| Combined scimitar syndrome and coronary artery disease is a rare combination of congenital and acquired cardiac disease in adults. Hence, no guidelines for surgical correction are available. We report on the case of a 60-year-old man with coronary artery disease and scimitar syndrome. The patient underwent arterial coronary revascularization and simultaneous correction of the scimitar syndrome utilizing a new surgical approach with a modified pericardium-baffle reconstruction of the anomalous right pulmonary vein. ( info) |
12/81. Obstructed hemianomalous pulmonary venous drainage: is intervention necessary? Obstructed infradiaphragmatic hemianomalous pulmonary venous drainage is associated with high mortality and morbidity, and treatment is best tailored to the individual patient. Resolution of an obstructed scimitar vein through collateralization has rarely been reported. We report two such cases and review the literature. ( info) |
13/81. Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies. We report a case of a healthy, asymptomatic 6-year-old boy in whom an anomalous right pulmonary vein was noted to drain into both the inferior vena cava and left atrium in association with findings consistent with scimitar syndrome. The anomalous pulmonary vein took a very circuitous route through the lungs before draining into the left atrium, a condition previously termed "meandering pulmonary vein." To aid in the diagnosis, cardiovascular magnetic resonance imaging and magnetic resonance angiography were used to delineate this complex course and the connection of the anomalous pulmonary vein. To our knowledge, this is the 1st reported case of a meandering pulmonary vein with dual drainage to the inferior vena cava and left atrium in association with other anomalies. ( info) |
14/81. Scimitar vein anomaly with multiple cardiac malformations, craniofacial, and central nervous system abnormalities in a brother and sister: familial scimitar anomaly or new syndrome? The scimitar vein "syndrome" is an anomaly of lobar aplasia or hypoplasia and total or partial anomalous venous drainage of one lung. We report a brother and sister born to nonconsanguineous Italian parents with a fatal infantile form of scimitar vein anomaly associated to multiple cardiac anomalies. The infants had major craniofacial anomalies. In addition, the boy had iris coloboma and enlarged cisterna magna; both sibs showed poor brain myelination at neuroimaging confirmed by histopathology in the girl. The cardiovascular system in the family members was fully investigated and all results were completely normal. The association of the above craniofacial anomalies has been occasionally mentioned in syndromes with anomalous venous return. The familial occurrence of isolated total anomalous pulmonary venous return has been documented in sibs, first cousins, and through consecutive generations. Familial pulmonary hypoplasia, as an isolated finding, has been observed in siblings and twins. To the best of our knowledge, even though five familial cases of scimitar "syndrome" have been described thus far, the constellation of anomalies shown by these two sibs has not been reported previously. It appears that scimitar vein "syndrome" is not a "syndrome" per se: it is most likely an anomaly of heterogeneous etiology. This family may represent its own novel syndrome of multiple congenital anomalies of which scimitar vein is a component. ( info) |
15/81. MRI of partial anomalous pulmonary venous return (scimitar syndrome). We report a case of anomalous pulmonary venous drainage into the inferior vena cava (scimitar syndrome). Cine MRI and 3-D contrast-enhanced MR angiography provides an non-invasive diagnostic technique in the evaluation of anomalous pulmonary venous return. ( info) |
| INTRODUCTION: Intravenous epoprostenol is frequently administered in adults and children for treatment of pulmonary hypertension. Although generally safe, pulmonary edema has been described in a few case reports of adult patients with pulmonary veno-occlusive disease. CASE REPORT: We present an infant who had an operation for scimitar syndrome and abnormal drainage of the right pulmonary veins into the inferior vena cava who developed pulmonary edema while receiving a prostacyclin infusion. The typical partial anomalous pulmonary venous drainage was operatively corrected at 6 days of age, and an accompanying coarctation was resected. At 7 months of age, diagnostic cardiac catheterization was performed to evaluate suspected pulmonary hypertension. Pulmonary pressure was elevated to supra-systemic values, and obstructed venous drainage of the right hypoplastic lung was demonstrated. To decrease pulmonary hypertension during weaning and extubation, epoprostenol infusion was initiated. Sixty minutes after extubation, massive acute pulmonary edema lead to reintubation. Mean airway pressure of 16 mm Hg (21 mbar) with pure oxygen ventilation was initially required, with an oxygenation index of 14, a ventilation index of 36, and an alveolar-arterial oxygen tension difference of 541 mm Hg. After discontinuation of epoprostenol, weaning and extubation was successful. CONCLUSION: pulmonary edema caused by prostacyclin infusion in patients with impaired postcapillary pulmonary drainage may also be encountered in children and has to be anticipated. ( info) |
17/81. Bispectral index in a 3-year old undergoing deep hypothermia and circulatory arrest. We report a 3-year-old girl who presented with scimitar syndrome and underwent hypothermic circulatory arrest for correction of anomalous pulmonary veins and an atrial septal defect. In this case the Bispectral Index (BIS) correlated significantly with the gradual onset of hypothermia and circulatory arrest. However, BIS remained low during the rewarming phase of cardiopulmonary bypass, in spite of adequate pump flows and stable haemodynamics. We postulate that this significant lag in BIS during the rewarming phase of deep hypothermic circulatory arrest may represent neuronal bewilderment or perhaps stunning, and differs from previous studies that show significant increase in BIS during rewarming from mild hypothermia. ( info) |
18/81. A novel operative approach to scimitar syndrome. scimitar syndrome is a rare congenital cardiac anomaly defined by an anomalous right pulmonary vein draining the right lung to the inferior vena cava. We describe a unique operative approach performed on 2 patients with infantile scimitar syndrome. ( info) |
19/81. scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI. We report an asymptomatic 8-year-old girl with anomalous pulmonary venous connection to the IVC and systemic arterial supply (scimitar syndrome). We present for the first time a description of gadolinium-enhanced 3D MR angiography that provided concurrent non-invasive complete anatomical (arterial and venous supply) and 'functional' (calculation of left-to-right shunt using phase-contrast-MRI performed in the ascending aorta, main pulmonary artery and anomalous pulmonary vein) diagnosis, avoiding the need for more traditional invasive techniques. As the shunt quantification was less than 2:1, conservative management was decided upon. ( info) |
20/81. A case series of five infants with scimitar syndrome. scimitar syndrome is a rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection of the right lung to the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply to the right lung. To date, this syndrome has not been reported or discussed in the nursing literature. In this article, 5 patients are presented to illustrate the variable presentation of this syndrome, ranging from infants who are asymptomatic to those with heart failure and severe pulmonary hypertension. The clinical signs and symptoms, diagnostic dilemmas, and medical and surgical management are discussed. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to the possibility of this syndrome. ( info) |