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Distance remains a deciding factor in the allocation scheme for donor organs. Balancing the principles of utility, justice and respect for persons requires a careful and evolving understanding of the implications of allocation criteria. We aim to describe the patterns of procurement distance with a focus on pediatric (ped) recipients and the relationship to ischemic time and graft survival. The OPTN database was used to determine procurement distance for ped and adult transplants (tx) from 1/1/2015 to 4/1/2021. Distance was divided into five groups: 1) ≤250, 2) 251-500, 3) 501-1000, 4) 1001-1500, 5) ≥1501 miles, and examined for association (Chi-square Kruskall-Wallis test) with age, diagnosis, status, ischemic time and graft survival at 1, 5 and 10 years (logrank test). Kaplan-Meier curves were generated for adult and ped recipients. Procurement distance by group was longer for peds recipients (40, 41, 16, 2, and 0.5%) than for adults (69, 24, 6, 0.9, and 0.1%), p<0.0001. Ischemic time by group in peds (3.2, 3.9, 4.8, 6.0, and 7.7 hours) show progressive increase with the majority of group 3 < 5.4 hrs (med(IQR) = 4.7 (4.1,5.4). Average (mean, SD) distance for status 1A (345, 282.4) was longer than 1B (327.8, 286.5) p = 0.019, but not different from status 2 (357.7, 348.3). There was no difference in distance for cardiomyopathy (83.5%) vs congenital heart disease (81.1%) at < 500 miles. Adolescents (11-17 yrs) had shorter distance: 89.9% < 500 than children < 1 year: 74.3% < 500 (p<0.0001). Logrank analysis of 10-year graft survival showed no difference by distance in peds while there was a difference in adults [Fig]. There was no difference in graft survival by procurement distance in peds heart tx. Ischemic time for most patients in distance group 3 was < 5.4 hours. It is worth considering whether prioritizing ped status 2 patients < 250 miles and adult status 3-6 < 500 miles before status 1A at 501 - 1000 miles is the best strategy both ethically and practically. [ABSTRACT FROM AUTHOR]

Human Leukocyte Antigen (HLA) sensitization can adversely impact heart transplant (HT) outcomes. We evaluated the effect of panel-reactive antibody (PRA) and desensitization (DS) strategies on outcomes of pediatric HT recipients in a multicenter cohort. The Pediatric Heart Transplant Society (PHTS) registry was queried for all patients transplanted from January 2004-March 2021 and linked by date of birth, HT date, and center to the Pediatric Health Information System (PHIS). Class I and II PRA at listing, PRA at HT, underlying diagnosis, age, sex, and graft loss were abstracted from PHTS. Inpatient DS therapy and associated charges were assessed via PHIS. Listed patients were divided by PRA into Low (< median PRA) and High (≥ median PRA) cohorts for both Class I and II PRA. DS-related change in PRA category pre-HT as well as 10-year graft survival were recorded. e linked 3,229 HT recipients (87.5% of total PHTS cohort) with PHIS (45% female, 51% congenital heart disease). Among those with PRA > 10%, median PRA for Class I and II was 36% and 48%, respectively. There were 223 High Class I and 191 High Class II patients. Of those with PRA > 10%, 10.8% received some DS therapy, including IVIg, rituximab, bortezomib and/or plasmapheresis pre-HT. DS therapies did not significantly alter absolute PRA or drive a change in PRA category from High to Low (p>0.05). Patients with Low PRAs at listing had greater 10-year graft survival than those with High PRA (p<0.05). DS therapy did not improve graft survival among patients who were highly sensitized at listing (Figure). Median pharmacy DS charges were $9.9K (IQR $3.9K-$28.4K). Elevated PRA adversely impacts graft survival in pediatric HT recipients. Efficacy of DS remains questionable. In patients highly sensitized at listing, efforts at DS are not associated with improved outcomes. Given cost and morbidity associated with DS, careful assessment of risks and benefits for individual patients is warranted. [ABSTRACT FROM AUTHOR]

Prinzmetal angina is a rare cause of intermittent chest pain in paediatrics. Here, we report the case of a 2-year-old female who presented with episodic chest pain, malaise, diaphoresis, fatigue, and poor perfusion on exam. During her hospitalisation, these episodes were associated with significant low cardiac output as evidenced by lactic acidosis and low mixed venous oxygen saturations. Her workup revealed an actin alpha cardiac muscle 1 (ACTC1) gene mutation and associated left ventricular non-compaction with decreased systolic function. She was started on oral heart failure medications as well as a calcium channel blocker but continued to have episodes which were found to promptly resolve with nitroglycerine. She was ultimately listed for cardiac transplant given her perceived risk of sudden death.

Background: Fontan associated liver disease (FALD) potentially impacts Fontan patients undergoing heart transplant. This multi-center study sought to identify pre-transplant risk factors and characterize any post-transplant liver recovery in those patients undergoing heart-alone transplant., Methods: Review of Fontan patients at 12 pediatric institutions who underwent heart transplant between 2001-2019. Radiologists reviewed pre and post-transplant liver imaging for fibrosis. Laboratory, pathology and endoscopy studies were reviewed., Results: 156 patients underwent transplant due to decreased ventricular function (49%), protein losing enteropathy (31%) or plastic bronchitis (10%); median age at transplant was 13.6 years (interquartile range IQR 7.8, 17.2) with a median of 9.3 years (IQR 3.2, 13.4) between the Fontan operation and transplant. Few patients had pre-transplant endoscopy (18%), and liver biopsy (19%). There were 31 deaths (20%). The median time from transplant to death was 0.5 years (95% Confidence Interval CI 0.0, 3.6). The five-year survival was 73% (95% CI 64%, 83%). Deaths were related to cardiac causes in 68% (21/31) and infection in 6 (19%). A pre-transplant elevation in bilirubin was a predictor of death. Higher platelet levels were protective. Immediate post-transplant elevations in creatinine, AST, ALT, and INR were predictive of death. Advanced liver fibrosis identified on ultrasound, computed tomography, or magnetic resonance imaging was not predictive of death. Liver imaging suggested some improvement in liver congestion post-transplant., Conclusions: Elevated bilirubin, but not fibrosis on liver imaging, was associated with post-heart transplant mortality in Fontan patients in this multicenter retrospective study. Additionally, heart transplant may alter the progression of FALD., (© 2022 Wiley Periodicals LLC.)