Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements

Abstract

Objectives: Heart transplantation (HT) in children with heterotaxy may be affected by anomalous cardiac position, venous return, and splenic function. Outcomes are not well described. We compared post-HT outcomes, including survival and resource utilization, among recipients with congenital heart disease (CHD) in the presence and absence of heterotaxy. Methods: Using linked Pediatric Health Information System and Scientific Registry of Transplant Recipients data (2001-2016), we identified 177 HT recipients with heterotaxy. We compared post-HT outcomes to 1,202 non-heterotaxy recipients with CHD in multivariable regression models. Length of stay (LOS) and cost from HT to discharge were also compared. Results: Heterotaxy HT recipients were older (median age 5.1 vs. 1.6 years, p<0.001) and more often Black, Asian, Hispanic, or "other" non-Caucasian (54 vs. 32%, p<0.001). Heterotaxy was independently associated with increased mortality (HR 1.61, [95% CI 1.20-2.15], p=0.001), even among 6-month survivors (HR 2.00 [1.17-3.43], p=0.012). Heterotaxy recipients more commonly required dialysis (OR 2.64 [1.52-4.56], p=0.001) and cardiac reoperation (OR 2.36 [1.29-3.41], p=0.003) prior to discharge. They had longer ischemic times (19 additional minutes [10.4-27.6], p<0.001, adjusted for organ procurement distance), ICU LOS (16 vs. 13 days, p=0.012), and hospital LOS (median 26 vs. 23 days, p=0.005). Post-HT hospitalization costs were also greater

Expanding analytic possibilities in pediatric solid organ transplantation through linkage of administrative and clinical registry databases

“…This effort has allowed for novel analyses that would not be possible with either database in isolation. This includes an in‐depth assessment of costs and resource utilization, assessment of practice variation, and the ability to identify and describe outcomes for subpopulations of interest . We anticipate that the extension of this linkage to include other pediatric solid organ transplants will similarly expand analytic possibilities.…”

Predictive factors contributing to prolonged recovery in patients after Fontan operation

“…Thus, only two patients were diagnosed as HLHS in the present study, and one had Fontan take-down and one experienced prolonged postoperative recovery. Some studies found that heterotaxy was a risk factor for haemodynamic instability and poor prognosis in Fontan patients [ 18 , 19 ]. During this work, the incidence of heterotaxy in prolonged recovery group was higher than that in standard recovery group (17.1% vs 8.5%, P = 0.042).…”

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

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