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1.
Pediatr Transplant ; 28(1): e14675, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38062996

RESUMO

Heart transplantation has become the standard of care for pediatric patients with end-stage heart disease throughout the world. Since the first transplant was performed in 1967, the number of transplants has grown dramatically with 13 449 pediatric heart transplants being reported to The International Society of Heart and Lung Transplant (ISHLT) between January 1992 and June 30, 2018. Outcomes have consistently improved over the last few decades, specifically short-term outcomes. Most recent survival data demonstrate that recipients who survive to 1-year post-transplant have excellent long-term survival with more than 60% of those who were transplanted as infants being alive 25 years later. Nonetheless, the rates of graft loss beyond the first year have remained relatively constant over time; driven primarily by our poor understanding and lack of treatments for chronic allograft vasculopathy (CAV). Acute rejection, CAV, graft failure, and infection continue to be the major causes of death within the first 5 years post-transplant. In addition, renal dysfunction, malignancy, and the need for re-transplantation remain as significant issues that require close follow-up. Looking forward, key challenges include improving donor utilization rates (including donation after cardiac death (DCD) and the use of ex vivo perfusion devices), the development of non-invasive biomarkers for rejection, efforts to mitigate the long-term effects of immunosuppression, and prevention of CAV. It is not possible to cover the entire evolution of pediatric heart transplantation over the last five decades, but in this review, we hope to touch on key observations, lessons learned, and practice changes that have advanced the field, as well as glance ahead to the next decade.


Assuntos
Transplante de Coração , Transplante de Coração-Pulmão , Doenças Vasculares , Lactente , Humanos , Criança , Rejeição de Enxerto/prevenção & controle , Estudos Retrospectivos , Doadores de Tecidos , Sobrevivência de Enxerto
2.
Pediatr Transplant ; 28(1): e14471, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37294621

RESUMO

The International Pediatric Transplant Association convened an expert consensus conference to assess current evidence and develop recommendations for various aspects of care relating to post-transplant lymphoproliferative disorders after solid organ transplantation in children. In this report from the Viral Load and Biomarker Monitoring Working Group, we reviewed the existing literature regarding the role of Epstein-Barr viral load and other biomarkers in peripheral blood for predicting the development of PTLD, for PTLD diagnosis, and for monitoring of response to treatment. Key recommendations from the group highlighted the strong recommendation for use of the term EBV DNAemia instead of "viremia" to describe EBV DNA levels in peripheral blood as well as concerns with comparison of EBV DNAemia measurement results performed at different institutions even when tests are calibrated using the WHO international standard. The working group concluded that either whole blood or plasma could be used as matrices for EBV DNA measurement; optimal specimen type may be clinical context dependent. Whole blood testing has some advantages for surveillance to inform pre-emptive interventions while plasma testing may be preferred in the setting of clinical symptoms and treatment monitoring. However, EBV DNAemia testing alone was not recommended for PTLD diagnosis. Quantitative EBV DNAemia surveillance to identify patients at risk for PTLD and to inform pre-emptive interventions in patients who are EBV seronegative pre-transplant was recommended. In contrast, with the exception of intestinal transplant recipients or those with recent primary EBV infection prior to SOT, surveillance was not recommended in pediatric SOT recipients EBV seropositive pre-transplant. Implications of viral load kinetic parameters including peak load and viral set point on pre-emptive PTLD prevention monitoring algorithms were discussed. Use of additional markers, including measurements of EBV specific cell mediated immunity was discussed but not recommended though the importance of obtaining additional data from prospective multicenter studies was highlighted as a key research priority.


Assuntos
Infecções por Vírus Epstein-Barr , Transtornos Linfoproliferativos , Transplante de Órgãos , Humanos , Criança , Herpesvirus Humano 4/genética , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/diagnóstico , Estudos Prospectivos , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/prevenção & controle , DNA Viral , Transplante de Órgãos/efeitos adversos , Biomarcadores , Carga Viral
3.
Pediatr Transplant ; 28(5): e14781, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38808744

RESUMO

The International Pediatric Transplant Association convened an expert consensus conference to assess current evidence and develop recommendations for various aspects of care relating to post-transplant lymphoproliferative disorders (PTLD) after pediatric solid organ transplantation. This report addresses the outcomes of deliberations by the PTLD Management Working Group. A strong recommendation was made for reduction in immunosuppression as the first step in management. Similarly, strong recommendations were made for the use of the anti-CD20 monoclonal antibody (rituximab) as was the case for chemotherapy in selected scenarios. In some scenarios, there is uncoupling of the strength of the recommendations from the available evidence in situations where such evidence is lacking but collective clinical experiences drive decision-making. Of note, there are no large, randomized phase III trials of any treatment for PTLD in the pediatric age group. Current gaps and future research priorities are highlighted.


