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2.
JACC Heart Fail ; 12(2): 380-391, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37676215

RESUMO

BACKGROUND: To date, no studies have identified an optimal metric to match donor-recipient (D-R) pairs in pediatric heart transplantation (HT). OBJECTIVES: This study sought to identify size mismatch metrics that predicted graft survival post-HT. METHODS: D-R pairs undergoing HT in Pediatric Heart Transplant Society database from 1993 to 2021 were included. Effects of size mismatch by height, weight, body mass index, body surface area, predicted heart mass, and total cardiac volume (TCV) on 1- and 5-year graft survival and morbidity outcomes (rejection and cardiac allograft vasculopathy) were evaluated. Cox models with stepwise selection identified size metrics that independently predicted graft survival. RESULTS: Of 7,715 D-R pairs, 36.0% were well matched (D-R ratio: -20% to +20%) by weight, 39.0% by predicted heart mass, 50.0% by body surface area, 57.0% by body mass index, 71.0% by height, and 93.0% by TCV. Of all size metrics, only D-R mismatch by height and TCV predicted graft survival at 1 and 5 years. Effects of D-R size mismatch on graft survival were nonlinear. At both 1 and 5 years post-HT, D-R undersizing and oversizing by height led to increased graft loss, with graft loss observed more frequently with undersizing. Moderately undersized donors by height (D-R ratio: <-30%) frequently experienced rejection post-HT (P < 0.001). Assessing D-R size matching by TCV, minimal donor undersizing was protective, while oversizing up to 25% was not associated with increased graft loss. CONCLUSIONS: In pediatric HT, D-R appear most optimally matched using TCV. Only D-R size mismatch by TCV and height independently predicts graft survival. Standardizing size matching across centers may reduce donor discard.


Assuntos
Insuficiência Cardíaca , Transplante de Coração , Obtenção de Tecidos e Órgãos , Humanos , Criança , Estudos Retrospectivos , Doadores de Tecidos , Sobrevivência de Enxerto
3.
J Heart Lung Transplant ; 42(12): 1743-1752, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37473824

RESUMO

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.


Assuntos
Transplante de Coração , Humanos , Criança , Adulto Jovem , Adulto , Estudos Retrospectivos , Transplante de Coração/efeitos adversos , Biópsia , Cateterismo Cardíaco , Rejeição de Enxerto/epidemiologia , Rejeição de Enxerto/patologia
4.
Pediatr Transplant ; 27(2): e14442, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36451335

RESUMO

BACKGROUND: Although ventricular failure is a late finding in adults with AC, we hypothesize that this is a presenting symptom in pediatric heart failure patients who undergo HT and that their ventricular arrhythmia burden could differentiate AC from other cardiomyopathies. METHODS: We performed a single-center retrospective cohort study reviewing 457 consecutive pediatric (≤18 years) HT recipients at our institution. Explanted hearts were examined to establish the primary diagnosis, based on pathologic findings. Demographic and clinical variables were compared between AC versus non-HCM cardiomyopathy cases. RESULTS: Forty-five percent (n = 205/457) had non-HCM cardiomyopathies as the underlying primary diagnosis. Ten cases (10/205 = 4.9%) were diagnosed with AC. All 10 had biventricular disease. In 8/10 patients (80%), AC diagnosis was unrecognized pre-HT. Compared with non-AC cardiomyopathies, the AC group was older at diagnosis (9.3 years vs. 4.3 years, p = .012) and transplant (11.1 years vs. 6.5 years, p = .010), had more ventricular arrhythmias (80.0% vs 32.8%, p = .003), and required more anti-arrhythmic use (80.0% vs 32.3%, p = .001). Genetic testing yielded causative pathogenic variants in all tested individuals (n = 5/5, 100%). CONCLUSION: AC is often an unrecognized cardiomyopathy pretransplant in children who undergo HT. Pediatric non-HCM phenotypes with heart failure who have a significant ventricular arrhythmia burden should be investigated for AC.


