1/81. Coil occlusion of aortopulmonary collateral arteries in an infant with scimitar syndrome. scimitar syndrome in infancy is a rare condition, presenting with severe congestive heart failure and pulmonary hypertension. The presence of large systemic-pulmonary collateral arteries may play a role in the cause of heart failure and pulmonary hypertension. A 4-month-old infant underwent coil occlusion of large anomalous systemic arteries supplying the right lower pulmonary lobe. Symptoms of severe congestive heart failure and pulmonary hypertension improved dramatically with coil occlusion, and surgical correction was performed 3 months later without any complications. Coil occlusion of anomalous systemic arteries can improve symptoms of heart failure and pulmonary hypertension in infants and may bring about a good surgical result for this disease. ( info) |
2/81. scimitar syndrome with pulmonary arteriovenous fistulas. Right abnormal pulmonary venous return into the inferior vena cava associated with abnormal fissure, dextrocardia, and systemic arterial supply of a variable degree, are the characteristics of the scimitar syndrome. We report on a patient in whom this rare syndrome was associated with pulmonary arteriovenous fistulas within the involved lung. ( info) |
| We present a case of tetralogy of fallot associated with scimitar syndrome. The patient was an 11-month old female who underwent successfully total repair of her lesion, including rerouting of the anomalous pulmonary vein to the left atrium. The diagnosis was suspected from the chest x-ray and echocardiography, and confirmed by angiography. To the best of our knowledge only 2 additional cases have previously been reported. ( info) |
4/81. Acquired anomalous intrapulmonary venous connection secondary to pulmonary venous stenosis. An unusual case of acquired development of anomalous intrapulmonary venous connection with pulmonary venous stenosis is presented. Appearances on a chest radiograph resembled the "scimitar" sign in a patient with previous surgery for partial anomalous pulmonary venous return. Spiral CT and pulmonary arteriography showed stenosis of the right upper pulmonary vein and an anomalous intrapulmonary venous connection between the right upper pulmonary vein and the right lower pulmonary vein. We consider the slow progression of pulmonary vein stenosis led to anomalous intrapulmonary venous connection as an intrapulmonary collateral. ( info) |
5/81. New interventional therapeutic approach for dual drainage of the scimitar vein. A patient with scimitar syndrome and dual drainage of a right-sided scimitar vein into the inferior vena cava and the left atrium underwent coil occlusion of the right aortopulmonary collateral artery and device occlusion of the lower scimitar vein drainage, leaving it flowing solely into the left atrium. ( info) |
| We report the 1st magnetic resonance demonstration of both an anomalous pulmonary venous drainage and systemic arterial supply in a patient with scimitar syndrome. With its superior imaging capabilities, gadolinium-enhanced magnetic resonance angiography provides a powerful diagnostic tool for this complex congenital lesion and offers the possibility of surgical repair and follow-up without conventional cardiac catheterization. ( info) |
7/81. The multiple facets of pulmonary sequestration. PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. methods: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations. ( info) |
8/81. Retrospective prenatal diagnosis of scimitar syndrome aided by three-dimensional power Doppler imaging. scimitar syndrome is a rare malformation of the arterial supply and venous drainage of the lung. We report the case of a fetus that presented with cardiac asymmetry and malposition of the fetal heart. Postnatally, scimitar syndrome was confirmed at cardiac catheterization. Retrospective reconstruction of three-dimensional power Doppler volumes, obtained during fetal life, allowed direct visualization of the abnormal aortopulmonary collateral vessel. This had not been seen on conventional scans. This case demonstrates many of the strengths of three-dimensional sonographic techniques for the delineation of complex vascular anatomy. It confirms that a prospective diagnosis of scimitar syndrome should be possible during fetal life. ( info) |
9/81. A sword for the left hand: an unusual case of left-sided scimitar syndrome. scimitar syndrome is a rare anomaly involving abnormalities of the heart and lung which classically involves the right side. A rare case of left-sided scimitar syndrome is described in an asymptomatic child, with a review of the literature. ( info) |
| scimitar syndrome and Wolff-Parkinson-White association has not been reported during the era of catheter ablation. We present our personal experience with percutaneous radiofrequency ablation, a technical challenge related with Scimitar's anatomical variants and follow-up after surgical repair of the latter. ( info) |