RESUMO
Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.
Assuntos
Veias Hepáticas/anormalidades , Síndrome de Cimitarra/diagnóstico , Anormalidades Múltiplas , Cateterismo Cardíaco , Ecocardiografia , Feminino , Humanos , Imageamento por Ressonância Magnética , Radiografia Torácica , Adulto JovemRESUMO
Pentalogy of Cantrell is a rare condition with a varied expression and a high mortality. We present a patient with the classic pentad (type 1), but with a previously undescribed constellation of cardiac manifestations including ectopia cordis totalis, total anomalous pulmonary venous return, and tetralogy of Fallot. This case reminds us of the challenges associated with the management of various forms of this condition. We discuss the prenatal diagnosis, genetic basis, postnatal evaluation, and management of this entity.
Assuntos
Anormalidades Múltiplas , Ectopia Cordis , Pentalogia de Cantrell , Veias Pulmonares/anormalidades , Tetralogia de Fallot , Ecocardiografia , Ectopia Cordis/diagnóstico , Ectopia Cordis/fisiopatologia , Ectopia Cordis/terapia , Evolução Fatal , Feminino , Humanos , Recém-Nascido , Pentalogia de Cantrell/diagnóstico , Pentalogia de Cantrell/fisiopatologia , Pentalogia de Cantrell/terapia , Flebografia/métodos , Gravidez , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/fisiopatologia , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/terapia , Tomografia Computadorizada por Raios X , Ultrassonografia Pré-Natal/métodos , Adulto JovemRESUMO
A mycotic aneurysm is a rare condition occasionally seen in patients with a history of prior cardiac or vascular surgery. Here we report the presentation of a mycotic aneurysm in a pediatric patient at the site of prior aortic coarctation repair. This patient's initial presentation suggested rheumatologic or oncologic disease, and after diagnosis he continued to show evidence of splenic, renal and vascular injury distal to the mycotic aneurysm site while being treated with antibiotics. We discuss the diagnosis, treatment and management of this condition.