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Surgical management of the scimitar syndrome
Author(s) -
Chowdhury Ujjwal K.,
Anderson Robert H.,
Sankhyan Lakshmi K.,
George Niwin,
Pandey Niraj N.,
Chauhan Abhinav S.,
Arora Yatin,
Goja Shikha
Publication year - 2021
Publication title -
journal of cardiac surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.428
H-Index - 58
eISSN - 1540-8191
pISSN - 0886-0440
DOI - 10.1111/jocs.15857
Subject(s) - medicine , scimitar syndrome , intracardiac injection , tracheobronchomalacia , radiology , hypoplasia , pulmonary vein stenosis , pulmonary hypertension , heart failure , magnetic resonance imaging , surgery , cardiology , atrial fibrillation , pulmonary vein , bronchoscopy , inferior vena cava
Background and Aim 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. Methods 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. Results 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. Despite timely surgical intervention, post‐repair obstruction of the scimitar vein, intra‐atrial baffle obstruction, or stenosis of the inferior caval vein were reported in up to two‐thirds of cases. The venous obstruction could not be related to any particular surgical technique. On long term follow‐up, one sixth of patients reported persistent dyspnoea and recurrent respiratory infections. Conclusions Any infants presenting with heart failure, right‐sided heart, and hypoplastic right lung should be evaluated to exclude the syndrome. An increased appreciation of variables will contribute to improved surgical management.