RESUMO
We studied 15 cases of the scimitar syndrome. The diagnosis was suspected in 87% of the patients by x-rays studies. Bronchography is useful in order to detect bronchial anomalies, such as hypoplasia or agenesis of the medial lobe which is responsible for the left isomerism. The hemodynamic study is important in symptomatic patients in order to demonstrate associated congenital heart disease as well as the degree of pulmonary hypertension. Angiography is fundamental in determining the anomalous venous drainage of the right lung, whether complete or partial, and in demonstrating the exact site of the connection which may be to the right atrium or more frequently to the inferior vena cava above or below the diaphragm. There are variant forms in so far as the pathway, extension and connection of the anomalous venous trunk which gives rise to the scimitar sign. This syndrome is due to a persistent connection of the right pulmonary veins to the proximal portion of the right vitelin vein which normally forms the suprahepatic segment of the inferior vena cava. Asymptomatic patients should have medical treatment symptomatic ones with an associated congenital heart disease and moderate pulmonary hypertension must be subject to surgery.
Assuntos
Síndrome de Cimitarra , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Radiografia , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/patologia , Síndrome de Cimitarra/fisiopatologiaRESUMO
We studied ten patients with unilateral absence of main branch of pulmonary artery (UAMBPA). Six patients with absent right pulmonary artery (ARPA) and four with the left pulmonary artery (ALPA). The presence of either atrial septal defect, ventricular septal defect and patent ductus arteriosus alone or combinated were associated with pulmonary hypertension (mean pressure of the pulmonary artery greater than 48 mm Hg). The patients without associated cardiac defects were asymptomatic and with little elevation of the pulmonary arterial pressure (mean pressure of the pulmonary artery less than 26 mmHg).
Assuntos
Artéria Pulmonar/anormalidades , Adolescente , Adulto , Cateterismo Cardíaco , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Lactente , MasculinoRESUMO
We studied 15 patients with the diagnosis of tetralogy of Fallot by means of conventional and axial angiocardiography. Axial studies were performed in the four chamber view (vertical and horizontal X-ray beams) and in the sitting-up view (vertical X-ray beam). With the right ventriculogram we analyzed the anatomic characteristics of this chamber, infundibular stenosis and the pulmonary branches. Left ventriculograms were performed in order to rule out other less common associated defects. In the sitting-up view we analyzed the characteristics of the right ventricular infundibulum and the pulmonary valve, artery and branches. None of our patients underwent aortography or coronary artery angiography. We conclude that utilization of axial angiocardiography is useful in tetralogy of Fallot because it permits a better appreciation of the anatomy of this malformation, the presence or absence of stenosis of the pulmonary branches and other less frequent associated defects. Aortography or coronary artery angiography must be performed in children in which the anatomy of the coronary arteries cannot be determined by either left or right ventriculograms.
Assuntos
Angiocardiografia , Tetralogia de Fallot/diagnóstico por imagem , Angiocardiografia/métodos , Criança , Pré-Escolar , Feminino , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Humanos , Lactente , Masculino , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/patologia , Tetralogia de Fallot/patologiaRESUMO
Se estudiaron 15 pacientes con tetralogia de Fallot por medio de angiografia convencional y axial. El estudio axial se realizo en 2 planos en la posicion cuatro camaras, y monoplano en la posicion semisentada. En la inyeccion en el ventriculo derecho se analizo la anatomia del ventriculo derecho, la estenosis infundibular y las caracteristicas de las ramas pulmonares.El ventriculograma izquierdo se utilizo para demostrar otras malformaciones agregadas. Con la posicion semisentada se revisaron las caracteristicas del infundibulo pulmonar, valvula, tronco y ramas pulmonares. En ningun paciente se realizo aortografia o coronariografia. Se concluye que las posiciones axiales para el estudio de la tetralogia de Fallot permiten una mejor valoracion de la anatomia de la misma, la presencia o ausencia de estenosis en el origen de las ramas pulmonares, y otras malformaciones agregadas menos frecuentes. Se enfatiza la necesidad de realizar aortografia o coronariografia selectiva cuando no se puede determinar las caracteristicas del arbol coronario en estos pacientes
Assuntos
Lactente , Pré-Escolar , Criança , Humanos , Masculino , Feminino , Angiocardiografia , Tetralogia de FallotRESUMO
It is presented a pulmonary artresia case with untouched interventricular partition wall surgically treated. It was established the continuity between right ventricle and pulmonary artery's trunk with duramater ceiling, for which it was made a pulmonary valve. Postoperation evolution was excelent and it was obteined a frank diminution of the right ventricle's systolic pression. It is discussed about therapeutic conducts in this kind of congenital badformation.