Assuntos
Cardiomiopatias , Insuficiência Cardíaca , Humanos , Estudos Retrospectivos , Cardiomiopatias/complicações , Cardiomiopatias/diagnóstico , Cardiomiopatias/patologia , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/cirurgia , Arritmias Cardíacas/complicações , Arritmias Cardíacas/diagnóstico , Antiarrítmicos
5.
Am J Cardiol ; 184: 72-79, 2022 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-36116954

RESUMO

Serum chloride plays an important role in fluid homeostasis and is associated with impaired diuretic responsiveness and mortality in adults with heart failure (HF). We sought to characterize the relationship of serum chloride and diuretic efficiency (DE) and to determine its prognostic importance in children hospitalized with acute decompensated HF (ADHF). We studied DE, defined as net fluid output/kg+constant per mg of loop diuretic/kg, in 200 children hospitalized with ADHF. Median serum chloride at admission was 102 mmol/L (interquartile range 99 to 105 mmol/L), and hypochloremia (chloride ≤96 mmol/L) was present in 16% of the population at admission. Serum chloride correlated with serum sodium (r = 0.66; p < 0.001) and bicarbonate (r = -0.39; p < 0.001). In the adjusted analysis, lower chloride was associated with reduced DE (p < 0.001). Serum sodium was associated with DE on the unadjusted analysis; however, the association was eliminated when added to the model with chloride (p = 0.442). Lower chloride was also associated with features of inadequate decongestion during hospitalization: a positive fluid balance (p = 0.003), greater cumulative loop diuretic dose per weight (p = 0.001), addition of a thiazide diuretic during hospitalization (p < 0.001), less weight loss (p = 0.025), and longer length of stay (p = 0.003). Chloride concentration was independently associated with death or transplant 1 year after admission (hazard ratio 0.94; p < 0.001). As a dichotomous variable, hypochloremia was independently associated with reduced DE (p < 0.001) and decreased 1-year transplant-free survival (hazard ratio 2.3, p < 0.001). Lower serum chloride at hospital admission is strongly and independently associated with impaired DE and reduced transplant-free survival in children hospitalized with ADHF.


Assuntos
Insuficiência Cardíaca , Desequilíbrio Hidroeletrolítico , Criança , Humanos , Inibidores de Simportadores de Cloreto de Sódio e Potássio/uso terapêutico , Cloretos , Hospitalização , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/cirurgia , Sódio , Diuréticos/uso terapêutico
6.
Pediatr Crit Care Med ; 23(10): e465-e475, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-35687091

RESUMO

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.


Assuntos
Coração Auxiliar , Trombose , Adulto , Anticoagulantes/efeitos adversos , Antitrombinas/efeitos adversos , Criança , Coração Auxiliar/efeitos adversos , Hemorragia/induzido quimicamente , Hemorragia/epidemiologia , Heparina/efeitos adversos , Hirudinas/efeitos adversos , Humanos , Fragmentos de Peptídeos/efeitos adversos , Proteínas Recombinantes/efeitos adversos , Estudos Retrospectivos , Trombose/epidemiologia , Trombose/etiologia , Trombose/prevenção & controle , Resultado do Tratamento , Adulto Jovem
7.
Pediatr Transplant ; 26(5): e14286, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35474630

RESUMO

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.


Assuntos
Antineoplásicos , Cardiomiopatias , Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Antineoplásicos/efeitos adversos , Cardiomiopatias/induzido quimicamente , Cardiomiopatias/terapia , Criança , Insuficiência Cardíaca/etiologia , Transplante de Coração/efeitos adversos , Coração Auxiliar/efeitos adversos , Humanos , Estudos Retrospectivos , Resultado do Tratamento
8.
ASAIO J ; 68(4): 584-591, 2022 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-35349525

RESUMO

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.


