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Background Adult congenital heart disease (ACHD) prevalence is increasing worldwide, with advanced heart failure (HF) as a leading cause of death. Limited data are available on durable mechanical circulatory support (MCS) in ACHD patients. Methods ACHD patients from the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) database were identified and propensity matched with non-ACHD patients using risk factors from the INTERMACS Seventh Annual Report. We compared these groups for the primary outcome of post-MCS mortality. We also investigated adverse event rates, functional status, and health-related quality of life. Results ACHD (n = 128) and non-ACHD (n = 512) patients were appropriately matched by baseline characteristics. ACHD patients had a longer length of stay at MCS implant (24 vs 19 days, p = 0.006) but similar rates of post-MCS adverse events and hospitalization. There were similar improvements in functional status and health related quality of life post-MCS in both groups. ACHD patients had significantly higher mortality post-MCS exclusively during the first 5 months after implant (p = 0.003) and a lower probability of receiving a transplant (p = 0.003). Risk factors for early mortality were biventricular or total artificial heart device implant and age > 50 years. Conclusions ACHD patients experience a higher early mortality after MCS but have similar adverse event rates and similar improvements in functional capacity and quality of life compared with non-ACHD patients. These data support expansion of MCS use in selected ACHD patients.

Purpose Transplantation (Tx) of young children with ABO incompatible (ABOi) hearts has evolved from an experimental approach to a routine procedure in many centers. Prior studies have shown equal survival after ABOi and ABO compatible (ABOc)-Tx, but also revealed altered B-cell response and less HLA sensitization following ABOi-Tx with yet unknown clinical impact. Using a large registry cohort, we investigated outcomes of ABOi recipients in comparison to all and directly matched ABOc recipients. Methods Data from the Pediatric Heart Transplant Society was analyzed comparing children Results ABOi-Tx was performed in 337 (16%) of children Conclusion In this large cohort of young children receiving ABOi-Tx in a clinical routine setting rates of graft survival, CAV and malignancy were equivalent to matched ABOc recipients. ABOi-Tx was associated with longer time to rejection, suggesting better graft acceptance associated with acquired blood group tolerance. ABOi-Tx was also associated with longer time to severe infection, potentially secondary to less rejection treatment. The benefits of ABOi-Tx seen in the matched cohort analysis persisted when analyzed against all ABOc-Tx children.

Purpose As heart transplant (HT) outcomes in Fontan patients have improved, there is interest in identifying markers of liver disease associated with increased mortality. In adults, an elevated Model for End Stage Liver Disease eXcluding INR (MELD-XI) score is associated with worse post-HT outcomes. We evaluated the relationship between MELD-XI scores at transplant and post-HT morbidity and mortality in Fontan patients. Methods The Pediatric Heart Transplant Society Registry was queried to identify pediatric Fontan patients listed for HT from 2005-2017. The MELD-XI score was calculated [MELD-XI= 5.112 x ln(total bilirubin) + 11.76 x ln(creatinine) + 9.44] at time of transplant. Those patients with a MELD-XI score at the time of HT above the 75thpercentile of the entire cohort were classified as the high MELD-XI score cohort; All others formed the low MELD-XI score cohort. Results Of 698 Fontan patients listed, 506 (73%) underwent HT. A score ≥ 11.5 identified the high MELD-XI score cohort. At HT, patients in the high MELD-XI cohort were more likely to be older (12 ±5.0 vs. 10.8 ±4.8 y, p=0.05), listed UNOS Status 1A (82% vs. 72%, p=0.05), on a ventilator (11% vs. 3%, p=0.001) and on ECMO (9.1% vs. 2.6%, p=0.009). There was no difference in time to first infection, treated rejection, CAV or malignancy between groups. However, both allograft and patient survival (Figure) was inferior in the high MELD-XI score cohort (p=0.02 and p=0.01 respectively). Multiphase parametric hazard modeling demonstrated that high MELD-XI score (HR=1.2, p Conclusion Pediatric Fontan patients with high MELD-XI scores and those who require mechanical circulatory support have increased mortality post-HT. Identification of Fontan patients with high MELD-XI scores during patient selection or while awaiting HT allows for active management of this risk factor which may reduce post-HT mortality.