ABSTRACT
In double-outlet atrium either the right or the left atrium empties into both ventricles while the other atrium remains disconnected from the ventricular cavities. Cases of double-outlet atrium with a single atrioventricular (AV) valve have been reported but no cases of double-outlet left atrium and only two of double-outlet right atrium (one with three valves) have been reported. A description of the second case of double-outlet right atrium with two AV valves and a review of the other reported case are presented. Common anatomic characteristics were: two AV valves in the right atrium that connected with two normal ventricles; disconnection of the left atrium from the ventricular cavities; an atrial septal defect in a malpositioned atrial septum; and drainage of a left superior vena cava into the left atrium. Mitral incompetence was found in both cases. Clinical findings were similar to those seen in ostium primum atrial septal defect. Two-dimensional echocardiography and angiocardiography are able to accurately demonstrate this entity. Successful surgical correction was performed in both cases. Two morphogenetic hypotheses are proposed: abnormal development of the septum primum or primitive malposition of the common atrioventricular canal.
Subject(s)
Heart Defects, Congenital/pathology , Cardiac Catheterization , Child , Echocardiography , Heart Atria/abnormalities , Heart Defects, Congenital/surgery , Heart Septal Defects, Atrial/pathology , Heart Valves/abnormalities , Humans , Male , Vena Cava, Superior/abnormalitiesSubject(s)
Ebstein Anomaly/classification , Angiocardiography , Child , Child, Preschool , Ebstein Anomaly/complications , Ebstein Anomaly/surgery , Electrocardiography , Heart Septal Defects, Ventricular/complications , Heart Septal Defects, Ventricular/surgery , Humans , Infant , Infant, Newborn , Methods , Pulmonary Valve Insufficiency/complications , Pulmonary Valve Insufficiency/surgeryABSTRACT
One case of anomalous origin of the right pulmonary artery from ascending aorta is reported. The anomalies in the origin of the main pulmonary branches are classified in Type A: Anomalous origin of a pulmonary artery from ascending aorta; Type B: Anomalous origin of a pulmonary artery from the aortic arch through a ductus arteriosus, and Type C: Absence of a pulmonary artery with pulmonary vasculariation through systemic arteries arising from descending aorta. The normal embryology of the 6th. aortic arch and the pulmonary branches and the differents theories postulated about the embryopathogenesis of these malformations are reviewed. The outstanding clinical, hemodynamic, angiocardiographic and surgical features are also discussed.
Subject(s)
Aorta/abnormalities , Pulmonary Artery/abnormalities , Aorta, Thoracic/diagnostic imaging , Female , Humans , Infant, Newborn , Pulmonary Artery/diagnostic imaging , RadiographyABSTRACT
112 cases of coarctation of the aorta and 8 cases of tubular hypoplasia of the aortic isthmus operated upon in the Children's Hospital "La Paz" from Madrid, are reviewed. All children were under 7 years of age. 64.2% of the cases of aortic coarctation were in the first year of life, 47.3% of them had associated lesions, being the most frequently present persistent ductus arteriosus and ventricular septal defect. Hospital mortality was 14.2%, what is considered as very acceptable. All the children operated upon for the correction of tubular hypoplasia of the aortic isthmus were in the first year of age. 75% of them had associated ductus arteriosus and ventricular septal defect, being hospital mortality of 62.5%. Most frequent postoperative complications and cause of death were due to broncopulmonary disorders secondary to the existence of a previous pulmonary hypertension.