Assuntos
Insuficiência Cardíaca , Coração Auxiliar , Cateterismo Cardíaco , Criança , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/cirurgia , Hemodinâmica , Humanos , Estudos Retrospectivos , Função Ventricular Esquerda
9.
Am J Med Genet A ; 188(6): 1858-1862, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35188328

RESUMO

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.


Assuntos
Cardiomiopatias , Cardiomiopatia Dilatada , Citoesqueleto de Actina/genética , Animais , Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/genética , Proteínas do Citoesqueleto/genética , Coração , Humanos , Recém-Nascido , Camundongos , Proteínas Musculares/genética , Sarcômeros
10.
Cardiol Young ; 32(8): 1302-1309, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34629125

RESUMO

INTRODUCTION: Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure. METHODS: We performed a single-centre retrospective cohort study of patients aged ≤21 years with systolic heart failure and iron deficiency who received oral iron between 01/2013 and 04/2019. Iron deficiency was defined as ≥2 of the following: serum iron <50 mcg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, transferrin saturation <15%. Iron studies and haematologic indices pre- and post-iron therapy were compared using paired-samples Wilcoxon test. RESULTS: Fifty-one children with systolic heart failure and iron deficiency (median age 11 years, 49% female) met inclusion criteria. Heart failure aetiologies included cardiomyopathy (51%), congenital heart disease (37%), and history of heart transplantation with graft dysfunction (12%). Median dose of oral iron therapy was 2.9 mg/kg/day of elemental iron, prescribed for a median duration of 96 days. Follow-up iron testing was available for 20 patients, of whom 55% (11/20) remained iron deficient despite oral iron therapy. CONCLUSIONS: This is the first report on the efficacy of oral iron therapy in children with heart failure. Over half of the children with heart failure did not respond to oral iron and remained iron deficient.


Assuntos
Anemia Ferropriva , Insuficiência Cardíaca Sistólica , Insuficiência Cardíaca , Deficiências de Ferro , Adulto , Anemia Ferropriva/tratamento farmacológico , Anemia Ferropriva/etiologia , Criança , Feminino , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca Sistólica/complicações , Insuficiência Cardíaca Sistólica/tratamento farmacológico , Humanos , Ferro/uso terapêutico , Masculino , Estudos Retrospectivos , Transferrina/uso terapêutico
11.
Pediatr Transplant ; 26(1): e14149, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34585490

RESUMO

BACKGROUND: Pediatric sHKTx remains uncommon in the US. We examined outcomes of pediatric sHKTx compared to PHTx alone. Our objective was to identify a threshold eGFR that justified pediatric sHKTx. METHODS: Data from the SRTR heart and kidney databases were used to identify 9245 PHTx, and 63 pediatric sHKTx performed between 1992 and 2017 (age ≤21 years). RESULTS: The median age for sHKTx was 16 years, and included 31 males (31/63 = 49%). Over half of sHKTx (36/63 = 57%) were performed in cases where pretransplant dialysis was initiated. Among patients who required pretransplant dialysis, the risk of death in sHKTx recipients was significantly lower than PHTx alone (sHKTx vs. PHTx: HR 0.4, 95% CI [0.2, 0.9], p = .01). In those without pretransplant dialysis, there was no improvement in survival between sHKTx and PHTx (p = .2). When stratified by eGFR, PHTx alone recipients had worse survival than sHKTx in the group with eGFR ≤35 ml/min/1.73 m2 (p = .04). The 1- and 5-year actuarial survival rates in pediatric sHKTx recipients were 87% and 81.5% respectively and was similar to isolated PHTx (p = .5). One-year rates of treated heart (11%) and kidney (7.9%) rejection were similar in sHKTx compared to PHTx alone (p = .7) and pediatric kidney transplant alone (p = .5) respectively. CONCLUSION: Pediatric sHKTx should be considered in HTx candidates with kidney failure requiring dialysis or eGFR ≤35 ml/min/1.73 m2 . The utility of sHKTx in cases of kidney failure not requiring dialysis warrants further study.


