Abstract :
A 7-year-old boy was admitted for evaluation to heart transplantation with a restrictive cardiopathy congenital associated to BAG3 gene. During childhood, he developed high pulmonary hipertension with a significant tricuspid insufficiency. At first post-operative, patient has evoluted with metabolic acidosis, shock refractory to volume and refractory hypoxemia presented at maneuvers physiotherapy. The levels of methemoglobin were checked on laboratory and the nitric oxide was immediately suspended. It started the administration of methylene blue in 3 mg/kg with progressive improvement of clinical situation. Levels of metheboglobin were 29,05 before the treatment and 5,2 after methylene blue administration. Conclusion: Metahemoglobinemia must be suspected in patients using NO. Elevated levels of metahemoglobin may be incompatible with life. Treatment should be instituted quickly and effectively. Keywords: Nitric oxide; Methemoglobin/analysis; Heart transplantation; Hemoglobins; Methemoglobinemia; Child. Paciente de 7 anos, sexo masculino, portador de miocardiopatia restritiva relacionada ao gene BAG3 e historico de hipertensao pulmonar com insuficiencia tricuspede importante, admitido para avalicao de transplante cardiaco. Apos a cirurgia deu entrada na UTI estavel mas evoluiu no 1 PO com acidose metabolica, choque refratario ao volume e hipoxemia refrataria a manobras de fisioterapia. Os niveis de metahemoglobina foram dosados e o oxido nitrico foi imediatamente suspenso. Iniciou-se administracao endovenosa de azul de metileno na dose 3mg/kg com melhora progressiva do quadro clinico. Os niveis de metahemoglobina eram 29,05 e 5,2 ao inicio e final da administracao da droga, respectivamente. Conclusao: Metahemoglobinemia deve ser suspeitada em pacientes que utilizam NO. Niveis elevados de metahemoglobina no sangue podem ser incompativeis com a vida. O tratamento deve ser instituido de forma rapida e eficaz. Descritores: Oxido nitrico; Metemoglobina/analise; Transplante de coracao; Hemoglobinas; Metemoglobinemia; Crianca.
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