Assuntos
Transtornos Linfoproliferativos , Transplante de Órgãos , Complicações Pós-Operatórias , Rituximab , Humanos , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/terapia , Criança , Adolescente , Rituximab/uso terapêutico , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/diagnóstico , Imunossupressores/uso terapêutico , Pré-Escolar
4.
Am J Transplant ; 23(12): 1893-1907, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37579817

RESUMO

The aim of this study (CTOTC-09) was to assess the impact of "preformed" (at transplant) donor-specific anti-HLA antibody (DSA) and first year newly detected DSA (ndDSA) on allograft function at 3 years after pediatric heart transplantation (PHTx). We enrolled children listed at 9 North American centers. The primary end point was pulmonary capillary wedge pressure (PCWP) at 3 years posttransplant. Of 407 enrolled subjects, 370 achieved PHTx (mean age, 7.7 years; 57% male). Pre-PHTx sensitization status was nonsensitized (n = 163, 44%), sensitized/no DSA (n = 115, 31%), sensitized/DSA (n = 87, 24%), and insufficient DSA data (n = 5, 1%); 131 (35%) subjects developed ndDSA. Subjects with any DSA had comparable PCWP at 3 years to those with no DSA. There were also no significant differences overall between the 2 groups for other invasive hemodynamic measurements, systolic graft function by echocardiography, and serum brain natriuretic peptide concentration. However, in the multivariable analysis, persistent first-year DSA was a risk factor for 3-year abnormal graft function. Graft and patient survival did not differ between groups. In summary, overall, DSA status was not associated with worse allograft function or inferior patient and graft survival at 3 years, but persistent first-year DSA was a risk factor for late graft dysfunction.


Assuntos
Transplante de Coração , Isoanticorpos , Humanos , Criança , Masculino , Feminino , Antígenos HLA , Doadores de Tecidos , Transplante de Coração/efeitos adversos , Transplante Homólogo , Soro Antilinfocitário , Sobrevivência de Enxerto , Rejeição de Enxerto , Estudos Retrospectivos
5.
Eur Radiol ; 33(8): 5446-5454, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36786904

RESUMO

OBJECTIVES: To assess abnormal liver enhancement on 15-20 min delayed 3D high-resolution late gadolinium enhancement (3D HR LGE) sequence in patients with Fontan circulation. METHODS: Retrospective study of pediatric Fontan patients (< 18 years old) with combined cardiac-liver MRI from January 2018 to August 2021. Abnormal hepatic enhancement was graded (0-3) for each lobe, summed for a total liver enhancement score (0-6), and compared to repaired tetralogy of Fallot (rTOF) patients. Correlations with other hepatic imaging biomarkers were performed. Temporal relationships of enhancement compared to traditional early portal venous and 5-7-min delayed phase liver imaging were analyzed. RESULTS: The Fontan group (n = 35, 13 ± 3.4 years old, median time from Fontan 10 (9-12) years) had 23/35 (66%) with delayed 3D HR LGE total liver enhancement score > 0 (range 0-5), with greater involvement of the right lobe (1 (0-1) vs 0 (0-1), p < 0.01). The rTOF group (n = 35, 14 ± 2.6 years old) had no abnormal enhancement. In the Fontan group, total liver enhancement was 3 (2-4) in the early portal venous phase, lower at 1 (1-2) in the 5-7-min delayed phase (p < 0.01), and lowest at 1 (0-2) in the 15-20-min delayed phase (p = 0.03). 3D HR LGE enhancement correlated inversely with portal vein flow (rs = - 0.42, p = 0.01) and positively with left lobe stiffness (rs = 0.51, p < 0.01). The enhancement score decreased in 13/35 (37%) between the 5-7- and 15-20-min delayed phases. CONCLUSIONS: Liver fibrosis can be assessed on 3D HR LGE sequences in patients with Fontan circulation, correlates with other imaging biomarkers of Fontan liver disease, and may add information for hepatic surveillance in this population. KEY POINTS: • Abnormal liver enhancement on 3D HR LGE sequences in Fontan patients likely represents liver fibrosis and is seen in up to 66% of children and adolescents with variable distribution and severity. • The degree of 3D HR LGE liver enhancement correlates with decreased portal vein flow and increased left hepatic lobe stiffness.


