Evaluating Failing Fontans for Heart Transplantation: Predictors of Death

Abstract

Background Late complications of the Fontan operation represent a significant management challenge. In general, failing Fontan patients have two modes of presentation: those with impaired ventricular function (IVF group) and those with preserved ventricular function but with failing Fontan physiology (protein-losing enteropathy-PLE, and plastic bronchitis-PB) (PVF group). In this study we evaluated whether failing Fontan patients referred for heart transplantation had a different outcome based on the mode of presentation. Methods We conducted a retrospective chart review of all Fontan patients evaluated for heart transplantation at a single institution from 1994–2008. Demographic, hemodynamic, and laboratory data were collected. Patients were stratified into IVF group or PVF group based on echocardiographic criteria. Descriptive statistics and Kaplan-Meier analysis were used for hypothesis testing. Results Thirty four Fontan patients were evaluated for heart transplantation. Based on echo description of systolic function, 18 were categorized as IVF and 16 patients as PVF. The IVF group had significantly lower cardiac index, SvO2, and significantly higher SVR compared to the PVF group (p<0.05). PLE or PB was present in 13/16 in the PVF group and 0/18 in the IVF group. Of the 34 patients, 20 received transplantation with similar rates amongst the IVF and PVF groups. Within one year from evaluation there were 2/18 deaths in the IVF group and 7/16 deaths in the PVF group (p=0.052) Conclusions Failing Fontans with PVF have decreased overall survival independent of whether they were transplanted. This trend indicates a need to improve the management and/or timing for transplantation amongst this population.

Transplantation in the single ventricle population

“…In our adult patients undergoing transplant for FFP, we identified preserved ventricular function as an independent predictor of mortality (24). Griffiths et al verified this in a younger cohort when they reported 1-year survival after OHT in impaired ventricular function patients at 88.9%, compared to 56.2% in those with preserved ventricular function, 80% of whom had PLE or PB (25).…”

“…PLE and PB present exclusively in patients with preserved ventricular function following Fontan completion (25). PLE is characterized by abnormal serum protein loss into the gut lumen.…”

Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

“…The relative infrequency of PLE, combined with our rudimentary knowledge of the interplay between the cardiovascular and lymphatic systems, has hindered understanding of the pathophysiologic processes underlying this disease, as well as the development of effective therapy. Transplantation is often the only reasonable option [5,6]. Historically, survival after diagnosis has been 50% at 5 years [4,7].…”

Management of the Fontan circulation in the adult patient

“…PLE prior to transplantation is associated with a poor long term outcome. (158,159) With improving early survival from staged Fontan surgery, there will be an inevitable increase in adults with failing Fontan circulations in whom transplantation will be considered. The prospect of a mechanical Fontan support device has been raised and a number of designs have undergone mathematical modeling (160,161) and animal studies.…”