An Analysis of 186 Transplants for Pediatric or Congenital Heart Disease: Impact of Pretransplant VAD.

BACKGROUND: We reviewed our management strategy and outcome data for all 181 patients with pediatric or congenital heart disease who received 186 heart transplants from January 1, 2011, to March 1, 2022, and evaluated the impact of pretransplant ventricular assist device (VAD). METHODS: Continuous variables are presented as mean (SD); median [interquartile range] (range). Categorical variables are presented as number (percentage). Univariable associations with long-term mortality were assessed with Cox proportional hazards models. Impact of pretransplant VAD on survival was estimated with multivariable models. RESULTS: Pretransplant VAD was present in 53 of 186 transplants (28.5%). Patients with VAD were younger (years): 4.8 (5.6); 1 [0.5-8] (0.1-18) vs 12.1 (12.7); 10 [0.7-17] (0.1-58); P = .0001. Patients with VAD had a higher number of prior cardiac operations: 3.0 (2.3); 2 [1-4] (1-12) vs 1.8 (1.9); 2 [0-3] (0-8); P = .0003. Patients with VAD were also more likely to receive an ABO-incompatible transplant: 10 of 53 (18.9%) vs 9 of 133 (6.8%); P = .028. Univariable associations with long-term mortality included: In multivariable analysis, pretransplant VAD did not impact survival while controlling for each one of the factors shown in univariable analysis to be associated with long-term mortality. Kaplan-Meier 5-year survival (95% CI) was 85.8% (80.0%-92.1%) for all patients, 84.3% (77.2%-92.0%) without pretransplant VAD, and 91.1% (83.1%-99.9%) with pretransplant VAD. CONCLUSIONS: Our single-institution analysis of 181 patients receiving 186 heart transplants for pediatric or congenital heart disease over 11.25 years reveals similar survival in patients with (n = 51) and without (n = 130) pretransplant VAD. The presence of a pretransplant VAD is not a risk factor for mortality after transplantation for pediatric or congenital heart disease.

Use of REGEN-COV in children after heart transplantation for treatment and post-exposure prophylaxis of COVID-19.

COVID-19 pandemic continues to evolve and new variants like Delta and Omicron have been discovered. REGEN-COV is a recombinant human monoclonal antibody to the spike protein of SARS-CoV-2 which received emergency use authorisation for treatment and post-exposure prophylaxis in patients with high risk of progression to severe disease. We review our experience with use of REGEN-COV in paediatric heart transplant patients.

Thenar Muscle Oxygen Saturation Using Vascular Occlusion Test: A Novel Technique to Study Microcirculatory Abnormalities in Pediatric Heart Failure Patients.

Heart failure (HF) is associated with microcirculatory changes secondary to neuro-humoral imbalance, vascular stiffness and increased sympathetic tone. Near Infra-Red Spectroscopy (NIRS) derived Thenar muscle tissue oxygenation levels (StO2) can provide an estimate of the functional status of microcirculation. There is a paucity of literature regarding evaluation of microcirculation in pediatric subjects with HF. We hypothesized that microcirculation and oxygen saturation dynamics as assessed by Thenar StO2 levels using vascular occlusion test (VOT) would be altered in HF subjects and that these changes may correlate with the severity of heart failure. We prospectively enrolled 60 pediatric subjects (29 healthy control, 31 HF). Baseline StO2 levels were measured using InSpectra™ StO2 probe placed over the Thenar eminence of right hand, followed by a VOT for 3 min, during which the changes in StO2 levels during the occlusion phase and post occlusion phase were recorded. Baseline Thenar StO2 levels (72 ± 8 vs 76 ± 5, p = 0.02) and time to baseline StO2 in seconds (150 ± 70 vs 200 ± 70, p = 0.007) were significantly lower in HF group compared to healthy control (HC). In addition, HF patients had a significantly lower trough StO2 (37 ± 9 vs 42 ± 11%, p = 0.04) and peak StO2 compared to HC (87 ± 8 vs 91 ± 5%, p = 0.01). However, there was no difference in the rate of desaturation, rate of resaturation or time to peak StO2 levels in between the 2 groups. Significant correlation was present between baseline Thenar StO2 levels and NYU Pediatric Heart Failure Index Score (NYU-PHFI) (p = 0.003). This study is the first to report an objective assessment of microcirculation and Thenar tissue oxygen dynamics in pediatric subjects with HF in comparison with HC. Our study suggests altered microcirculation and oxygenation patterns in these subjects as well as correlation with a validated pediatric heart failure clinical score. Large-scale prospective studies are needed to further study the utility of this novel technology in HF subjects.

End-organ recovery post-ventricular assist device can prognosticate survival.

