Assuntos
Tetralogia de Fallot/cirurgia , Adulto , Fatores Etários , Arritmias Cardíacas/etiologia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/cirurgia , Gravidez , Complicações Cardiovasculares na Gravidez/fisiopatologia , Insuficiência da Valva Pulmonar/etiologia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagemRESUMO
Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is an uncommon but serious disease involving the mediastinal structures. A high index of suspicion is essential to establish the diagnosis of FM and starting the appropriate therapy for patients. Here, we report a case of a young female who presented with chest symptoms and subsequently underwent different laboratory and radiologic investigations and an excisional biopsy. The findings of these investigations were consistent with the diagnosis of idiopathic FM. Her disease was associated with complete occlusion of three pulmonary veins and the left main pulmonary artery. The patient was treated with initial high-dose steroids followed by maintenance steroid and methotrexate therapy with very good long-term disease control. Clinical response, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate were used to monitor disease activity and response to therapy.