Iron deficiency and anemia in pediatric dilated cardiomyopathy are associated with clinical, biochemical, and hematological markers of severe disease and adverse outcomes.

BACKGROUND: There is limited evidence regarding the prevalence and impact of iron deficiency (ID) in children with dilated cardiomyopathy (DCM). METHODS: Retrospective single-center review of all children between 2010 and 2020 with a diagnosis of DCM and complete iron studies. ID was defined as ≥2 of ferritin <20 µg/liter, iron <9 µmol/liter, transferrin >3 g/liter, or transferrin saturation (TSat) <15%. Clinical and laboratory indices and freedom from a composite adverse event (CAE) of mechanical circulatory support (MCS), heart transplant, or death were compared between children with and without ID. RESULTS: Of 138 patients with DCM, 47 had available iron studies. Twenty-nine (62%) were iron deficient. Children with ID were more likely to be receiving inotropes (17, 59%, p = 0.005) or invasive/noninvasive ventilation (13, 45%, p = 0.016) than those who were iron replete. They had a higher incidence of anemia (22, 76%, p = 0.004) and higher NT-proBNP (1,590 pmol/liter, IQR 456-3,447, p = 0.001). Children with ID had significantly less freedom from the CAE at 1-year (54% ± 10%), 2-years (45 ± 10), and 5-years (37% ± 11%) than those without (p = 0.011). ID and anemia were the only significant predictors of the CAE on univariate Cox regression. CONCLUSIONS: ID is highly prevalent in children with DCM. Iron studies are undermeasured in clinical practice, but ID is associated with severe heart failure (HF) and an increased risk of the CAE. The need for iron replacement therapy should be considered in children who present in HF with DCM.

Quality care close to home: Objectives and early outcomes of a second paediatric heart transplant service in Australia.

AIM: We describe the experience of a new paediatric heart transplant (HT) centre in Australia. New South Wales offers quaternary paediatric cardiac services including comprehensive care pre- and post-HT; however, perioperative HT care has previously occurred at the national paediatric centre or in adult centres. Internationally, perioperative HT care is highly protocol-driven and a majority of HT occurs in low volume centres. Establishing a low volume paediatric HT centre in New South Wales offers potential for quality HT care close to home. METHODS: Retrospective review of programme data for the first 12 months was undertaken. Patient selection was audited against the programme's intended initiation criteria. Longitudinal patient data on outcomes and complications were obtained from patient medical records. RESULTS: The programme's initial phase offered HT to children with non-congenital heart disease and no requirement for durable mechanical circulatory support. Eight patients met criteria for HT referral. Three underwent interstate transfer to the national paediatric centre. Five children (13-15 years, weight 36-85 kg) underwent HT in the new programme. Individual predicted 90-day mortality was 1.3-11.6%, with increased risk for recipients transplanted from veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and with restrictive/hypertrophic cardiomyopathies. Survival at 90 days and for duration of follow-up is 100%. Observed programme benefits include mitigation of family dislocation and improved continuity of care within a family-centred programme. CONCLUSION: Audit of the first 12 months' activity of a second paediatric HT centre in Australia demonstrates adherence to proposed patient selection criteria and excellent 90-day patient outcomes. The programme demonstrates feasibility of care close to home, providing continuity for all patients including those requiring increased rehabilitation and psychosocial support post-transplantation.