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We sought to develop and validate a new risk stratification score for mortality for children supported with a ventricular assist device (VAD). This retrospective, multicenter study used data from patients undergoing VAD implantation between April 2018 and February 2023 at 44 participating institutions in the Advanced Cardiac Therapies Improving Outcomes (ACTION) network. Multivariable Cox proportional-hazards modeled mortality after VAD implantation. A total of 1,022 patients were enrolled. The 1 year mortality was 19% (95% confidence interval [CI]: 16-23). The multivariable model was used to build the ACTION VADs risk stratification score with four components: ventilation, advanced organ support (dialysis or ECMO), diagnosis, and size (weight ≤5 kg). One point is added for each risk factor. Based on the sum of the risk factors, patients were classified into four classes: class 0-green (4% mortality at 1 year), class 1-yellow (16% mortality at 1 year), class 2-orange (21% mortality at 1 year), and class 3 or higher-red (42% mortality at 1 year). The score performed well, with area under the curve (AUC) of 0.72 and excellent calibration. The ACTION VADs score for mortality can be calculated easily and offers risk stratification and prognostic information for pediatric VAD candidates. This is the first validated risk assessment tool for pediatric mechanical circulatory support.
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Background: Traditionally, low cardiac output has been considered the primary hemodynamic driver of renal function and injury. Adult data suggest that central venous pressure (CVP) is a more important factor. Objectives: The authors hypothesized that in children with cardiovascular disease, higher CVP predicts lower estimated glomerular filtration rate (eGFR) and worsening renal function (WRF). Methods: We performed a single-center cohort study of patients aged 3 months to 21 years with biventricular circulation undergoing cardiac catheterization. Pearson's correlation and linear and Cox regression analyses were performed to determine associations with eGFR at the time of catheterization and WFR within 180 days after catheterization. Results: 312 patients had median age 7.9 years (IQR: 2.3 to 14.5 years), median eGFR 97 mL/min/1.73 m2 (IQR: 81-118 mL/min/1.73 m2), median CVP 7 mm Hg (IQR: 5-9 mm Hg), and median cardiac index 3.7 mL/min/m2 (IQR: 2.9-4.6 mL/min/m2). Nearly half (48%) were transplant recipients. In multivariable analysis, CVP was independently associated with eGFR (ß = -2.65; 95% CI: -4.02, -1.28; P < 0.001), as was being a transplant recipient (ß = -10.20; 95% CI: -17.74, -2.65; P = 0.008), while cardiac index was not. Fifty-one patients (16%) developed WRF. In a proportional hazards model adjusting for cardiac index, only higher CVP (HR: 1.10; 95% CI: 1.04-1.17; P = 0.002) and greater contrast volume by weight (HR: 1.05; 95% CI: 1.01-1.10; P = 0.021) predicted WRF. CVP ≥7 mm Hg likewise predicted WRF (HR: 2.57; 95% CI: 1.29-5.12; P = 0.007). Conclusions: Among children with a spectrum of cardiovascular disease, higher CVP is associated with lower eGFR and development of WRF, independent of cardiac index.
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PURPOSE OF REVIEW: While pediatric myocarditis incidence has increased since the coronavirus disease 2019 (COVID-19) pandemic, there remain questions regarding diagnosis, risk stratification, and optimal therapy. This review highlights recent publications and continued unanswered questions related to myocarditis in children. RECENT FINDINGS: Emergence from the COVID-19 era has allowed more accurate description of the incidence and prognosis of myocarditis adjacent to COVID-19 infection and vaccine administration as well that of multi-system inflammatory disease in children (MIS-C). As cardiac magnetic resonance technology has shown increased availability and evidence in pediatric myocarditis, it is important to understand conclusions from adult imaging studies and define the use of this imaging biomarker in children. Precision medicine has begun to allow real-time molecular evaluations to help diagnose and risk-stratify cardiovascular diseases, with emerging evidence of these modalities in myocarditis. SUMMARY: Recent information regarding COVID-19 associated myocarditis, cardiac magnetic resonance, and molecular biomarkers may help clinicians caring for children with myocarditis and identify needs for future investigations.