Assuntos
Técnica de Fontan , Tetralogia de Fallot , Humanos , Criança , Adolescente , Meios de Contraste , Gadolínio , Estudos Retrospectivos , Cirrose Hepática/diagnóstico por imagem , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Imageamento por Ressonância Magnética/métodos , Biomarcadores
6.
Pediatr Transplant ; 27 Suppl 1: e14317, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36468320

RESUMO

BACKGROUND: There has not been a comprehensive global survey of pediatric-deceased donor allocation practices across all organs since the advent of deceased donor transplantation at the end of the 20th century. As an international community that is responsible for transplanting children, we set out to survey the existing landscape of allocation. We aimed to summarize current practices and provide a snapshot overview of deceased donor allocation practices to children across the world. METHODS: The International Registry in Organ Donation and Transplantation (IRODAT, www.irodat.org) was utilized to generate a list of all countries in the world, divided by continent, that performed transplantation. We reviewed the published literature, published allocation policy, individual website references and associated links to publicly available listed allocation policies. Following this, we utilized tools of communication, relationships, and international fellowship to confirm deceased donation pediatric centers and survey pediatric allocation practices for liver, kidney, heart, and lung across the world. We summarize pediatric allocation practices by organ when available using source documents, and personal communication when no source documents were available. RESULTS: The majority of countries had either formal or informal policies directed toward minimizing organ distribution disparity among pediatric patients. CONCLUSION: Children have long-term life to gain from organ donation yet continue to die while awaiting transplantation. We summarize global strategies that have been employed to provide meaningful and sustained benefit to children on the waitlist.


Assuntos
Transplante de Órgãos , Obtenção de Tecidos e Órgãos , Criança , Humanos , Doadores de Tecidos , Rim , Fígado
7.
Pediatr Transplant ; 27(6): e14560, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37377051

RESUMO

BACKGROUND: Neonatal Marfan syndrome is a rare disease with mortality in the first year of life reported as high as 95% predominantly due to progressive heart failure from valvar regurgitation and cardiomyopathy. Multisystem involvement and uncertain prognosis have historically precluded transplant candidacy, and current management options are of limited success. CASE REPORT: We present a baby girl with a postnatal diagnosis of neonatal Marfan syndrome who at 1 year of age underwent mitral valve and tricuspid valve repair with postoperative profound left ventricular and moderate right ventricular dysfunction necessitating biventricular assist device (BiVAD) support and subsequent heart transplant. A number of noncardiac issues persisted in our patient; however, she enjoyed a good quality of life for the initial 3 years posttransplant. Unfortunately, she subsequently developed rapidly progressive coronary allograft vasculopathy (CAV) with progressive deterioration in function and cardiac arrest. CONCLUSION: To our best knowledge, this is only the second case of neonatal Marfan syndrome to undergo heart transplant reported in the literature and the first with BiVAD support as a bridge to candidacy. This is also the first case of neonatal Marfan syndrome associated with intragenic duplication. This case though demonstrating that earlier listing, ventricular assist device (VAD) support and even primary transplant as treatment in neonatal Marfan syndrome should all be considered viable options but also portends a cautionary tale given the spectrum of comorbidities in this rare and severe disorder.