Assuntos
Insuficiência Cardíaca/cirurgia , Transplante de Coração/métodos , Falência Renal Crônica/cirurgia , Transplante de Rim/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Taxa de Filtração Glomerular , Sobrevivência de Enxerto , Insuficiência Cardíaca/complicações , Transplante de Coração/mortalidade , Humanos , Lactente , Estimativa de Kaplan-Meier , Falência Renal Crônica/complicações , Falência Renal Crônica/mortalidade , Falência Renal Crônica/terapia , Transplante de Rim/mortalidade , Modelos Logísticos , Masculino , Diálise Renal , Estudos Retrospectivos , Resultado do Tratamento
12.
J Heart Lung Transplant ; 41(2): 133-136, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34911654

RESUMO

BACKGROUND: BACKGROUND: There is a paucity of data regarding the antibody response to SARS-CoV-2 vaccination in children after solid organ transplant. METHODS: We retrospectively reviewed the SARS-CoV-2 Anti-Spike IgG antibodies measured following SARS-CoV-2 vaccination at our pediatric heart transplant (HTx) center. RESULTS: Among patients (median age 17.1 years) in whom antibody testing was performed (median 118 days post-vaccine completion), a SARS-CoV-2 Anti-Spike IgG antibody was detected in 28 of 40 (70%) post-HTx recipients (median antibody level 10.9 AU/ml). Neutropenia, diabetes mellitus, and previous use of rituximab were associated with absence of a detectable antibody. All 7 post-HTx patients with a known pre-vaccination SARS-CoV-2 viral infection had a detectable SARS-CoV-2 Anti-Spike IgG. All 12 vaccinated pre-HTx patients had a detectable antibody (median antibody level 11.6 AU/ml) including 5 patients that maintained detectable antibodies post-HTx. There were no cases of myocarditis among the total of 17 pre-HTx and 81 post-HTx patients that underwent SARS-CoV-2 vaccination. CONCLUSION: Our data suggest that a significant proportion of pediatric HTx recipients have no detectable antibody response after SARS-CoV-2 vaccination and support the recommendation to complete the vaccination series prior to HTx in those pediatric patients waiting for HTx.


Assuntos
Anticorpos Antivirais/sangue , Vacinas contra COVID-19 , COVID-19/prevenção & controle , Transplante de Coração , SARS-CoV-2/imunologia , Glicoproteína da Espícula de Coronavírus/imunologia , Adolescente , Fatores Etários , Formação de Anticorpos , COVID-19/sangue , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Adulto Jovem
13.
Pediatr Transplant ; 26(3): e14212, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34921483

RESUMO

BACKGROUND: Valvular disease in pediatric and young adult donor hearts may be a relative contraindication to graft use. Outcomes following the use of donor hearts with bicuspid aortic valve (BAV) have not been previously reported in children. We describe 4 cases of pediatric heart transplantation (HTx) utilizing a donor heart with a BAV. CASE SERIES: Of the 469 HTx included in this study, 4 utilized a donor heart with a BAV. All recipients were female; median age was 11 years (range 0.3 to 19 years). In all cases, the BAV was not discovered until after HTx. All donors were less than 30 years old. The patients were followed for a median of 6 years (range 2 to 9 years) with all patients alive at last follow-up. Two patients have transitioned to adult care, and 2 patients continue to follow in our clinic. In follow-up, no patient has required an aortic valve intervention or had infective endocarditis. At last review, no patient had greater than mild aortic insufficiency or more than mild aortic stenosis. Three patients developed mild-to-moderate left ventricular hypertrophy in the first year post-transplant that improved over time. One patient experienced a peri-operative embolic stroke at time of transplant unrelated to the BAV. CONCLUSION: On short- and intermediate-term follow-up, pediatric and young adult donor hearts with BAV demonstrated acceptable graft longevity and valvular function. A functionally normal BAV in a pediatric heart transplant donor should not be a contraindication to organ acceptance.