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COVID-19 , Miocarditis , Humanos , Miocarditis/diagnóstico , COVID-19/epidemiología , COVID-19/complicaciones , COVID-19/diagnóstico , Niño , SARS-CoV-2 , Biomarcadores , Imagen por Resonancia Magnética/métodos , Pronóstico , Síndrome de Respuesta Inflamatoria SistémicaRESUMEN
BACKGROUND: Much of our knowledge of organ rejection after transplantation is derived from rodent models. METHODS: We used single-nucleus RNA sequencing to investigate the inflammatory myocardial microenvironment in human pediatric cardiac allografts at different stages after transplantation. We distinguished donor- from recipient-derived cells using naturally occurring genetic variants embedded in single-nucleus RNA sequencing data. RESULTS: Donor-derived tissue resident macrophages, which accompany the allograft into the recipient, are lost over time after transplantation. In contrast, monocyte-derived macrophages from the recipient populate the heart within days after transplantation and form 2 macrophage populations: recipient MP1 and recipient MP2. Recipient MP2s have cell signatures similar to donor-derived resident macrophages; however, they lack signatures of pro-reparative phagocytic activity typical of donor-derived resident macrophages and instead express profibrotic genes. In contrast, recipient MP1s express genes consistent with hallmarks of cellular rejection. Our data suggest that recipient MP1s activate a subset of natural killer cells, turning them into a cytotoxic cell population through feed-forward signaling between recipient MP1s and natural killer cells. CONCLUSIONS: Our findings reveal an imbalance of donor-derived and recipient-derived macrophages in the pediatric cardiac allograft that contributes to allograft failure.
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Aloinjertos , Rechazo de Injerto , Trasplante de Corazón , Macrófagos , Humanos , Trasplante de Corazón/efectos adversos , Macrófagos/metabolismo , Rechazo de Injerto/inmunología , Rechazo de Injerto/genética , Masculino , Femenino , Niño , Preescolar , Miocardio/patología , Supervivencia de Injerto , Lactante , Células Asesinas Naturales/inmunología , Células Asesinas Naturales/metabolismo , AdolescenteRESUMEN
BACKGROUND: Despite growing cardiogenic shock (CS) research in adults, the epidemiology, clinical features, and outcomes of children with CS are lacking. OBJECTIVES: This study sought to describe the epidemiology, clinical presentation, hospital course, risk factors, and outcomes of CS among children hospitalized for acute decompensated heart failure (ADHF). METHODS: We examined consecutive ADHF hospitalizations (<21 years of age) from a large single-center retrospective cohort. Patients with CS at presentation were analyzed and risk factors for CS and for the primary outcome of in-hospital mortality were identified. A modified Society for Cardiovascular Angiography and Interventions shock classification was created and patients were staged accordingly. RESULTS: A total of 803 hospitalizations for ADHF were identified in 591 unique patients (median age 7.6 years). CS occurred in 207 (26%) hospitalizations. ADHF hospitalizations with CS were characterized by worse systolic function (P = 0.040), higher B-type natriuretic peptide concentration (P = 0.032), and more frequent early severe renal (P = 0.023) and liver (P < 0.001) injury than those without CS. Children presenting in CS received mechanical ventilation (87% vs 26%) and mechanical circulatory support (45% vs 16%) more frequently (both P < 0.001). Analyzing only the most recent ADHF hospitalization, children with CS were at increased risk of in-hospital mortality compared with children without CS (28% vs 11%; OR: 1.91; 95% CI: 1.05-3.45; P = 0.033). Each higher CS stage was associated with greater inpatient mortality (OR: 2.40-8.90; all P < 0.001). CONCLUSIONS: CS occurs in 26% of pediatric hospitalizations for ADHF and is independently associated with hospital mortality. A modified Society for Cardiovascular Angiography and Interventions classification for CS severity showed robust association with increasing mortality.
