1/4. The angiocardiographic appearance of the endocardial cushion defect in selected transposition and malposition complexes.Although the angiocardiographic features of the endocardial cushion defect (ECD) have been well described in the patient with two normally related ventricles (D-ventricular loop in situs solitus) and normally related great arteries, little attention has been focused on the angiocardiographic appearance of the ECD in patients with complex cardiovascular disease. Because of recent surgical advances in the treatment of single ventricle and double outlet right ventricle complexes, it has become increasingly important to document the status of the atrioventricular valves in these patients. The angiocardiographic features of the ECD are therefore described in two patients, one with D-loop, single left ventricle and outlet chamber, D-transposition of the great arteries, bilateral conus and pulmonary stenosis and the other with dextrocardia, situs inversus, double outlet right ventricle, common atrioventricular canal and pulmonary stenosis. In both patients, the left ventricular outflow tract, despite aortic-mitral discontinuity, is characterized by elongation, scalloping and serration, and the classic appearance of the "gooseneck" deformity is observed on left ventricular angiography. It thus appears that aortic-mitral continuity and two well-formed, normally related ventricles and great arteries are not invariable necessary to the manifestation of the "gooseneck" deformity in the patient with complex cardiovascular disease and associated endocardial cushion defect.- - - - - - - - - - ranking = 1keywords = dextrocardia (Clic here for more details about this article) |
2/4. Familial dextrocardia, divergent strabismus and situs inversus of optic disc.A family is described in which three of four siblings have major congenital cardiac defects in association with ocular abnormalities. The eldest sibling has isolated dextrocardia and the second has total situs inversus. The fourth child has both atrial and ventricular septal defects, with pulmonary hypertension and right ventricular hypertrophy. The mother and third sibling have normal hearts but exhibit a number of ocular defects. The principal ocular anomalies demonstrated in this family are divergent strabismus, bilateral situs inversus of the optic disc, and myopia. The hereditary aspect of these multiple findings is discussed.- - - - - - - - - - ranking = 5keywords = dextrocardia (Clic here for more details about this article) |
3/4. Successful surgical repair of solitus, dextrocardia, atrioventricular discordance, and double outlet right ventricle with l-malposition of the aorta.The authors report a rare case of situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, atrial and ventricular septal defects, origin of both great arteries from the morphologic right ventricle, pulmonary valvar and subvalvar stenosis and l-malposition of the aorta which has been correctly diagnosed and succesfully operated on. The repair was accomplished by closing the ventricular septal defect through a ventriculotomy in the systemic venous ventricle (morphologic left), and placing a conduit between this ventricle and the pulmonary artery which had been ligated proximally. The conduit was a fresh segment of homograft valved ascending aorta fixed on a Dacron woven tube. The authors discuss: firstly the reasons for performing this type of repair instead of the intraventricular one recommended by the Mayo Clinic group in the past and secondly the peculiar location of the conducting tissue and the method of avoiding it when approaching the ventricular septal defect.- - - - - - - - - - ranking = 5keywords = dextrocardia (Clic here for more details about this article) |
4/4. Successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance associated with dextrocardia and pulmonary stenosis.The association of complete atrioventricular canal with other complex congenital cardiac anomalies has represented a significant challenge for the cardiac surgion. The combination of double-outlet right ventricle with complete atrioventricular canal has been particularly difficult to correct, with no surgical successes having been reported until recently. This is a report of the first successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance (ventricular incersion) associated with common atrium, bilateral superior venae cavae, dextrocardia, and pulmonary stenosis. The specialized conduction tissue was identified by intracardiac electrophysiological mapping, and normal sinus rhythm was preserved. Postoperative cardiac catheterization showed excellent hemodynamics. One year postoperatively, the patient was attending school, playing swimming without difficulty, and taking no cardiac medications. This good result lends encouragement for considering total repair for similar patients with the combination of double-outlet right ventricle, complete atrioventricular canal, and other associated congenital cardiac anomalies.- - - - - - - - - - ranking = 5keywords = dextrocardia (Clic here for more details about this article) |