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Scimitar Syndrome Explained
Scimitar syndrome (SS) is a rare congenital heart defect characterized by anomalous venous drainage of part or the entire right lung to the inferior vena cava, variable right lung hypoplasia, and variable systemic blood supply to part of the right lung.
Symptoms vary dramatically and are difficult to ascribe to the anomalous pulmonary vein drainage, to the presence of associated cardiac anomalies or to a combination of both. Most patients present with symptoms during early infancy and need prompt surgical intervention.
Treatment decisions are often challenging in patients with isolated SS (i.e. without associated congenital heart defect [CHDs]) who are often incidentally diagnosed during adolescence or adulthood and they usually remain asymptomatic or mildly symptomatic for many years and are able to lead a normal life.
Based on current treatment strategies; surgical correction of SS is beneficial in reducing symptoms. Surgical repair is indicated in symptomatic patients and in patients with significantly increased pulmonary blood flow. The overall survival of patients with SS is high, and survival probability is lower in patients with associated CHDs and in patients with pulmonary hypertension. In asymptomatic patients, it must be tailored to a comprehensive hemodynamic evaluation (i.e. proved considerable pulmonary overload of the scimitar vein drainage) and to the patient’s age because there is a high risk of developing postoperative scimitar drainage stenosis/occlusion. We believe that in selected patients, correction of associated CHDs together with the therapeutic occlusion of SAS to the lung may be beneficial, and so avoid or postpone the need for surgical correction of SS to an older age, and so decrease the risk of late morbidities. A continuous follow-up is indicated.
To learn more about diagnosis and imaging for Scimitar syndrome (SS) download this chapter from author, Vladimiro Vida’s The Complete Reference for Scimitar Syndrome: Anatomy, Epidemiology, Diagnosis and Treatment below:
About the Author
Dr. Vladimiro Vida is a surgeon in the Department of Cardiac, Thoracic and Vascular Sciences at the Pediatric Cardiac Surgery Unit at the University of Padua Medical School in Italy. In 2007 while spending time at the Children’s Hospital in Boston and Harvard Medical School, Dr. Vida was introduced to his first case of Scimitar Syndrome.
He was given the opportunity to coordinate 4 multicenter studies (2 on national and 2 on international basis). These studies focused on the treatment of patients with this syndrome that were finalized with 3 scientific publications (plus one on going, the international scimitar registry collection data of 501 patients with this syndrome). Dr. Vida has also been invited by numerous societies to present the results of his experience. His major research areas include congenital heart disease in adults, congenital heart surgery, minimally invasive surgery, and Scimitar Syndrome.
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