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Insuficiencia Cardíaca , Choque Cardiogénico , Adulto , Humanos , Niño , Choque Cardiogénico/epidemiología , Choque Cardiogénico/terapia , Choque Cardiogénico/etiología , Estudios Retrospectivos , Insuficiencia Cardíaca/epidemiología , Hospitalización , Factores de Riesgo , Mortalidad HospitalariaRESUMEN
BACKGROUND: In pediatrics, implantable continuous-flow ventricular assist devices (IC-VAD) are often used as a "temporary" support, bridging children to cardiac transplantation during the same hospital admission. METHODS: We conducted a retrospective review of our consecutive patients undergoing IC-VAD support at a tertiary pediatric heart center between 2008 and 2022. RESULTS: We identified 100 IC-VAD implant encounters: HeartWare HVAD (67; 67%), HeartMate II (17; 17%), and HeartMate 3 (16; 16%). The median (range) age, weight, and body surface area at implantation were 14.1 (3.0-56.5) years, 54.8 (13.3-140) kg, and 1.6 (0.6-2.6) m2, respectively. Cardiomyopathy (58; 58%) was the most common etiology, followed by congenital heart disease (37; 37%, including 13 single ventricle). At 6 months of IC-VAD support, 94 (94%) encounters achieved positive outcomes: ongoing support (59; 59%), transplant (33; 33%), and cardiac recovery (2; 2%). Eighty-two encounters (82%) resulted in home discharge with ongoing VAD support, including 38 (46%, out of 82) requiring readmission and 7 (9%, out of 82) resulting in death. There was a clinically significant decrease in morbidity rates before versus after home discharge: bleeding (1.55 vs 0.06), infection (0.84 vs 0.37), and stroke (0.84 vs 0.15 event per patient-year). Overall, 86 encounters (86%) reached positive end points at the latest follow-up (64 transplant, 15 ongoing support, and 7 recovery). Infection (29%; 4 of 14) was the most common cause of negative outcomes, followed by cerebrovascular accident (21%; 3), and unresolved frailty (21%; 3). The estimated overall survival at 1, 2, and 5 years was 90%, 86%, and 77%, respectively. CONCLUSIONS: This study suggests the feasibility of outpatient management of pediatric IC-VAD support. The ability to offer true long-term support maximizes the potential of IC-VAD support, not limited to a temporary bridging tool for heart transplantation.
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Cardiopatías Congénitas , Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Auxiliar , Accidente Cerebrovascular , Niño , Humanos , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/terapia , Corazón Auxiliar/efectos adversos , Resultado del Tratamiento , Trasplante de Corazón/efectos adversos , Estudios RetrospectivosRESUMEN
Adult patients on left ventricular assist device (LVAD) support have increased morbidity and mortality after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. There are no reported clinical data describing outcomes among pediatric patients on ventricular assist device (VAD) support infected with SARS-CoV-2. We conducted a retrospective study using the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry to evaluate patient characteristics and clinical outcomes after SARS-CoV-2 infection. A total of 22 children on VAD support (median age at infection 10.6 years) from 16 centers tested positive for SARS-CoV-2. Cardiomyopathy (59.1%) and congenital heart disease (40.9%) were the most common primary diagnoses. The type of support included LVAD in 19 (86.4%), biventricular assist device (BIVAD) in one (4.5%), and single ventricle VAD in two (9%) patients. At the time of infection, 50% were outpatients, 23% were inpatients on a general cardiology floor, and 27% were in the cardiac intensive care unit (CICU). Most patients (82%) were symptomatic at time of diagnosis, but only 13% required escalation of respiratory support, and 31% received SARS-CoV-2 therapies. Notably, no mortality occurred, and significant morbidity was rare after SARS-CoV-2 infection in pediatric patients on VAD support.
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COVID-19 , Insuficiencia Cardíaca , Corazón Auxiliar , Adulto , Humanos , Niño , Corazón Auxiliar/efectos adversos , Insuficiencia Cardíaca/terapia , Estudios Retrospectivos , Resultado del Tratamiento , SARS-CoV-2 , Sistema de RegistrosRESUMEN
Heart rate variability (HRV) is a noninvasive indicator of the health of neurocardiac interactions of the autonomic nervous system. In adults, decreased HRV correlates with increased cardiovascular mortality. However, the relationship between HRV and outcomes in children with acute decompensated heart failure (ADHF) has not been described. Patients < 21 years old hospitalized with ADHF from 2013 to 2019 were included (N = 79). Primary outcome was defined as death, heart transplant, or mechanical circulatory support (MCS). The median standard deviation of the R-to-R interval in 5-min intervals (SDNN) was calculated from telemetry data obtained across the first 24 h of admission. Patients who met the primary outcome had significantly lower median SDNN (13.8 [7.8, 29.1]) compared to those who did not (24.6 [15.3, 84.4]; p = 0.004). A median SDNN of 20 ms resulted in a sensitivity of 68% and specificity of 69%. Median SDNN < 20 ms represented decreased freedom from primary outcome (p = 0.043) and a hazard ratio of 2.2 in multivariate analysis (p = 0.016). Pediatric patients with ADHF who died, underwent heart transplant, or required MCS had significantly decreased HRV at presentation compared to those that did not. This supports HRV as a noninvasive tool to improve prognostication in children in ADHF.
