Heart Transplant Outcomes in Children with Fontan-Associated Plastic Bronchitis: A Pediatric Heart Transplant Society Analysis

Abstract

Plastic bronchitis is a rare post Fontan complication with limited treatment options. Heart transplantation has evolved as a potential curative option, but outcomes have not been well-defined. This study aims to assess contemporary waitlist and post-transplant outcomes in patients with plastic bronchitis. All Fontan patients were identified in the Pediatric Heart Transplant Society database (2010-2018). Waitlist and post-transplant outcomes were compared between Fontan patients with and without plastic bronchitis. Competing outcomes and Kaplan Meier analyses were used to assess the impact of plastic bronchitis on waitlist and post-transplant survival. A secondary analysis excluded those with protein losing enteropathy (PLE) from the comparison cohort. Of 645 Fontan patients listed for heart transplant, 69 (8%) had plastic bronchitis. At listing, patients with plastic bronchitis were younger (9.2 vs 10.6 years, p=0.02), but had no other differences at baseline including hemodynamic data. Fewer Fontan patients with plastic bronchitis were listed in the more recent era (46 [15.4%] in 2010-2014 vs. 23 [6.6%] in 2015-2018, p<0.01). Overall, there was no difference in waitlist (p=0.30) or post-transplant (p=0.66) survival [Figure]. The results were similar after excluding patients with PLE. Contrary to prior reports, in this large series plastic bronchitis did not have a negative impact on survival to or after heart transplantation in Fontan patients. A 50% reduction in listing in the most recent era suggests improvement in medical management of this rare and life-threatening condition.