Assuntos
Cardiomiopatias , Transplante de Coração , Síndrome de Marfan , Lactente , Recém-Nascido , Feminino , Humanos , Síndrome de Marfan/complicações , Síndrome de Marfan/diagnóstico , Qualidade de Vida , Cardiomiopatias/complicações , Valva Tricúspide
8.
Pediatr Transplant ; 27(2): e14461, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36593638

RESUMO

BACKGROUND: Elevated pulmonary vascular resistance (PVR) in the setting of left heart failure may contribute to poor outcomes after pediatric heart transplant (HTx), but peri-transplant management is variable. METHODS: We sought to characterize international practice by surveying physicians at pediatric HTx centers. RESULTS: We received 49 complete responses from 39 centers in 16 countries. Most respondents are pediatric cardiologists (90%), practice at centers offering heart (86%) and lung (55%) transplant, and perform pre-HTx acute vasoreactivity testing (AVT, 88%) in patients with elevated PVR. Half (51%) reported defining a PVR cutoff for HTx eligibility as ≤6 WU m2 (56%) post-AVT (84%). The highest post-AVT PVR ever accepted for HTx ranged from 3-14.4 (median 6) WU m2 . To treat elevated pre-transplant PVR, phosphodiesterase type 5 inhibitors are most common (65%) followed by oxygen (31%), nitric oxide (14%), endothelin receptor antagonists (11%), and prostacyclins (6%). Nearly a third (31%) do not routinely use pulmonary vasodilators without implantation of a left ventricular assist device (LVAD). Case scenarios highlight treatment variability: in a restrictive cardiomyopathy scenario, HTx listing with post-transplant vasodilator therapy was favored, whereas in a Shone's complex patient with fixed PVR, LVAD ± pulmonary vasodilators followed by repeat catheterization was most common. Management of dilated cardiomyopathy with reactive PVR was variable. Most continue vasodilator therapy until HTx (16%), PVR normalizes (16%) or ≤6 months. CONCLUSIONS: Management of elevated PVR in children awaiting HTx is heterogenous. Evidence-based guidelines are needed to allow for longitudinal determination of optimal outcomes and standardized care.


Assuntos
Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Hipertensão Pulmonar , Humanos , Criança , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/terapia , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/cirurgia , Resistência Vascular/fisiologia , Vasodilatadores , Resultado do Tratamento , Estudos Retrospectivos
9.
Pediatr Transplant ; 26(3): e14218, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34985793

RESUMO

BACKGROUND: Heart transplantation has become the standard of care for pediatric patients with end-stage heart disease, and outcomes have consistently improved over the last few decades. CAV, however, remains a leading cause of morbidity and mortality in heart transplantation and is the leading cause of death beyond 3 years post-transplantation. We sought out to provide an in-depth overview of CAV in the pediatric heart transplant population. METHODS: Database searches were conducted in both Medline and Embase on the topic of cardiac vasculopathy in pediatric heart transplant recipients. The search used five broad concept terms: heart transplant; pediatric; CAV; diagnosis, prognosis, and risk factors; and guidelines and reviews. References were captured if there was at least one term in each of the concepts. The search was limited to articles in the English language. RESULTS: A total of 148 articles were identified via the literature search with further articles identified via review of references. Pediatric data regarding the etiology and development of CAV remain limited although knowledge about the immune and non-immune factors playing a role are increasing. CAV continues to be difficult to detect with many invasive and non-invasive methods available, yet their effectiveness in the detection of CAV remains suboptimal. There remains no proven medical intervention to treat or reverse established CAV disease, and CAV is associated with high rates of graft loss once detected. However, several medications are used in hopes of preventing, slowing progression, or modifying the outcomes. CONCLUSION: This review provides a comprehensive overview of CAV, discusses its clinical presentation, risk factors, diagnostic tools used to identify CAV in the pediatric population, and highlights the current therapeutic options and the need for ongoing research.


Assuntos
Cardiopatias , Transplante de Coração , Doenças Vasculares , Aloenxertos , Criança , Cardiopatias/etiologia , Humanos , Fatores de Risco , Doenças Vasculares/diagnóstico , Doenças Vasculares/etiologia
10.
Pediatr Transplant ; 26(3): e14214, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35178843