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BACKGROUND: The significance of atypical infiltrates (eosinophils or plasma cells) on endomyocardial biopsy (EMB) after pediatric heart transplant (HTx) is not known. We hypothesized that atypical infiltrates are associated with worse post-HTx outcomes. METHODS: We performed a retrospective cohort study of consecutive patients <21 years old who underwent primary HTx between 2013 and 2017. Multiorgan transplants were excluded. The presence of atypical infiltrates and burden of atypical infiltrates (rare vs predominant) on EMB were recorded. Primary outcome was a composite of cardiac allograft vasculopathy, graft failure (relisting or retransplant), or death. Presence of atypical infiltrates was evaluated: (1) overall using Cox regression with time-dependent covariates and (2) if present by 1 year post-HTx using Kaplan-Meier analysis. RESULTS: Atypical infiltrates were present in 24 out of 95 patients (25%) and were associated with a higher likelihood of reaching the composite outcome (hazard ratio (HR) 6.22, 95% confidence interval (CI) 2.60-14.89, p < 0.0001). This persisted when controlling for rejection in multivariable analysis. There was also a greater risk of the composite outcome if ≥2 nonconsecutive EMBs had atypical infiltrates (HR 11.80, 95%CI 3.17-43.84, p = 0.0002) or if atypical infiltrates were the predominant feature on EMB (HR 30.58, 95%CI 9.34-100.06, p < 0.0001). Patients with atypical infiltrates by 1-year post-HTx had a 5-year freedom from the composite outcome of 48%, compared to 90% if no atypical infiltrates had been present by this timepoint (log rank p = 0.002). CONCLUSIONS: The presence of atypical infiltrates on EMB is associated with significantly worse outcomes in children following HTx. These patients require closer follow-up to assess for developing graft dysfunction.
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Trasplante de Corazón , Humanos , Niño , Adulto Joven , Adulto , Estudios Retrospectivos , Trasplante de Corazón/efectos adversos , Biopsia , Cateterismo Cardíaco , Rechazo de Injerto/epidemiología , Rechazo de Injerto/patologíaRESUMEN
BACKGROUND: Patients are usually maintained on at least 2 immunosuppressive drugs (ISDs) after the first year post heart transplant. Anecdotally, some children are switched to single-drug monotherapy (a single ISD) for various reasons and varying durations. Outcomes associated with differences in immunosuppression after heart transplantation are unknown for children. OBJECTIVES: A priori we defined a noninferiority hypothesis for monotherapy compared to ≥2 ISDs. The primary outcome was graft failure, a composite of death and retransplantation. Secondary outcomes included rejection, infection, malignancy, cardiac allograft vasculopathy and dialysis. METHODS: This international, multicenter, retrospective, observational cohort study used data from the Pediatric Heart Transplant Society. We included patients who underwent first-time heart transplant <18 years of age between 1999 and 2020 with ≥1 year of follow-up data available. RESULTS: Our analysis included 3493 patients with a median time post-transplant of 6.7 years. There were 893 patients (25.6%) switched to monotherapy at least once with the remaining 2600 patients always on ≥2 ISDs. The median time on monotherapy after the first year post-transplant was 2.8 years (range 1.1-5.9 years). We found an adjusted hazard ratio (HR) of 0.65 (95%CI: 0.47-0.88) favoring monotherapy compared to ≥2 ISDs (p = 0.002). There were no meaningful differences in the incidence of secondary outcomes between groups, except for a lower rate of cardiac allograft vasculopathy in patients on monotherapy (HR 0.58, 95%CI: 0.45-0.74). CONCLUSIONS: For pediatric heart transplant recipients placed on monotherapy, immunosuppression with a single ISD after the first year post-transplant was noninferior to standard therapy with ≥2 ISDs in the medium term. CONDENSED ABSTRACT: Some children are switched to a single immunosuppressive drug (ISD) for various reasons after heart transplant, but outcomes associated with differences in immunosuppression are unknown for children. We assessed graft failure in children on a single ISD (monotherapy) compared to ≥2 ISDs in a cohort of 3493 children with a first heart transplant. We found an adjusted hazard ratio of 0.65 (95%CI: 0.47-0.88) favoring monotherapy. We concluded that for pediatric heart transplant recipients placed on monotherapy, immunosuppression with a single ISD after the first year post-transplant was non-inferior to standard therapy with ≥2 ISDs in the medium term.
