

Kaplan–Meier survival curve according to major intraoperative bleeding (time in years).

Figure 1

Kaplan–Meier survival curve for the overall population of adult failing Fontan patients undergoing heart transplantation (time in years).

Figure 2

Effect of major intraoperative bleeding on survival after heart transplantation in adult failing Fontan patients (time in years).

Figure 3

Effect of acute graft dysfunction on survival after heart transplantation in adult failing Fontan patients (time in years).

Figure 4

Effect of acute graft dysfunction on survival after heart transplantation in adult failing Fontan patients (time in years).

Figure 5

Major intraoperative hemorrhage was the key factor associated with post–heart transplantation mortality in an adult failing Fontan population. This was reflected in a 30-day mortality rate of 57%.