RESUMO

BACKGROUND: Despite significant medical advances in the field of pediatric heart transplantation (HT), acute rejection remains an important cause of morbidity and mortality. Endomyocardial biopsy (EMB) remains the gold-standard method for diagnosing rejection but is an invasive, expensive, and stressful process. Given the potential adverse consequences of rejection, routine post-transplant rejection surveillance protocols incorporating EMB are widely employed to detect asymptomatic rejection. Each center employs their own specific routine rejection surveillance protocol, with no consensus on the optimal approach and with high inter-center variability. The utility of high-frequency and long-term routine surveillance biopsies (RSB) in pediatric HT has been called into question. METHODS: Sources for this comprehensive review were primarily identified through searches in biomedical databases including MEDLINE and Embase. RESULTS: The available literature suggests that the diagnostic yield of RSB is low beyond the first year post-HT and that a reduction in RSB intensity from high-frequency to low-frequency can be done safely with no impact on early and mid-term survival. Though there are emerging non-invasive methods of detecting asymptomatic rejection, the evidence is not yet strong enough for any test to replace EMB. CONCLUSION: Overall, pediatric HT centers in North America should likely be doing fewer RSB than are currently performed. Risk factors for rejection should be considered when designing the optimal rejection surveillance strategy. Noninvasive testing including emerging biomarkers may have a complementary role to aid in safely reducing the need for RSB.


Assuntos
Transplante de Coração , Miocárdio , Biópsia , Criança , Rejeição de Enxerto/epidemiologia , Transplante de Coração/efeitos adversos , Humanos , Miocárdio/patologia , Fatores de Risco
11.
Pediatr Transplant ; 26(7): e14331, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35851709

RESUMO

BACKGROUND: Professionals working in pediatric transplantation commonly encounter complex ethical dilemmas. Most ethical research in transplantation is related to adult practice. We aimed to gain insight into ethical issues faced by transplant professionals when dealing with pediatric transplant recipients. METHODS: A two-stage study was designed; the first part was a questionnaire completed by 190 (80%) members of the International Pediatric Transplant Association (IPTA) from over 30 different countries. This was followed by a multidisciplinary focus group that explored the preliminary data of the survey. RESULTS: A total of 38% (56 of 149) respondents of the questionnaire had experienced an ethical issue between 2016 and 2018. Surgeons were more likely to have encountered an ethical issue as compared with physicians (60% vs. 35.7%, p = .035). Clinicians from Europe were more likely to have experienced an ethical issue in living organ donation compared with those from North America (78.9% vs. 52.5%, p = .005), with common ethical concerns being psychosocial evaluation and follow-up care of donors. The focus group highlighted the importance of a multidisciplinary approach to ethical issues. CONCLUSION: The results of this study can direct future research into pediatric transplantation ethics with the aim of producing educational resources, policies, and ethical guidelines.


Assuntos
Transplante de Órgãos , Médicos , Obtenção de Tecidos e Órgãos , Adulto , Criança , Humanos , Inquéritos e Questionários , Doadores de Tecidos
12.
Pediatr Transplant ; 26(5): e14276, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35340105

RESUMO

INTRODUCTION: Brugada syndrome is an inherited channelopathy characterized by arrhythmia and an increased risk of sudden cardiac death (SCD). Implantation of a defibrillator for primary or secondary prevention is the only effective strategy to decrease the risk of SCD in Brugada syndrome. We present a case in which a cardiac donor had a pathogenic variant for Brugada syndrome, discovered on genetic testing after transplantation. CASE REPORT: A young child with dilated cardiomyopathy underwent orthotopic heart transplantation from a donor with in-hospital cardiac arrest in the context of fever and a normal ECG. Approximately 1 month after transplant, the donor's post mortem genetic testing revealed a pathogenic loss-of-function SCN5A variant associated with Brugada syndrome, which was confirmed on genetic testing on a post-transplant endomyocardial biopsy from the recipient. The recipient's post-transplant electrocardiographic monitoring revealed persistent right bundle branch block and progressive, asymptomatic sinus node dysfunction. The recipient was managed with precautionary measures including aggressive fever management, avoidance of drugs that increase arrhythmia risk in Brugada syndrome, and increased frequency of arrhythmia surveillance. The recipient remains asymptomatic at over 3 years post-transplant with preserved graft function and no documented ventricular arrhythmias. CONCLUSION: We describe the clinical course of "acquired" Brugada syndrome in a cardiac allograft recipient, which has not been previously reported. The time-sensitive nature of donor organ selection, especially in critically ill recipients, combined with the growing use of molecular autopsies in patients with unexplained etiologies for death may increasingly result in important donor genetic information being made available after transplantation.