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Cardiopatías , Trasplante de Corazón , Niño , Humanos , Estudios Retrospectivos , Inmunosupresores/uso terapéutico , Terapia de Inmunosupresión , Estudios de Cohortes , Rechazo de Injerto/epidemiología , Rechazo de Injerto/prevención & control , Rechazo de Injerto/etiología , Receptores de TrasplantesRESUMEN
OBJECTIVE: We report the largest pediatric single-center experience with an Impella (Abiomed Inc) catheter-based axial pump support. METHODS: We conducted a retrospective cohort study of all patients with acute decompensated heart failure or cardiogenic shock requiring catheter-based axial pump support between October 2014 and February 2022. The primary outcome per individual encounter (hospital admission) was defined as bridge-to-recovery, bridge-to-durable ventricular assist device support, bridge-to-cardiac transplantation, or death at 6 months after catheter-based axial pump explantation. Adverse events were defined according to the Pediatric Interagency Registry for Mechanical Circulatory Support criteria. RESULTS: Our final study cohort included 37 encounters with 43 catheter-based axial pump implantations. A single catheter-based axial pump device was used for support in 33 encounters (89%), with 2 catheter-based axial pump devices used in 3 (8%) separate encounters and 3 catheter-based axial pump devices used in 1 (3%) encounter. The median [range] age, weight, and body surface area at implantation were 16.8 [6.9-42.8] years, 61.1 [23.1-123.8] kg, and 1.7 [0.8-2.5] m2, respectively. The predominant causes of circulatory failure were graft failure/rejection in 16 patients (43%), followed by cardiomyopathy in 7 patients (19%), arrhythmia refractory to medical therapies in 6 patients (16%), myocarditis/endocarditis in 4 patients (11%), and heart failure due to congenital heart disease in 4 patients (11%). Competing outcomes analysis showed a positive outcome with bridge-to-recovery in 58%, bridge-to-durable VAD support in 14%, and bridge-to-cardiac transplantation in 14% at 6 months. Fourteen percent of encounters resulted in death at 6 months. CONCLUSIONS: We demonstrate that catheter-based axial pump support in children results in excellent 1- and 6-month survival with an acceptable adverse event profile.
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Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Auxiliar , Humanos , Niño , Adolescente , Adulto Joven , Adulto , Estudios Retrospectivos , Resultado del Tratamiento , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/terapia , Choque Cardiogénico , CatéteresRESUMEN
AIMS: We set out to design a reliable, semi-automated, and quantitative imaging tool using cardiac magnetic resonance (CMR) imaging that captures LV trabeculations in relation to the morphologic endocardial and epicardial surface, or perimeter-derived ratios, and assess its diagnostic and prognostic utility. METHODS AND RESULTS: We queried our institutional database between January 2008 and December 2018. Non-compacted (NC)-to-compacted (C) (NC/C) myocardium ratios were calculated and our tool was used to calculate fractal dimension (FD), total mass ratio (TMR), and composite surface ratios (SRcomp). NC/C, FD, TMR, and SRcomp were assessed in relation to LVNC diagnosis and outcomes. Univariate hazard ratios with cut-offs were performed using clinically significant variables to find 'at-risk' patients and imaging parameters were compared in 'at-risk' patients missed by Petersen Index (PI). Ninety-six patients were included. The average time to complete the semi-automated measurements was 3.90 min (SEM: 0.06). TMR, SRcomp, and NC/C were negatively correlated with LV ejection fraction (LVEF) and positively correlated with indexed LV end-systolic volumes (iLVESVs), with TMR showing the strongest correlation with LVEF (-0.287; P = 0.005) and SRcomp with iLVESV (0.260; P = 0.011). We found 29 'at-risk' patients who were classified as non-LVNC by PI and hence, were missed. When compared with non-LVNC and 'low-risk' patients, only SRcomp differentiated between both groups (1.91 SEM 0.03 vs. 1.80 SEM 0.03; P = 0.019). CONCLUSION: This method of semi-automatic calculation of SRcomp captured changes in at-risk patients missed by standard methods, was strongly correlated with LVEF and LV systolic volumes and may better capture outcome events.
