RESUMO
OBJECTIVE: To determine the frequency, predisposing factors, clinical presentation, and outcome of posttransplantation lymphoproliferative disorders (PTLDs) in pediatric thoracic organ transplant recipients. METHODS: Retrospective review of the medical records of all 120 children who survived longer than 1 month after thoracic organ transplantation at our center. RESULTS: PTLD was diagnosed in 14 patients (11.7%), including 7.7% of heart and 19.5% of heart-lung/lung recipients. Presentation of PTLD was variable, ranging from asymptomatic lung nodules on chest radiograph to diffuse multiorgan failure. Treatment with a reduction of immunosuppression and antiviral therapy resulted in resolution of PTLD in eight patients. Eight patients died. PTLD contributed to death in five. No patient seropositive for Epstein-Barr virus (EBV) before transplantation had PTLD. There was a significant association between primary EBV infection after transplantation and the presence of PTLD. CONCLUSIONS: PTLD occurs with greater frequency in pediatric thoracic organ transplant recipients than in the adult transplant population. Primary EBV infection after transplantation is the major risk factor for the development of PTLD. Patients in whom primary EBV infection develops after transplantation should be managed with a reduction in immunosuppression and with heightened surveillance for the development of PTLD.
Assuntos
Transtornos Linfoproliferativos/etiologia , Transplante de Órgãos/efeitos adversos , Cirurgia Torácica , Adolescente , Adulto , Antivirais/administração & dosagem , Antivirais/uso terapêutico , Causas de Morte , Criança , Pré-Escolar , Feminino , Transplante de Coração/efeitos adversos , Transplante de Coração-Pulmão/efeitos adversos , Infecções por Herpesviridae/complicações , Infecções por Herpesviridae/tratamento farmacológico , Herpesvirus Humano 4 , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Lactente , Recém-Nascido , Transplante de Fígado/efeitos adversos , Pneumopatias/etiologia , Transplante de Pulmão/efeitos adversos , Transtornos Linfoproliferativos/tratamento farmacológico , Masculino , Insuficiência de Múltiplos Órgãos/etiologia , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Resultado do Tratamento , Infecções Tumorais por Vírus/complicações , Infecções Tumorais por Vírus/tratamento farmacológicoRESUMO
BACKGROUND: Mechanical circulatory support for intractable heart failure as a bridge to transplantation has been used infrequently in children. The lack of clinically available ventricular assist devices has resulted in the use of conventional extracorporeal circuits with oxygenator as the main modality for circulatory support. In this study we reviewed our experience with extracorporeal membrane oxygenation (ECMO) support in children with irreversible heart failure who were awaiting heart transplantation. METHODS AND RESULTS: Since 1985, 14 children were placed on ECMO support for circulatory failure and were considered candidates for heart transplantation: 8 children had postcardiotomy contractile failure, 3 had dilated cardiomyopathy, and 3 had viral myocarditis. Five of these children had cardiac arrest and were placed on support during cardiopulmonary resuscitation. Mean duration of ECMO support was 109 +/- 20 hours. Eight patients developed pulmonary edema requiring decompression of the left ventricle, 3 by blade atrial septostomy and 5 by left atrial vent cannula. Nine of 14 received a heart transplant, 1 child recovered spontaneously (myocarditis), and 4 died of sepsis on ECMO. Of the children who received transplants, 6 were early survivors with 1 late death (lymphoproliferative disease), for a total of 7 of 14 (50%) early and 6 of 14 (43%) late survivors. CONCLUSIONS: Our experience suggests that ECMO is an effective means of circulatory support as a bridge to transplantation in children. Decompression of the left ventricle is often required to prevent pulmonary edema. Sepsis and bleeding remain a limitation to prolonged mechanical support with ECMO in children.