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Surgical Management of the Scimitar Syndrome
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  • Ujjwal Chowdhury,
  • Robert Anderson,
  • Lakshmi Sankhyan,
  • Niwin George,
  • Niraj Pandey,
  • Abhinavsingh Chauhan,
  • Yatin arora,
  • Shikha Goja
Ujjwal Chowdhury
All India Institute of Medical Sciences
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Robert Anderson
1Institute of Genetic Medicine, Newcastle University, Newcastle-upon-Tyne, United Kingdom Birmingham Children’s Hospital, Birmingham, United Kingdom
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Lakshmi Sankhyan
All India Institute of Medical Sciences
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Niwin George
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Niraj Pandey
All India Institute of Medical Sciences
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Abhinavsingh Chauhan
All India Institute of Medical Sciences
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Yatin arora
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Shikha Goja
All India Institute of Medical Sciences
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Abstract

The morphology variations of the so-called scimitar vein are many and varied. We present a synthesis of 92 published investigations of the overall scimitar syndrome. We reviewed the clinical presentations, diagnostic modalities, surgical approaches, and outcomes. 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. In short, they defined the disease 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. 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. 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.

Peer review status:ACCEPTED

15 Jul 2021Submitted to Journal of Cardiac Surgery
16 Jul 2021Assigned to Editor
16 Jul 2021Submission Checks Completed
17 Jul 2021Review(s) Completed, Editorial Evaluation Pending
18 Jul 2021Editorial Decision: Accept