Assuntos
Doença da Válvula Aórtica Bicúspide , Transplante de Coração , Doenças das Valvas Cardíacas , Adolescente , Adulto , Valva Aórtica/cirurgia , Criança , Pré-Escolar , Feminino , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Doadores de Tecidos , Adulto Jovem
14.
ASAIO J ; 68(9): 1182-1190, 2022 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34967787

RESUMO

Data are limited regarding body mass index (BMI) in pediatric patients supported by ventricular assist devices (VAD) and associated clinical outcomes and complications. We performed a retrospective single-center cohort study including patients aged ≤21 years on durable continuous-flow VAD support for ≥30 days from 2009 to 2020. Patients were classified based on BMI percentile at implant using the US Centers for Disease Control and Prevention criteria: underweight (<5th percentile), healthy weight (5th-<85th percentile, reference group), overweight (85th-<95th percentile), and obese (≥95th percentile). Primary outcomes were hospital mortality and length of stay (LOS) after implant. Secondary outcomes included infectious complications and pump thrombosis. Seventy-two patients (58 HeartWare, 13 HeartMateII, 1 HeartMate3) were included. At implant, the study cohort comprised 13% underweight, 53% healthy weight, 18% overweight, and 17% obese. BMI increased across all categories during support, with 29% gaining BMI categories. No patients with obesity reduced their BMI category. At explant, the study cohort comprised 1% underweight, 54% healthy weight, 22% overweight, and 22% obese. There was no significant difference in hospital mortality, postoperative LOS, or pump thrombosis. Patients who were overweight had more frequent non-VAD infections. Patients with obesity required longer duration on VAD support and were less likely to be transplanted. We concluded that pediatric patients on VAD support who are overweight or have obesity do not improve their BMI and instead have significant increase. Larger studies are needed to assess the impact of abnormal BMI on VAD complications in pediatric patients.


Assuntos
Coração Auxiliar , Sobrepeso , Índice de Massa Corporal , Criança , Estudos de Coortes , Coração Auxiliar/efeitos adversos , Humanos , Obesidade/complicações , Sobrepeso/complicações , Estudos Retrospectivos , Magreza/complicações
15.
Children (Basel) ; 8(6)2021 Jun 02.
Artigo em Inglês | MEDLINE | ID: mdl-34199474

RESUMO

Heart failure is a life-changing diagnosis for a child and their family. Pediatric patients with heart failure experience significant morbidity and frequent hospitalizations, and many require advanced therapies such as mechanical circulatory support and/or heart transplantation. Pediatric palliative care is an integral resource for the care of patients with heart failure along its continuum. This includes support during the grief of a new diagnosis in a child critically ill with decompensated heart failure, discussion of goals of care and the complexities of mechanical circulatory support, the pensive wait for heart transplantation, and symptom management and psychosocial support throughout the journey. In this article, we discuss the scope of pediatric palliative care in the realm of pediatric heart failure, ventricular assist device (VAD) support, and heart transplantation. We review the limited, albeit growing, literature in this field, with an added focus on difficult conversation and decision support surrounding re-transplantation, HF in young adults with congenital heart disease, the possibility of destination therapy VAD, and the grimmest decision of VAD de-activation.

