Relationship of Post-Transplant Lymphoproliferative Disorders (PTLD) Subtypes and Clinical Outcome in Pediatric Heart Transplant Recipients: A Retrospective Single Institutional Analysis/Experience of 558 Patients.

Post-transplant lymphoproliferative disorders (PTLD) are heterogenous lymphoproliferative disorders that develop as a consequence of immunosuppression in transplant recipients. We sought to determine if subtypes of PTLD correlated with different outcomes. We performed a retrospective review of PTLD occurring in pediatric heart transplant recipients. A total of 558 children and infants underwent cardiac transplantation at our institution between 1985 and 2019 and were followed until March 2021. Forty-nine of 558 patients developed PTLD (8.8%). As compared to older children (>one year of age), infant recipients (

Quality of life after Ross procedure in children.

INTRODUCTION: Health-related quality of life in children who have undergone the Ross procedure has not been well characterised. The aim of this study was to characterise health-related quality of life in this cohort and compare to children with other CHD. METHOD: In this cross sectional, single-centre study, health-related quality of life was assessed in patients who underwent a non-neonatal Ross procedure using the Pediatric Quality of Life Inventory. Ross cohort scores were compared with healthy norms, patients with CHD requiring no surgical intervention or had curative surgery (Severity 2, S2) and patients who were surgically repaired with ≥1 surgical procedure and with significant residual lesion or need for additional surgery (Severity 3, S3). Associations between Pediatric Quality of Life Inventory score and patient factors were also examined. RESULTS: 68 patients completed surveys. Nearly one-sixth of patients had overall scores below the cut-off for at-risk status for impaired health-related quality of life. There was no difference in overall health-related quality of life score between the Ross cohort and healthy children (p = 0.56) and S2 cohort (p = 0.97). Health-related quality of life was significantly higher in the Ross cohort compared to S3 cohort (p = 0.02). This difference was driven by a higher psychosocial health-related quality of life in the Ross cohort as compared to S3 cohort (p = 0.007). Anxiety scores were significantly worse in the Ross cohort compared to both S2 (p = 0.001) and S3 (p = 0.0017), respectively. CONCLUSION: Children who have undergone a Ross procedure report health-related quality of life equivalent to CHD not requiring therapy and superior to CHD with residual lesions. Despite these reassuring results, providers should be aware of potential anxiety among Ross patients.

Organ transplantation using COVID-19-positive deceased donors.

The COVID-19 pandemic has influenced organ transplantation decision making. Opinions regarding the utilization of coronavirus disease-2019 (COVID-19) donors are mixed. We hypothesize that COVID-19 infection of deceased solid organ transplant donors does not affect recipient survival. All deceased solid organ transplant donors with COVID-19 testing results from March 15, 2020 to September 30, 2021 were identified in the OPTN database. Donors were matched to recipients and stratified by the COVID-19 test result. Outcomes were assessed between groups. COVID-19 test results were available for 17 694 donors; 150 were positive. A total of 269 organs were transplanted from these donors, including 187 kidneys, 57 livers, 18 hearts, 5 kidney-pancreases, and 2 lungs. The median time from COVID-19 testing to organ recovery was 4 days for positive and 3 days for negative donors. Of these, there were 8 graft failures (3.0%) and 5 deaths (1.9%). Survival of patients receiving grafts from COVID-19-positive donors is equivalent to those receiving grafts from COVID-19-negative donors (30-day patient survival = 99.2% COVID-19 positive; 98.6% COVID-19 negative). Solid organ transplantation using deceased donors with positive COVID-19 results does not negatively affect early patient survival, though little information regarding donor COVID-19 organ involvement is known. While transplantation is feasible, more information regarding COVID-19-positive donor selection is needed.

Novel Multidisciplinary Management of Acute Kidney Injury After Infant Orthotopic Heart Transplantation.

Acute kidney injury following orthotopic heart transplantation in pediatric recipients is often multifactorial, requiring balance of immune suppression, nephrotoxic medication exposure, nutrition, and fluid status. Therapeutic options are often limited by patient size and hemodynamic stability. We describe a four-month, 4.9-kg female bridged by mechanical circulatory support to transplant after failed stage 1 palliation secondary to recurrent aortic stenosis and severe ventricular dysfunction. Posttransplant, kidney injury was managed by transcatheter relief of central obstruction from an anastomotic stricture and continuous renal replacement therapy, allowing uninterrupted immune suppression, medication, and nutrition delivery until sufficient recovery of renal function.

Heart transplantation in two adolescents with Danon disease.

Danon disease (DD) is an X-linked dominant disorder caused by a mutation in the lysosomal-associated membrane protein-2 (LAMP-2) gene coding for the LAMP-2 protein. We report two cases of successful heart transplantation (HT) in adolescent brothers with DD, including one who was bridged to HT for 34 days with a HeartWare left ventricular assist device. In both patients, the post-transplant course was complicated by profound skeletal muscle weakness that resolved with corticosteroid withdrawal. These cases highlight that both HT and ventricular assist device support are feasible in patients with DD. Corticosteroid use may exacerbate skeletal myopathy, and therefore, steroid minimization may be warranted whenever possible.

Use of Hemodynamic Ramp Study to Optimize Continuous-Flow Ventricular Assist Device in a Fontan Patient.

The utility of hemodynamic ramp studies in single ventricle patients supported by continuous flow left ventricular assist devices (CF-LVADs) is largely unknown. We report the novel use of the hemodynamic ramp test to optimize a 26 year-old Fontan-palliated single-ventricle patient supported with a continuous-flow ventricular assist device (Heartware HVAD). Modest improvements in Fontan hemodynamics resulted in significant and persistent clinical improvement and symptomatic relief.

Psychiatric disorder and incessant tachyarrhythmia in a child.

The case of a 14-year-old female with ectopic atrial tachycardia who had been followed for a history of anxiety and depression is presented. The patient was admitted to the intensive care unit after she attempted suicide, at which point there was electrocardiographic evidence of the atrial arrhythmia. During subsequent invasive electrophysiology study, a focus near the tricuspid annulus was localized and targeted for ablation, followed by resolution of the psychiatric symptoms. The case highlights the overlap between psychiatric complaints and true cardiac arrhythmia. A review of the literature is presented, with a special emphasis on distinguishing these two entities as well as a synopsis of this uncommon arrhythmia in the pediatric population.