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No Compactación Aislada del Miocardio Ventricular , Imagen por Resonancia Cinemagnética , Humanos , Niño , Imagen por Resonancia Cinemagnética/métodos , Función Ventricular Izquierda , Valor Predictivo de las Pruebas , Imagen por Resonancia Magnética , Volumen SistólicoRESUMEN
BACKGROUND: There are scarce data describing outcomes of pediatric temporary ventricular assist device support. METHODS: A retrospective single-center study was conducted to review clinical outcomes of all consecutive patients with temporary ventricular assist device between 1996 and 2021. Given the complex clinical course in some patients requiring multiple temporary ventricular assist device runs, outcome analysis was based on "encounters" (hospitalizations with temporary ventricular assist device, regardless of the number of devices used). RESULTS: In total, 126 temporary ventricular assist devices were implanted in 108 patients, resulting in a total of 114 encounters: 70 (61%) extracorporeal centrifugal pumps and 44 (39%) catheter-based axial pumps. The median (range) age and weight at temporary ventricular assist device implant were 10.1 years (1 day to 42.8 years) and 33.6 (2.5-128) kg, respectively. Underlying etiologies of cardiac dysfunction were cardiomyopathy (34, 30%), cardiac transplant graft dysfunction (29, 25%), congenital heart disease (23, 20%; 9 single ventricle), myocarditis (22, 19%), and other (6, 5%). Interagency Registry for Mechanically Assisted Circulatory Support Profile was 1 in 75 (66%) and 2 in 39 (34%). Support configuration was left ventricular assist device (104, including 9 systemic ventricular assist devices), right ventricular assist device (5), and biventricular assist device (5). The median (range) support duration was 6 (1-61) days. Overall, 97 (85%) encounters reached a positive primary end point: bridge-to-recovery (55), bridge-to-bridge (31), and bridge-to-transplant directly with temporary ventricular assist device (11). Seventeen (15%) encounters resulted in death during temporary ventricular assist device support: multiorgan failure (12), stroke (4), and cardiac arrest (1). The 6-month survivals with catheter-based axial pumps and extracorporeal centrifugal pumps were 84% (95% confidence interval, 74-96) and 67% (95% confidence interval, 57-79), respectively (P = .08). The 1- and 5-year survivals of 82 hospital survivors were 90% and 84%, respectively. CONCLUSIONS: This study suggests temporary ventricular assist device support is feasible in children with favorable outcomes.