16.
Pediatr Transplant ; 25(7): e14066, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34120386

RESUMO

BACKGROUND: Pediatric HLT remains uncommon in the United States and criteria for HLT are unclear. The objectives of this study were to review the indications, and outcomes of pediatric HLT. METHODS: Data from the Scientific Registry of Transplant Recipients heart and liver databases were used to identify 9245 pediatric isolated heart transplants (PHT), 14 134 pediatric isolated liver transplant (PLT), and 20 pediatric HLT (16 patients underwent sHLT [same organ donor] and four patients with a history of PHT followed by PLT [different organ donors]; age ≤21 years) between 1992 and 2017. Outcomes included patient survival, and 1-year rates of acute heart and liver rejection. RESULTS: The median age for pediatric HLT was 15.6 (IQR: 10.5, 17.9) years, and included 12 males (12/20 = 60%). In the HLT group, the most common indication for HT was CHD (12/20 = 60%), and the most common indication for liver transplant was cirrhosis (9/20 = 45%). The 1, 3, and 5 year actuarial survival rates in pediatric simultaneous HLT recipients (n = 16) were 93%, 93%, and 93%, respectively, and was similar to isolated PHT alone (88%, 81%, and 75.5%, respectively and isolated PLT alone (84%, 82%, and 80%), respectively. There was no heart or liver rejection reported in the HLT group versus 9.9% in heart and 10.6% in liver transplant-only groups, respectively. CONCLUSION: Pediatric HLT is an uncommon but acceptable option for recipients with combined end-organ failure, with intermediate survival outcomes comparable to those of single-organ recipients.


Assuntos
Transplante de Coração , Transplante de Fígado , Avaliação de Processos e Resultados em Cuidados de Saúde , Adolescente , Criança , Estudos de Coortes , Feminino , Rejeição de Enxerto , Sobrevivência de Enxerto , Humanos , Masculino , Sistema de Registros , Taxa de Sobrevida , Estados Unidos
17.
ASAIO J ; 67(9): 1036-1043, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-33470634

RESUMO

The Jarvik 2015 Ventricular Assist Device (VAD) (Jarvik Inc, New York, NY) is the first and currently only continuous-flow VAD specifically designed for small children, and it is being evaluated in the so-called Pump for Kids, Infants, and Neonates (PumpKIN) trial. Due to the strict inclusion criteria of the trial, there have been a group of patients who failed to meet the criteria and therefore received the Jarvik 2015 VAD under the designation of "compassionate use." This is the same phenomenon seen previously during the Berlin Heart EXCOR trial. While we await the results of the PumpKIN trial, which will report the device performance in a strictly selected population, the compassionate use cases represent actual "real world" experiences. We describe herein our experience of two compassionate use cases. In particular, this report has a special emphasis on the power consumption and hemolysis and inflammatory lab profile of the Jarvik 2015 VAD as hemocompatibility was the primary focus of the developmental and the preclinical phases.


Assuntos
Insuficiência Cardíaca , Coração Auxiliar , Criança , Ensaios Clínicos como Assunto , Insuficiência Cardíaca/cirurgia , Hemólise , Humanos , Lactente , Recém-Nascido
18.
Pediatr Transplant ; 25(3): e13904, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33179431

RESUMO

Tracheostomy is associated with increased mortality and resource utilization in children with CHD. However, the prevalence and hospital outcomes of tracheostomy in children with HTx are not known. We describe the prevalence and compare the post-HTx hospital outcomes of pediatric patients with Pre-TT and Post-TT to those without tracheostomy. A multi-institutional retrospective cohort study was performed using the Pediatric Health Information System database. Hospital mortality, mediastinitis, LOS, and costs were compared among patients with Pre-TT, Post-TT, and no tracheostomy. Pre-TT was identified in 29 (1.1%) and Post-TT was identified in 41 (1.6%) of 2603 index HTx hospitalizations. Patients with Pre-TT were younger and more likely to have CHD, a non-cardiac birth defect, or an airway anomaly compared to those without Pre-TT. Pre-TT was not independently associated with increased post-HTx in-hospital mortality. Age at HTx < 1 year, CHD, and Post-TT were associated with increased in-hospital mortality. Pre-TT that occurred during the HTx hospitalization and Post-TT were associated with increased resource utilization. Tracheostomy was not associated with mediastinitis.


