Surgical management of the scimitar syndrome.

Journal of Cardiac Surgery
Ujjwal K ChowdhuryShikha Goja


We sought to address the varied anatomical details, the diagnostic challenges, associated cardiopulmonary anomalies, the techniques, and outcomes of management, including re-interventions of scimitar syndrome. A total of 92 published investigations of scimitar syndrome were reviewed. Diagnostic information was provided by clinical presentations, radiographic findings, transthoracic and transesophageal echocardiography, computed-tomographic angiography, magnetic resonance imaging, angiocardiography, and ventilation/perfusion scans. These investigations served to elucidate the origin, course, and termination of the scimitar vein, the intracardiac anatomy, the presence of associated defects, and the patterns of any accompanying pulmonary lesions prior to surgical intervention. Of the patients described, up to four-fifths presented during infancy, with cardiac failure, increased pulmonary flow, and pulmonary hypertension. Associated cardiac and extracardiac defects, particularly hypoplasia of the right lung, are present in up to three-quarters of cases. Overall operative mortality has been cited between 4.8% and 5.9%. Mortality was highest in patients with preoperative pulmonary hypertension, and those undergoing surgery in infancy...Continue Reading


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