Heart Transplantation in Children with Mitochondrial Disease

Abstract

Objectives: To compare the outcomes and comorbidities of children with mitochondrial disease undergoing heart transplantation with children without mitochondrial disease.

Study design: Using a unique linkage between the Pediatric Health Information System and Scientific Registry of Transplant Recipients databases, pediatric heart transplantation recipients from 2002 to 2016 with a diagnosis of cardiomyopathy were included. Post heart transplantation survival and morbidities were compared between patients with and without mitochondrial disease.

Results: A total of 1330 patients were included, including 47 (3.5%) with mitochondrial disease. Survival after heart transplantation was similar between patients with and without mitochondrial disease over a median follow-up of 4 years. Patients with mitochondrial disease were more likely to have a stroke after heart transplantation (11% vs 3%; P = .009), require a longer duration of mechanical ventilation after heart transplantation (3 days vs 1 day; P < .001), and have a longer intensive care unit stay after heart transplantation (10 vs 6 days; P = .007). The absence of a hospital readmission within the first post-transplant year was similar among patients with and without mitochondrial disease (61.7% vs 51%; P = .14). However, patients with mitochondrial disease who were readmitted demonstrated a longer length of stay compared with those without (median, 14 days vs 8 days; P = .03).

Conclusions: Patients with mitochondrial disease can successfully undergo heart transplantation with survival comparable with patients without mitochondrial disease. Patients with mitochondrial disease have greater risk for post-heart transplantation morbidities including stroke, prolonged mechanical ventilation, and longer intensive care unit and readmission length of stay. These results suggest that the presence of mitochondrial disease should not be an absolute contraindication to heart transplantation in the appropriate clinical setting.

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Figure 1:

Identifying the patient population Flow diagram describing the patient selection process. Abbreviation: MtD = mitochondrial disorder.

Figure 2:

Kaplan-Meier survival analysis comparing freedom from death or retransplantation between heart transplant recipients with and without mitochondrial disease.

Figure 3.

Kaplan-Meier survival curve comparing freedom from readmission in the first year after transplant between patients with and without mitochondrial disease.

Figure 4:

Kaplan-Meier survival analysis comparing freedom from death or retransplantation between heart transplant recipients with mitochondrial disease and age/support matched controls.