Outcome of Listing for Cardiac Transplantation for Failed Fontan

Abstract

Background-The Fontan procedure is a successful palliation for children with single-ventricle physiology; however, many will eventually require heart transplantation. The purpose of this study was to determine risk factors for death awaiting transplantation and to examine results after transplantation in Fontan patients. Methods and Results-A retrospective, multi-institutional review was performed of 97 Fontan patients Ͻ18 years of age listed at 17 Pediatric Heart Transplant Study centers from 1993 to 2001. Mean age at listing was 9.7 years (0.5 to 17.9 years); 25% were Ͻ4 years old; 53% were United Network for Organ Sharing status 1; 18% required ventilator support. Pretransplantation survival was 78% at 6 months and 74% at 12 months and was similar to 243 children with other congenital heart disease (CHD) and 747 children without congenital heart disease (No-CHD), who were also awaiting transplantation. Patients who were younger, status 1, had shorter interval since Fontan, or were on a ventilator were more likely to die while waiting. At 6 months, the probability of receiving a transplant was similar for status 1 and 2 (65% versus 68%); however, the probability of death was higher for status 1 (22% versus 5%). Seventy patients underwent transplantation. Survival was 76% at 1 year, 70% at 3 years, and 68% at 5 years, slightly less than CHD and No-CHD patients. Causes of death included infection (30%), graft failure (17%), rejection (13%), sudden death (13%), and graft coronary artery disease (9%). Protein-losing enteropathy (present in 34 patients) resolved in all who survived Ͼ30 days after transplantation. Conclusions-Heart transplantation is an effective therapy for pediatric patients with a failed Fontan. Although early posttransplantation survival is slightly lower than other patients with CHD, long-term results are encouraging, and protein-losing enteropathy can be expected to resolve. (Circulation. 2006;114:273-280.)

Transplantation in the single ventricle population

“…These patients, typically adolescents or adults, present late with insidious decline, often "tolerating" their symptomatology. PLE without transplantation carries a 50% mortality at 5 years; resolution of PLE post-OHT is reported in 100% of early survivors, highlighting its development as a compelling indication for transplant (26,27). However, destabilization of multiple homeostatic mechanisms leads to high peri-operative risk which must be considered when counselling patients (4,28).…”

“…However, recent interrogation of the Paediatric Heart Transplant Study Database showed improved 1-year survival from 71% [1993][1994][1995][1996][1997][1998][1999][2000][2001][2002][2003][2004][2005][2006] to 89% [2007][2008][2009][2010][2011][2012][2013][2014] for those undergoing OHT for FFP, reaching comparable outcomes to other CHD patients (42). Other paediatric registry data has shown comparable outcomes between Fontan and nonFontan SV OHT patients (27,43,44). In our own adult data of OHT for FFP, we have shown a 2-fold improvement in 30-day survival from 40% to 76% over similar eras (24).…”

“…This identifies HLHS as a sub-group in whom early failure could be anticipated, and primary OHT pursued. OHT as a rescue for early failure at less than 6 months following Fontan completion has been associated with higher mortality than OHT after Glenn shunt (27). Surgeons with growing experience, may push the boundaries of who and when to up-stage, resulting in a higher number of early Fontan failures being referred for transplant.…”

Heart Failure in Children

“…In the case of the Fontan patient, protein losing enteropathy is another important manifestation of the failing heart. 6 …”

Protein‐losing enteropathy in an adult with non‐ischaemic cardiomyopathy: complete reversal by heart transplantation

“…Because PLE has adverse prognostic consequences, reversibility of PLE in HF patients is critically important when considering radical HF therapy, such as cardiac transplantation. PLE has been shown to reverse after transplant in young patients with failed Fontan circulation,8 but its reversibility is not established in other conditions and in adults. Here, we report a patient in whom PLE with immune deficiency developed as a complication of hypokinetic non‐dilated cardiomyopathy, probably due to combination of increased ventricular stiffness and encircling epicardial electrode,9 that completely resolved after heart transplantation.…”