BACKGROUND: This study examines our institutional ventricular assist devices (VADs) experience over two decades to understand trends towards predictors of mortality. METHODS: Retrospective study of patients aged 0-21years supported with a VAD from January 1996 to May 2015. Patient data was examined pre and post-VAD implant among survivors and non-survivors. RESULTS: Thirty-six patients identified (8 supported by Thoratec® VAD and 28 supported by EXCOR Berlin Heart®). Patient's diagnosis included dilated cardiomyopathy (DCM) (n=19,53%), congenital heart disease (CHD) (n=12,33%), and other (n=5,14%). Median age and body surface area (BSA) were 1.0years[0-7years] and 0.41[0.24-0.92], respectively. Survival to discharge was 75% with no deaths with DCM. The survival rate for patients with CHD was 42%. Univariate analysis showed diagnosis of CHD, smaller BSA and respiratory failure post-implant (Intermacs criteria) as risk factors for mortality. Median duration of VAD support was lower in non-survivors, 14 vs 63days (p=0.03). Renal function at time of transplant or death was normal/pRIFLE Risk category in 20(74%) of survivors and 2(22%) of non-survivors (p=0.06). Post-implant, peak total bilirubin in the first week trended lower in survivors (p=0.06). CONCLUSIONS: Persistent end-organ impairment in the first 2weeks after VAD placement could be a useful prognostic marker for survival to transplant.

Use of 320-detector computed tomographic angiography for infants and young children with congenital heart disease.

Pediatric patients with complex congenital heart disease (CHD) face a lifetime of treatment with interventional therapeutic and palliative procedures. Echocardiography remains the mainstay for noninvasive imaging of congenital heart lesions. This often is supplemented with diagnostic cardiac catheterization for additional anatomic and physiologic characterization. However, recent technological improvements in computed tomography (CT) and magnetic resonance imaging (MRI) have led to an increased focus on the use of these techniques given their better safety profile. This study aimed to review the authors' experience with a 320-slice multidetector CT scanner in the evaluation of CHD in children. This retrospective case study investigated 22 infants and young children with a provisional diagnosis of CHD. Their anatomic evaluation was performed using a 320-slice Aquilon ONE CT scanner. Of these 22 patients, 14 were examined without cardiac gating. This was subsequently modified to a prospective gated, targeted protocol to decrease the radiation dose. The images were interpreted by an experienced radiologist and a pediatric cardiologist. Continuous variables were expressed as mean and standard deviation or range, and the two imaging protocols were compared. A comparison of exposure rates with those from other pediatric studies that had used the 64-slice CT angiography also was performed. For the first group of patients, with nongated CT examinations, the mean effective whole-body radiation dose was 1.8 ± 0.71 millisieverts (mSv) (range, 0.96-3.2 mSv). For the second group, the mean was 0.8 ± 0.39 mSv (range, 0.4-1.5 mSv). Although the radiation dose was reduced dramatically, clinicians must be vigilant about the cumulative risk of radiation exposure.

Post-transplant lymphoproliferative disorder in children: recent outcomes and response to dual rituximab/low-dose chemotherapy combination.

PTLD is a major complication after transplantation. Treatment options for PTLD are not standardized, usually sequential, starting with reduction in immunosuppression. Recently, we have used a dual combination of rituximab and reduced dose chemotherapy (R/C) directly after failed RI. We retrospectively identified 30 pediatric PTLD cases across four organ systems at our center from 1995 to 2008. We assessed recent outcomes of PTLD in children, comparing the responses to different regimens. Two-yr failure-free survival was best in renal and heart recipients (80-88%), followed by liver (57%) and lung (0%). Of note, two patients were Epstein-Barr peripheral blood viral load low positive but tumor EBER negative. Three patients had no detectable viral load but were EBER positive. The R/C regimen (n = 8) had the highest CR rate (100%), low recurrence (12%) and lowest mortality (12%). Interferon (n = 4) had 75% CR, 33% recurrence and 25% mortality. Rituximab/prednisone (n = 5) had 80% CR, 50% recurrence and 20% mortality. Other chemotherapy (n = 7, including all 4 T-cell PTLDs) had 57% CR, 0% recurrence and 14% mortality. Direct dual R/C combination therapy after failed RI is effective and offers another treatment option for B-cell PTLD.

Biomedical and psychosocial evaluation of "cured" adults with congenital heart disease.

As a result of significant advances in diagnostic, surgical, interventional, and pharmacological approaches, up to 95% of infants born with a congenital heart defect now survive into adulthood and there are at least 800,000 adult congenital heart disease patients living in the United States. Unfortunately, many of these individuals consider themselves "cured" or "fixed" and might have the misperception of a cure for a variety of reasons. The "cured" label is problematic and congenital heart disease is most accurately considered a chronic condition. This article outlines the concerns associated with the cured label. This is followed by the presentation of 4 illustrating case studies. Members of an adult congenital cardiology healthcare team must be prepared to address the full spectrum of concerns faced by patients who experience unexpected health deterioration. This spectrum includes both biomedical and psychosocial factors.