11/14. Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome.The association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum and interruption or coarctation of the aortic isthmus has not previously been reported as a syndrome. This combination of anomalies was encountered in five new patients and was previously reported in three. Two patients have undergone surgery with successful results. In contrast to the sagittally oriented conventional proximal aortopulmonary septal defect, the patients in this series had a more distal type of defect, possibly representing a partial persistence of the common arterial trunk. The pulmonary arterial bifurcation may malattach to this undivided truncal segment and, as a result, the right pulmonary artery may be partially or completely shifted into the aorta. This abnormal right pulmonary arterial origin may lead to "steal" from the aortic flow during embryogenesis and to hypoplasia of the aortic arch. This concept is supported by the angiographic observation that the greater the rightward displacement of the right pulmonary artery, the greater the hypoplasia of the arch. The diagnostic angiographic sign is a strikingly high origin of the right pulmonary artery together with aortic arch hypoplasia or atresia. Closure of the aortopulmonary septal defect with implantation of the right pulmonary artery in the pulmonary trunk and repair of the aortic arch anomaly is the recommended surgical treatment.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
12/14. Unusual cardiac malformations in splenogonadal fusion-peromelia syndrome: relationship to normal development.A male newborn infant, studied at autopsy, showed continuous splenogonadal fusion and severe peromelia associated with an unusual, possibly unique, cardiac malformation complex. The cardiac lesions included multiple right ventricular diverticula, tricuspid atresia, mitral to semilunar valve discontinuity, and absent muscular outflow tract septum. Tabulation of the time of appearance of relevant anatomic features in 351 normal human embryos of Carnegie stages 9 through 23 showed that the teratogenic influence in the present case probably occurred by stages 16-17. The nature and distribution of lesions observed suggest that proliferating undifferentiated mesenchyme was the target of the unknown injury.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
13/14. Criss-cross heart--a case with horizontal septum, complete transposition, pulmonary atresia and ventricular septal defect.Criss-cross heart is a recently described anomaly in which the systemic and pulmonary blood streams cross at the atrioventricular (AV) level, without mixing. A case of criss-cross heart is described in which the right atrium, in a solitus position, communicated with a left-superior positioned, morphologically right ventricle, and the left atrium communicated with a normally located, morphologically left ventricle. The interventricular septum occupied a horizontal plane. Associated defects were complete d-transposition of the great arteries with l-positioned aorta, pulmonary atresia, ventricular septal defect, atrial septal defect, and patent ductus arteriosus. To the best of our knowledge this is the first angiocardiographic demonstration of this rare combination of lesions. The literature on criss-cross heart and horizontal septum is reviewed. It is stressed that regardless of whether the criss-cross phenomenon is an anatomical fact or an angiocardiographic illusion, it is an established angiocardiographic entity and should be recognized as such.- - - - - - - - - - ranking = 5keywords = atresia (Clic here for more details about this article) |
14/14. tricuspid atresia and pregnancy.tricuspid atresia is one of the rarest heart defects, and it is rarely associated with prolonged survival. This paper describes the third known case of pregnancy associated with tricuspid atresia. This is the first detailed report of the clinical course of a patient with this disorder. Complications included heart failure, multiple pulmonary emboli, cerebral embolization, and premature labor. Recommendations are made regarding patient care.- - - - - - - - - - ranking = 6keywords = atresia (Clic here for more details about this article) |
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