RESUMO
Mixed connective tissue disease (MCTD) syndrome in children may lead to large aortic aneurysms, which in turn pose a difficult surgical problem. Valve-sparing root replacement is not always a viable option as the disease process invariably affects the aortic valve leaflets. Among pediatric patients, the Ross procedure is contraindicated on account of weakness of the pulmonary root, while Bentall surgery is the 'gold standard' treatment of aortic aneurysm, with reproducible and excellent long-term results. The case is presented of a three-year-old girl with a large thoracic aortic aneurysm in whom Bentall's surgery was performed, with a good result. The present patient, with MCTD syndrome, was too young to have undergone aortic root replacement with a composite mechanical valved graft.
Assuntos
Aneurisma da Aorta Torácica/cirurgia , Valva Aórtica/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Doença Mista do Tecido Conjuntivo/complicações , Alotransplante de Tecidos Compostos Vascularizados/métodos , Aneurisma da Aorta Torácica/diagnóstico , Aneurisma da Aorta Torácica/etiologia , Aneurisma da Aorta Torácica/fisiopatologia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/patologia , Pré-Escolar , Feminino , Humanos , Resultado do TratamentoRESUMO
Abnormalities in the aorta of patients with tetralogy of Fallot (TOF) can lead to aortic dilatation and aortic regurgitation. We report a six-year-old male with TOF with dysplastic aortic valve, severe aortic regurgitation, and aortic aneurysm involving the aortic root, ascending aorta, and the proximal aortic arch, who underwent TOF repair with aortic replacement up to the proximal aortic arch with a composite graft without circulatory arrest.