Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

Abstract

Introduction Treatment of adult congenital heart disease patients who require advanced therapies remains challenging due to high perioperative and wait‐list mortality and limited donors. Patients palliated with Fontan are at the highest risk of early mortality due to multiorgan involvement and few centers able to safely transplant them. We sought to evaluate the early outcomes of heart transplants in these adult Fontan patients. Methods Using the Nationwide Inpatient Sample database, we identified all adults aged at least 18 years old who underwent heart transplantation across U.S. hospitals from 2004 to 2014. We then identified those with specific ICD‐9 codes to include tricuspid atresia, hypoplastic left heart syndrome and common ventricle. Multivariate regression models were created to adjust for potential confounders. Results A total of 93 Fontan patients underwent heart transplant during the study time (0.5% of all heart transplants). Compared to non‐Fontan heart transplantations, Fontan patients were younger, with a higher incidence of liver disease and coagulopathy. Fontan patients receiving heart transplant had higher mortality during transplant hospitalization compared to non‐Fontan patients (26.3% vs 5.3% OR, 18.10, CI, 5.06‐65.0 P < .001). Extracorporeal membrane oxygenator (ECMO) usage and bleeding were also higher in the Fontan cohort with an OR of 5.30 (P = .016) and 5.32 (P = .015) for ECMO and bleeding, respectively. The remaining outcomes were similar for both cohorts. Conclusion Adults with Fontan palliation undergoing heart transplantation have exceptionally high inpatient mortality, which is nearly five times that of non‐Fontan heart transplant recipients, perhaps related to a delayed referral, surgical complexity, and coexistent, underrecognized liver failure.

Cardiac transplantation in adult congenital heart disease with prior sternotomy

“…In general, transfusion of blood and blood products is associated with an increased incidence of renal failure, infection, as well as respiratory, cardiac, and neurologic complications after cardiac surgery 10,19 . Although our study did not demonstrate significant differences in outcomes, previous studies have shown that OHT outcomes are worse for patients with SV physiology, compared to BV physiology 2,20 . Early referral before liver dysfunction is likely to reduce perioperative complications, especially in SV physiology patients 2 …”

“…10,19 Although our study did not demonstrate significant differences in outcomes, previous studies have shown that OHT outcomes are worse for patients with SV physiology, compared to BV physiology. 2,20 Early referral before liver dysfunction is likely to reduce perioperative complications, especially in SV physiology patients. 2 Liver congestion/cirrhosis is common in our SV cohort.…”

“…2,20 Early referral before liver dysfunction is likely to reduce perioperative complications, especially in SV physiology patients. 2 Liver congestion/cirrhosis is common in our SV cohort. There are little data with regard to the reversibility of liver cirrhosis after isolated OHT in Fontan patients.…”

“…In the recent era, an increasing number of patients with congenital heart disease have become adults as a result of improved surgical techniques and postoperative management. 1,2 Accordingly, the incidence of heart failure in adult congenital heart disease (ACHD) patients has continued to increase. 3,4 Orthotopic heart transplantation (OHT) is one of the effective treatment options for ACHD patients with end-stage heart failure.…”

Heart failure in congenital heart disease: management options and clinical challenges

“…After the initial 30 days patients with ACHD have a superior longterm survival compared with non-CHD adults [116]. In the Fontan population mortality remains high [117]. Identifying the appropriate time for successful heart transplant is one of the most difficult aspects of caring for adults with CHD.…”