Assuntos
Síndrome de Brugada , Aloenxertos , Arritmias Cardíacas/complicações , Arritmias Cardíacas/genética , Síndrome de Brugada/complicações , Síndrome de Brugada/diagnóstico , Criança , Morte Súbita Cardíaca/etiologia , Eletrocardiografia/efeitos adversos , Humanos
13.
Pediatr Transplant ; : e14333, 2022 Nov 11.
Artigo em Inglês | MEDLINE | ID: mdl-36369733

RESUMO

The International Pediatric Transplant Association (IPTA) Consensus Conference on Practice Guidelines for the Diagnosis, Prevention, and Management of Post-Transplant Lymphoproliferative Disorders after Solid Organ Transplantation in Children took place on March 12-13, 2019, and the work of conference members continued until the end of December 2021. The goal was to produce evidence-based consensus guidelines on the definitions, diagnosis, prevention, and management of PTLD and related disorders based on the critical review of the literature and consensus of experts. This report describes the goals, organization, and methodology of the consensus conference and follow-up activities. The results of each working group (Definitions, Prevention, Management, and Epstein-Barr viral [EBV] load/Biomarker Monitoring) are presented in separate manuscripts within this volume of Pediatric Transplantation.

14.
Pediatr Transplant ; : e14350, 2022 Nov 11.
Artigo em Inglês | MEDLINE | ID: mdl-36369745

RESUMO

The International Pediatric Transplant Association (IPTA) convened an expert consensus conference to assess current evidence and develop recommendations for various aspects of care relating to post-transplant lymphoproliferative disorder after solid organ transplantation in children. In this report from the Prevention Working Group, we reviewed the existing literature regarding immunoprophylaxis and chemoprophylaxis, and pre-emptive strategies. While the group made a strong recommendation for pre-emptive reduction of immunosuppression at the time of EBV DNAemia (low to moderate evidence), no recommendations for use could be made for any prophylactic strategy or alternate pre-emptive strategy, largely due to insufficient or conflicting evidence. Current gaps and future research priorities are highlighted.

15.
Cardiol Young ; 32(7): 1041-1047, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34486505

RESUMO

Heart failure due to dilated cardiomyopathy is a major indication for paediatric cardiac transplantation. Endocardial fibroelastosis is a recognised pathological finding of unknown prognostic significance in paediatric dilated cardiomyopathy. To evaluate the nature of the association between left ventricular endocardial fibroelastosis and paediatric dilated cardiomyopathy, we reviewed surgical pathology reports of dilated cardiomyopathy explants (1986-2016) in order to characterise the pathological findings and to compare and contrast their frequency among four age groups: less than 1 year; 1-5 years; 6-10 years; and greater than 11 years. The 89 explants (47 males and 42 females) were all characterised by increased weight and left ventricular chamber dilatation without increased wall thickness. Ninety-five per cent of the specimens in the two youngest subsets had left ventricular endocardial fibroelastosis. Compared to the oldest age group, recipients aged 1-5 years had a 6-fold increase and those younger than 1 year a 19-fold increase in the odds of observing left ventricular endocardial fibroelastosis. Explants with and without endocardial fibroelastosis were otherwise phenotypically similar. In paediatric dilated cardiomyopathy endocardial fibroelastosis is a very common pathological finding, especially in infants and young children. We propose that the descriptive, clinico-pathological designation "Dilated Cardiomyopathy with Endocardial Fibroelastosis" should be adopted to facilitate future investigation into the potential prognostic/therapeutic significance of left ventricular endocardial fibroelastosis.


Assuntos
Cardiomiopatia Dilatada , Fibroelastose Endocárdica , Transplante de Coração , Cardiomegalia , Criança , Pré-Escolar , Fibroelastose Endocárdica/complicações , Endocárdio/patologia , Feminino , Ventrículos do Coração , Humanos , Lactente , Masculino
16.
Pediatr Nephrol ; 36(11): 3595-3605, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-33599850

RESUMO

Over the past few decades, there has been increasing recognition of kidney disease in children with non-kidney solid organ transplantation. The risk of kidney disease in children undergoing heart or liver transplantation is higher than the general population as the underlying disease and its associated management may directly impair kidney function. Both heart and liver failures contribute to hypoperfusion and kidney ischemia before patients reach the point of transplant. The transplant surgery itself can often be complicated by acute kidney injury (AKI), which may be further exacerbated by a complicated postoperative course. In the short- and long-term post-transplant period, these children are at risk of acute illness, exposed to nephrotoxic medications, and susceptible to rare but severe infections and immunologic insults that may contribute to AKI and chronic kidney disease (CKD). In some, CKD can progress to kidney failure with replacement therapy (KFRT). CKD and KFRT are associated with increased morbidity and mortality in this patient population. Therefore, it is critical to monitor for and recognize the risk factors for kidney injury in this population and mitigate these risks. In this paper, the authors provide an overview of kidney disease pertaining to heart and liver transplantation in children with guidance on monitoring, diagnosis, prevention, and management.