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Insuficiencia Cardíaca , Corazón Auxiliar , Niño , Humanos , Corazón Auxiliar/efectos adversos , Insuficiencia Cardíaca/terapia , Estudios Retrospectivos , Resultado del Tratamiento , Factores de TiempoRESUMEN
OBJECTIVES: Bivalirudin is a direct thrombin inhibitor that is being increasingly used for anticoagulation in children after ventricular assist device (VAD) implantation. While the data on bivalirudin use in pulsatile flow VADs are growing, reports on its use in patients on continuous flow (CF) VAD as well as comparisons of associated outcomes with unfractionated heparin (UFH) remain limited. DESIGN: Retrospective cohort study. SETTING: Single tertiary-quaternary referral center. PATIENTS: All patients less than 21 years old on CF-VAD support who received bivalirudin or UFH for anticoagulation between the years 2016 and 2020. INTERVENTIONS: Not applicable. MEASUREMENTS AND MAIN RESULTS: Clinical characteristics compared between the cohorts included time to target range of anticoagulation, markers of hemolysis, and prevalence of hemocompatibility-related adverse events such as major hemorrhagic complications, ischemic stroke, and pump thrombosis. In 42 unique patients (41 HeartWare HVAD [Medtronic, Minneapolis, MN], one HeartMate 3 LVAD [Abbott Laboratories, Abbott Park, IL]) during the study period, a total of 67 encounters of IV anticoagulation infusions (29 UFH and 38 bivalirudin) were retrospectively reviewed. In comparison with use of UFH, bivalirudin was associated with lesser odds of major bleeding complications (odds ratio [OR], 0.29; 95% CI, 0.09-0.97; p = 0.038). We failed to identify any difference in odds of major thrombotic complications (OR, 2.53; 95% CI, 0.47-13.59; p = 0.450). Eight of the patients (28%) on UFH were switched to bivalirudin due to hemorrhagic or thrombotic complications or inability to achieve therapeutic anticoagulation, while two of the patients (5%) on bivalirudin were switched to UFH due to hemorrhagic complications. Bivalirudin was used for a "washout" in eight cases with concern for pump thrombosis-six had resolution of the pump thrombosis, while two needed pump exchange. CONCLUSIONS: Use of bivalirudin for anticoagulation in patients on CF-VAD support was associated with lesser odds of hemorrhagic complications compared with use of UFH. Bivalirudin "washout" was successful in medical management of six of eight cases of possible pump thrombosis.
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Corazón Auxiliar , Trombosis , Adulto , Anticoagulantes/efectos adversos , Antitrombinas/efectos adversos , Niño , Corazón Auxiliar/efectos adversos , Hemorragia/inducido químicamente , Hemorragia/epidemiología , Heparina/efectos adversos , Hirudinas/efectos adversos , Humanos , Fragmentos de Péptidos/efectos adversos , Proteínas Recombinantes/efectos adversos , Estudios Retrospectivos , Trombosis/epidemiología , Trombosis/etiología , Trombosis/prevención & control , Resultado del Tratamiento , Adulto JovenRESUMEN
Background Children with Down syndrome (DS) have a high risk of cardiac disease that may prompt consideration for heart transplantation (HTx). However, transplantation in patients with DS is rarely reported. This project aimed to collect and describe waitlist and post- HTx outcomes in children with DS. Methods and Results This is a retrospective case series of children with DS listed for HTx. Pediatric HTx centers were identified by their participation in 2 international registries with centers reporting HTx in a patient with DS providing detailed demographic, medical, surgical, and posttransplant outcome data for analysis. A total of 26 patients with DS were listed for HTx from 1992 to 2020 (median age, 8.5 years; 46% male). High-risk or failed repair of congenital heart disease was the most common indication for transplant (N=18, 69%). A total of 23 (88%) patients survived to transplant. All transplanted patients survived to hospital discharge with a median posttransplant length of stay of 22 days. At a median posttransplant follow-up of 2.8 years, 20 (87%) patients were alive, 2 (9%) developed posttransplant lymphoproliferative disorder, and 8 (35%) were hospitalized for infection within the first year. Waitlist and posttransplant outcomes were similar in patients with and without DS (P=non-significant for all). Conclusions Waitlist and post-HTx outcomes in children with DS selected for transplant listing are comparable to pediatric HTx recipients overall. Given acceptable outcomes, the presence of DS alone should not be considered an absolute contraindication to HTx.
