https://www.um.edu.mt/library/oar/handle/123456789/3608 2026-04-09T13:59:39Z https://www.um.edu.mt/library/oar/handle/123456789/3953 Title: Late presentation and successful treatment of classical scimitar syndrome Authors: Grech, Victor E.; Xuereb, R.; Xuereb, M.; Manche, Alexander; Schembri, K.; DeGiovanni, Joseph V. Abstract: Scimitar syndrome is a form of partial anomalous pulmonary venous drainage that is dramatically visible on plain chest radiography (CXR). In these individuals the entire venous drainage from the right lung enters a single anomalous large vein that descends to the inferior vena cava. This descending vein is visible on CXR as a curvilinear density along the right heart border and resembles the curved Turkish sword that gives the condition its name. Scimitar syndrome forms part of the large spectrum of associated conditions known as venolobar syndrome. These include right lung hypoplasia or sequestered segments of right lung, congenital heart disease and various others. We report the case of a young woman who presented incidentally, with a murmur, at 16 years of age. Full investigation including angiography showed a large atrial septal defect with right heart dilation and scimitar syndrome. She underwent surgical correction with uneventful and complete correction by baffling of the scimitar vein from its entry into the inferior vena to the left atrium through the enlarged atrial septal defect. 2003-01-01T00:00:00Z https://www.um.edu.mt/library/oar/handle/123456789/3941 Title: The use of anticoagulation in pediatric cardiac disease Authors: Boris, Jeffrey R.; Harris, Matthew A. Abstract: Palliation and repair of increasingly complex congenital heart defects as well as the emergence of novel contexts has led to multiple scenarios in which a real or potential risk of thromboembolism may exist. While various anticoagulation methodologies have been well defined for adults, there are few studies relating directly to pediatric patients. This article reviews a number of specific pediatric disease states, the representative pediatric literature, and, where appropriate, the corresponding adult literature. In so doing, the art and science of pediatric cardiac anticoagulation is defined with the hope to engender further thought regarding future directions of study and therapy. 2003-01-01T00:00:00Z https://www.um.edu.mt/library/oar/handle/123456789/3940 Title: A review of congenital heart block Authors: Friedman, Deborah M.; Glickstein, J.; Duncanson, L.J.; Buyon, Jill P. Abstract: Congenital heart block is a rare disorder. It has an incidence of about 1 in 22,000 live births. It may be associated with high mortality and morbidity. This should generate a high index of suspicion for early diagnosis and aggressive therapy when appropriate. The congenital heart block associated with neonatal lupus is considered a form of passively acquired autoimmune disease in which maternal autoantibodies to the intracellular ribonucleoproteins Ro (SS-A) and La (SS-B), cross the placenta and injure the previously normal fetal heart. Women with serum titers of anti-Ro antibody carry a 3% risk of having a child with neonatal lupus syndrome. Recurrence rates are about 18%. We believe that serial echocardiograms should be acquired so that early diagnosis is made and aggressive therapy administered, if signs of conduction system disease such as PR interval prolongation by Doppler are found, so as to optimize the outcome. Establishment of guidelines for therapy have been set empirically, should signs of congenital heart block develop. Those patients whose congenital heart block is associated with structural heart disease have a higher morbidity and mortality, which is determined more by the underlying structural congenital heart disease than it is by the need for a pacemaker per se. 2003-01-01T00:00:00Z