Assuntos
Transplante de Coração , Nefropatias , Transplante de Fígado , Criança , Transplante de Coração/efeitos adversos , Humanos , Nefropatias/epidemiologia , Transplante de Fígado/efeitos adversos
17.
Circulation ; 140(1): e9-e68, 2019 07 02.
Artigo em Inglês | MEDLINE | ID: mdl-31132865

RESUMO

In this scientific statement from the American Heart Association, experts in the field of cardiomyopathy (heart muscle disease) in children address 2 issues: the most current understanding of the causes of cardiomyopathy in children and the optimal approaches to diagnosis cardiomyopathy in children. Cardiomyopathies result in some of the worst pediatric cardiology outcomes; nearly 40% of children who present with symptomatic cardiomyopathy undergo a heart transplantation or die within the first 2 years after diagnosis. The percentage of children with cardiomyopathy who underwent a heart transplantation has not declined over the past 10 years, and cardiomyopathy remains the leading cause of transplantation for children >1 year of age. Studies from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry have shown that causes are established in very few children with cardiomyopathy, yet genetic causes are likely to be present in most. The incidence of pediatric cardiomyopathy is ≈1 per 100 000 children. This is comparable to the incidence of such childhood cancers as lymphoma, Wilms tumor, and neuroblastoma. However, the published research and scientific conferences focused on pediatric cardiomyopathy are sparcer than for those cancers. The aim of the statement is to focus on the diagnosis and classification of cardiomyopathy. We anticipate that this report will help shape the future research priorities in this set of diseases to achieve earlier diagnosis, improved clinical outcomes, and better quality of life for these children and their families.


Assuntos
American Heart Association , Cardiomiopatias/classificação , Cardiomiopatias/diagnóstico , Adolescente , Cardiomiopatias/epidemiologia , Cardiomiopatias/genética , Criança , Testes Genéticos/normas , Humanos , Sistema de Registros/normas , Estados Unidos/epidemiologia
18.
Pediatr Transplant ; 24(8): e13832, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33105067

RESUMO

BACKGROUND: As survival in pediatric heart transplantation (HTx) has improved due to medical advances, the analysis of long-term outcomes impacting quality of life such as cognition and development becomes increasingly important. Neuropsychological assessments provide a comprehensive understanding of individual needs, allowing for the development of tailored recommendations and interventions. METHODS: Routine neuropsychological assessment was completed between 5 and 7 years of age in this cohort of pediatric HTx recipients at our center (Jan 2014-Oct 2018), including tests of general intellect (WPPSI-IV, WISC-V), academics (WIAT-II/III), perceptual-motor abilities (Beery VMI), and memory (CMS). Relevant medical variables were collected. RESULTS: Among 25 children, the median age at testing was 6.7 (IQR:5.8-7.4) years, with a median time since HTx of 5.2 (IQR:4.8-6.8) years. Medical diagnoses included congenital heart disease (CHD; 56%) and cardiomyopathy (44%). Cognitive functioning across the intellectual, academic, and perceptual-motor domains fell within the low-average range, while memory abilities fell within the average range. DSM-5 clinical diagnoses were provided for 14 (56%) children: Intellectual Disability-Mild (20%), Learning Disability (20%), Language Disorder (8%), and Attention-Deficit/Hyperactivity Disorder (12%). The presence of neurological issues and/or CHD predicted poorer performance on various neuropsychological domains. CONCLUSIONS: Over 50% of this cohort of pediatric heart transplant recipients seen for routine post-HTx neuropsychological assessment received a clinical psychological diagnosis, notably higher than rates in the general population. This population requires monitoring to ensure that high risk children are identified and successfully supported in school and their community.


