RESUMO
Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.
Assuntos
Humanos , Lactente , Veias Pulmonares/cirurgia , Veias Pulmonares/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/diagnóstico por imagem , Cardiopatias Congênitas , Constrição Patológica , Estenose de Veia Pulmonar/cirurgia , Estenose de Veia Pulmonar/etiologia , Estenose de Veia Pulmonar/diagnóstico por imagemAssuntos
Humanos , Masculino , Valva Pulmonar/anormalidades , Tosse , Radiografia Torácica , Tomografia Computadorizada por Raios X , Comunicação Interatrial , Ecocardiografia , Recidiva , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/terapiaRESUMO
Se presenta el caso de un masculino de 15 meses de edad con síndrome de cimitarra y atresia pulmonar con comunicación interventricular. El diagnóstico se hizo mediante cateterismo cardíaco y angiocardiografía y confirmado por el estudio de autopsia. Se hacen las consideraciones clínicas y quirúrgicas de esta excepcional asociación que a la fecha es, hasta donde sabemos la primera reportada en la literatura.
We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.