Assuntos
Transplante de Coração , Traqueostomia/estatística & dados numéricos , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Mortalidade Hospitalar , Humanos , Lactente , Masculino , Estudos Retrospectivos , Traqueostomia/mortalidade , Resultado do Tratamento
19.
Pediatr Transplant ; 25(3): e13868, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-32949098

RESUMO

The clinical course of COVID-19 in pediatric solid organ transplant recipients remains ambiguous. Though preliminary experiences with adult transplant recipients have been published, literature centered on the pediatric population is limited. We herein report a multi-center, multi-organ cohort analysis of COVID-19-positive transplant recipients ≤ 18 years at time of transplant. Data were collected via institutions' respective electronic medical record systems. Local review boards approved this cross-institutional study. Among 5 transplant centers, 26 patients (62% male) were reviewed with a median age of 8 years. Six were heart recipients, 8 kidney, 10 liver, and 2 lung. Presenting symptoms included cough (n = 12 (46%)), fever (n = 9 (35%)), dry/sore throat (n = 3 (12%)), rhinorrhea (n = 3 (12%)), anosmia (n = 2 (8%)), chest pain (n = 2 (8%)), diarrhea (n = 2 (8%)), dyspnea (n = 1 (4%)), and headache (n = 1 (4%)). Six patients (23%) were asymptomatic. No patient required supplemental oxygen, intubation, or ECMO. Eight patients (31%) were hospitalized at time of diagnosis, 3 of whom were already admitted for unrelated problems. Post-transplant immunosuppression was reduced for only 2 patients (8%). All symptomatic patients recovered within 7 days. Our multi-institutional experience suggests the prognoses of pediatric transplant recipients infected with COVID-19 may mirror those of immunocompetent children, with infrequent hospitalization and minimal treatment, if any, required.


Assuntos
COVID-19/complicações , COVID-19/imunologia , Rejeição de Enxerto/prevenção & controle , Hospedeiro Imunocomprometido , Imunossupressores/uso terapêutico , Transplante de Órgãos , Assistência Perioperatória/métodos , Adolescente , COVID-19/diagnóstico , COVID-19/terapia , Criança , Pré-Escolar , Feminino , Rejeição de Enxerto/imunologia , Hospitalização/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Masculino , Assistência Perioperatória/estatística & dados numéricos , Índice de Gravidade de Doença , Resultado do Tratamento
20.
Pediatr Transplant ; 24(4): e13711, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32320118

RESUMO

Educational development is an important component of quality of life for children with heart transplant. Aims include determining prevalence of and risk factors for modified education placement in a large representative sample of pediatric heart transplant recipients. Participants included 1495 patients (age 6-18 years) from the PHTS database. Data on education placement and clinical predictors were collected at listing and at 1 and 3 years post-transplant. At listing, 88% of patients were in typical education placement, while 12% were in modified education. Males (P = .02), those with CHD (P < .0001), those with non-private insurance (P < .0001), and those with longer hospital stay (P = .001) were more likely to be in a modified education placement at time of listing. Age, race, listing status, mechanical support, and waitlist time were not significantly associated with placement. The prevalence of typical education placement was similar (87% at 1-year and 86% at 3-year) post-transplant. Predictors of modified education placement at 3-year follow-up included placement at listing (OR = 12.9 [95% CI 7.6-21.9], P < .0001), non-private insurance (OR = 2.0 [95% CI 1.3-3.2], P = .001), CHD (OR = 1.8 [95% CI 1.1-2.7, P = .01), history of post-transplant infection (OR = 1.9 [95% CI 1.2-2.9, P = .007), and number of post-transplant infections (OR = 1.3 [95% CI 1.1-1.5, P = .002). Among pediatric heart transplant recipients, males, those with non-private insurance, those with CHD, and those who experience post-transplant infections are at greatest risk for modified academic placement, which persists for several years post-transplant and deserves targeted intervention.


Assuntos
Escolaridade , Transplante de Coração , Deficiências da Aprendizagem/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Adolescente , Criança , Estudos de Coortes , Feminino , Humanos , Masculino , Fatores de Risco
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