Assuntos
Transtornos Cognitivos/diagnóstico , Transtornos Cognitivos/epidemiologia , Transplante de Coração , Criança , Feminino , Humanos , Masculino , Testes Neuropsicológicos , Ontário/epidemiologia
19.
Pediatr Transplant ; 24(1): e13609, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31713958

RESUMO

Pneumocystis jirovecii pneumonia (PJP) prophylaxis after pediatric solid organ transplant (SOT) is routinely recommended, but practice varies. Online survey was sent in 2018 to 707 members of the International Pediatric Transplant Association. A total of 105 responded, representing 47 institutions in 18 countries consisting of transplant physicians (66%), transplant surgeons (19%), nurse practitioners (6%), infectious disease physicians (5%), or pharmacists (4%). PJP prophylaxis was reported by 88%, while 12% did not routinely give prophylaxis. The majority not using PJP prophylaxis performed renal transplants (67%) citing low incidence of PJP (62%). Trimethoprim/sulfamethoxazole was first-line agent (95%). PJP prophylaxis for 4-6 months was the most frequent duration following kidney (48%, 27/56), liver (42%, 13/31), and heart (40%, 10/25) transplant. Abdominal multivisceral providers equally gave 10-12 months (47%) or lifelong (47%); most lung transplant providers gave lifelong prophylaxis (85%). Across all organs, 21% provided lifetime prophylaxis. After completion of prophylaxis, 32% do not restart for any reason; majority of the rest would restart for treatment of acute graft rejection. 83% reported no PJP cases in the prior 12 months; 14% reporting 1-5 infections. Only 3% reported a case of PJP infection on prophylaxis; none in SOT. PJP prophylaxis is routinely provided to pediatric SOT patients though practice and duration vary by center and organ type. Durations of 4-6 months were most common for renal, liver, and heart transplant recipients, while 10-12 months or lifelong prophylaxis were commonly reported for abdominal multivisceral recipients and most lung transplant recipients are given lifelong prophylaxis.


Assuntos
Fidelidade a Diretrizes/estatística & dados numéricos , Transplante de Órgãos , Pneumocystis carinii , Pneumonia por Pneumocystis/prevenção & controle , Cuidados Pós-Operatórios/métodos , Complicações Pós-Operatórias/prevenção & controle , Padrões de Prática Médica/estatística & dados numéricos , Anti-Infecciosos/uso terapêutico , Criança , Pesquisas sobre Atenção à Saúde , Disparidades em Assistência à Saúde/estatística & dados numéricos , Humanos , Pediatria , Pneumonia por Pneumocystis/etiologia , Cuidados Pós-Operatórios/estatística & dados numéricos , Guias de Prática Clínica como Assunto , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico
20.
Pediatr Transplant ; 24(2): e13589, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31562687

RESUMO

Heart transplant providers often focus on post-transplant outcomes when making donor decisions, potentially at the expense of higher waitlist mortality. This study aimed to assess public opinion regarding the selection of donor hearts and the balance between pre- and post-transplant risk. The authors generated a survey to investigate public opinion regarding donor acceptance. The survey was shared freely online across social media platforms in April-May 2019. A total of 718 individuals responded to the survey, with an equal distribution between patients and family members. Respondents consistently favored post-transplant outcomes over waitlist outcomes. About 83.9% of respondents favored a hospital with longer waitlist times, worse waitlist outcomes, but excellent post-transplant survival over a hospital with short waitlist times, a high waitlist survival, and inferior post-transplant survival. This preference was no different between pediatric and adult populations (P = .7), patient and family members (P = .935), or those with a pre- vs post-transplant perspective (P = .985). Patients and their family members consistently favor improved post-transplant survival over waitlist survival when considering the risks of accepting a donor organ. These findings suggest that current practice patterns of donor selection align with the opinions of patients and family members with heart failure or who have undergone heart transplantation.


Assuntos
Atitude Frente a Saúde , Seleção do Doador/métodos , Insuficiência Cardíaca/cirurgia , Transplante de Coração/mortalidade , Listas de Espera/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Pesquisas sobre Atenção à Saúde , Insuficiência Cardíaca/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Participação do Paciente , Preferência do Paciente , Doadores de Tecidos , Adulto Jovem
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