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Síndrome de Down , Cardiopatías Congénitas , Trasplante de Corazón , Niño , Síndrome de Down/complicaciones , Síndrome de Down/epidemiología , Femenino , Cardiopatías Congénitas/cirugía , Trasplante de Corazón/efectos adversos , Trasplante de Corazón/métodos , Humanos , Masculino , Estudios Retrospectivos , Resultado del Tratamiento , Listas de EsperaRESUMEN
BACKGROUND: Guidance and data on ventricular assist device (VAD) support for children with chemotherapy-induced cardiomyopathy, particularly within the first 2 years after chemotherapy, are limited. METHODS: We performed a single-center retrospective case series, reviewing medical records of children <18 years of age with chemotherapy-induced cardiomyopathy and advanced heart failure (HF) who received durable VAD support. RESULTS: Six patients met inclusion criteria-5 HeartWare™ HVAD, 1 Berlin Heart EXCOR® . Median age at cancer diagnosis was 6 years (IQR 4.5-10 years). Median dose of anthracycline received was 540 mg/m2 (IQR 450-630 mg/m2 ). All patients developed HF within 1 year after initiation of cancer treatment (median 8 months, IQR 6-11.5 months) and were initiated on durable VAD support at a median of 8 months after completion of cancer treatment (IQR 3.3-43.5 months). Four patients had significant right ventricular dysfunction needing oral pulmonary vasodilator therapy, one patient had a major bleeding complication, and two patients had thromboembolic strokes while on VAD support. Median duration of VAD support was 7.5 months (IQR 3-11.3 months). Two patients underwent VAD explant due to recovery of LV function, one died due to cancer progression, and three underwent heart transplantation. CONCLUSIONS: Durable VAD support should be considered as a therapeutic option for children who have advanced HF due to chemotherapy-induced cardiomyopathy, even within 2 years of completing cancer treatment. A multi-disciplinary approach is essential for appropriate patient selection prior to implant and to ensure comprehensive care throughout the duration of VAD support.
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Antineoplásicos , Cardiomiopatías , Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Auxiliar , Antineoplásicos/efectos adversos , Cardiomiopatías/inducido químicamente , Cardiomiopatías/terapia , Niño , Insuficiencia Cardíaca/etiología , Trasplante de Corazón/efectos adversos , Corazón Auxiliar/efectos adversos , Humanos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Understanding optimal ventricular assist device (VAD) parameters for pediatric patients is valuable given the inherent issue of patient-device size mismatch and heterogeneous cardiac anatomy in children. We evaluated our center's experience of continuous-flow VAD (CF-VAD) optimization using cardiac catheterization. We performed a retrospective analysis of all patients on CF-VAD support who underwent hemodynamic heart catheterization from 2013 to 2018. Fifteen patients had 16 hemodynamic catheterizations performed. The indications for hemodynamic optimization by catheterization included clinical signs of heart failure while on CF-VAD (9 of 16, 56%), pretransplant evaluation of pulmonary hypertension (2 of 16, 13%), or assessment of myocardial recovery (5 of 16, 31%). The median age at catheterization was 12 years (interquartile range: 8-16). Median baseline speed of device was 2333 ± 253 rotations per minute. The goal was to find the speed at which optimal hemodynamics were achieved, defined by low wedge pressure with an acceptable central venous pressure. Of the 16 catheterizations, there were 9 (56%) speed increases to achieve optimal hemodynamics and 5 (33%) speed decreases for hemodynamic optimization or for potential explant. The speed was not changed in 2 (13%) catheterizations as the patients were determined to be at an optimal hemodynamic state. Overall, VAD settings were optimized in 75% (14 of 16) of hemodynamic catheterizations. There were no adverse events related to catheterization. Thus, we conclude that catheterization-based hemodynamic assessment is safe and effective for optimizing VAD speed and provides guidance on medical management in children supported on CF-VAD.
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Insuficiencia Cardíaca , Corazón Auxiliar , Cateterismo Cardíaco , Niño , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/cirugía , Hemodinámica , Humanos , Estudios Retrospectivos , Función Ventricular IzquierdaRESUMEN
Leiomodin-2 (LMOD2) is an important regulator of the thin filament length, known to promote elongation of actin through polymerization at pointed ends. Mice with Lmod2 deficiency die around 3 weeks of age due to severe dilated cardiomyopathy (DCM), resulting from decreased heart contractility due to shorter thin filaments. To date, there have been three infants from two families reported with biallelic variants in LMOD2, presenting with perinatal onset DCM. Here, we describe a third family with a child harboring a previously described homozygous frameshift variant, c.1243_1244delCT (p.L415Vfs*108) with DCM, presenting later in infancy at 9 months of age. Family history was relevant for a sibling who died suddenly at 1 year of age after being diagnosed with cardiomegaly. LMOD2-related cardiomyopathy is a rare form of inherited cardiomyopathy resulting from thin filament length dysregulation and should be considered in genetic evaluation of newborns and infants with suspected autosomal recessive inheritance or sporadic early onset cardiomyopathy.