RESUMO
Immunoglobulin (Ig) G4-related disease is a novel disease entity characterized by diffuse lymphoplasmacytic infiltrates and the presence of abundant IgG4-positive plasma cells in extensive fibrosis, frequently associated with tumorous swelling lesion and elevated serum IgG4 concentrations. Immunoglobulin G4-related disease has been described in almost every organ system but rarely affects the heart. We describe a rare case of IgG4-related disease of the heart causing aortic regurgitation and heart block in a 59-year-old woman. The excised lesion revealed a high ratio of IgG4-positive to IgG-positive plasma cells, providing a definite diagnosis of IgG4-related disease. The aortic valve was replaced using Manouguian technique, resulting in a favorable outcome.
Assuntos
Insuficiência da Valva Aórtica/etiologia , Bloqueio Cardíaco/etiologia , Implante de Prótese de Valva Cardíaca/métodos , Hipergamaglobulinemia/complicações , Imunoglobulina G/sangue , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Ecocardiografia Doppler , Feminino , Seguimentos , Bloqueio Cardíaco/diagnóstico , Bloqueio Cardíaco/cirurgia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Humanos , Hipergamaglobulinemia/diagnóstico , Imuno-Histoquímica , Pessoa de Meia-Idade , Reoperação/métodos , Medição de Risco , Índice de Gravidade de Doença , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/métodosRESUMO
We performed successful ascending-arch aortic replacement and concomitant ascending-to-descending aorta bypass with exclusion of a descending thoracic aneurysm, via median sternotomy, for a ruptured aortic aneurysm involving the entire thoracic aorta. The patient was an 80-year-old man with cardiopulmonary dysfunction and a history of lung tuberculosis. This operation, which has been used for complex descending thoracic aortic lesions such as recoarctation, is a feasible option for a diffuse thoracic aortic aneurysm when single-stage repair is mandatory.
