RESUMO
Se expone el caso de un adolescente de 14 años de edad, con síndrome de Marfan y antecedente de tres cirugías cardiovasculares previas: valvuloplastia aórtica y mitral a los cinco años y valvuloplastia aórtica y reconstrucción de la aorta torácica con tubo de pericardio bovino a sus diez años. En primer tiempo quirúrgico se realizó reemplazo valvular aórtico por válvula mecánica y valvuloplastia mitral y tricuspidea, y en segundo tiempo quirúrgico, durante la misma hospitalización, exclusión endovascular de aneurisma de aorta descendente asintomático sin complicaciones. Antes del egreso se diagnosticó una endofuga tipo II que se manejó con observación clínica. Luego de un año del procedimiento, los controles clínico y tomográfico son satisfactorios.
We describe the case of a 14-year-old adolescent with Marfan syndrome and a history of three previous cardiovascular surgeries: aortic and mitral valve replacement at the age of 5 and aortic valve replacement and reconstruction of the thoracic aorta with a tube of bovine pericardium at the age of ten. In the first surgical procedure the aortic valve was replaced by a mechanical valve, and mitral and tricuspid valvuloplasty was performed. In a second surgical procedure during the same hospitalization, endovascular exclusion of the asymptomatic descending aortic aneurysm was realized without complications. Before discharge, a type II endoleak was diagnosed and managed through clinical observation. After a year of the procedure, clinical and tomographic controls are satisfactory.
Assuntos
Artérias , Procedimentos Endovasculares , Cirurgia TorácicaRESUMO
Right pulmonary artery to left atrial fistula is a rare pathology characterised by a right to left shunt. Another important aspect of this pathology is the difficulty encountered in making a diagnosis, which is why the diagnosis is frequently delayed into adulthood. A description of two cases is used to emphasise the importance of the different modes of echocardiography as a diagnostic tool in diagnosis, as well as the two different clinical forms that it adopts: a group of patients suffering cardiac failure and cyanosis without apparent cause generally in neonates and a second group of mostly older patients with dyspnoea and cyanosis without apparent cause. Symptoms thus differ depending on the time of presentation and are related to the size of the fistula.
Assuntos
Fístula Arteriovenosa/diagnóstico , Átrios do Coração/anormalidades , Artéria Pulmonar/anormalidades , Fístula Arteriovenosa/classificação , Fístula Arteriovenosa/complicações , Pré-Escolar , Angiografia Coronária/métodos , Diagnóstico Precoce , Ecocardiografia/métodos , Átrios do Coração/diagnóstico por imagem , Humanos , Recém-Nascido , Masculino , Artéria Pulmonar/diagnóstico por imagem , Radiografia TorácicaRESUMO
La pentalogía de Cantrell involucra defectos de la línea media abdominal supraumbilical, parte inferior del esternón, diafragma anterior, pericardio diafragmático y malformaciones congénitas intracardiacas (1, 2). Su pronóstico está supeditado al diagnóstico temprano, la severidad de la malformación cardiaca y las anomalías asociadas (3). Se reporta el caso de una gestante de 28 semanas con diagnóstico fetal de pentalogía de Cantrell: ectopia cordis verdadera toraco-abdominal (con anomalía intracardiaca) y fisura esternal, y se resalta el diagnóstico prenatal que permitió definir su pronóstico y planear el manejo.
Pentalogy of Cantrell involves defects of the midlline supraumbilical abdomen, lower sternum, anterior diaphragm, diaphragmatic pericardium and intracardiac congenital defects. Prognosis depends on early diagnosis, severity of cardiac malformation and associated anomalies. We report the case of a 28 weeks pregnant woman with fetal diagnosis of pentalogy of Cantrell: true thoraco-abdominal ectopia cordis (with intracardiac anomaly) and sternal cleft, and highlight prenatal diagnosis that allowed to define prognosis and plan management.