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Variants of the scimitar syndrome

Published online by Cambridge University Press:  16 September 2015

Ilaria Bo Open the ORCID record for Ilaria Bo [Opens in a new window] ,
Julene S. Carvalho ,
Emma Cheasty ,
Michael Rubens  and
Michael L. Rigby
Ilaria Bo*
Affiliation:
Division of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom
Julene S. Carvalho
Affiliation:
Division of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom Fetal Medicine Unit, St George’s University Hospital NHS Foundation Trust, London, United Kingdom St George’s University of London, London, United Kingdom
Emma Cheasty
Affiliation:
Department of Radiology, Royal Brompton Hospital, London, United Kingdom
Michael Rubens
Affiliation:
Department of Radiology, Royal Brompton Hospital, London, United Kingdom
Michael L. Rigby
Affiliation:
Division of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom National Heart and Lung Institute, Imperial College, London, United Kingdom
*
Correspondence to: I. Bo, Royal Brompton Hospital, Sydney Street, London SW3 6NP, United Kingdom. Tel: +0 751 084 4821; Fax: +0 044 207 351 8125; E-mail: [email protected]
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Abstract

Introduction

The scimitar syndrome comprises hypoplastic right pulmonary artery and lung, anomalous right pulmonary venous drainage to the inferior caval vein, aortopulmonary collateral(s) to the right lung, and bronchial anomalies.

Aim

The aim of this study was to describe the morphological and clinical spectrum of variants from the classical scimitar syndrome in a single institution over 22 years.

Results

In total, 10 patients were recognised. The most consistent feature was an aortopulmonary collateral to the affected lung (90%), but there was considerable variation in the site and course of pulmonary venous drainage. This was normal in 3 (one with meandering course), anomalous right to superior caval vein in 1, to the superior caval vein and inferior caval vein in 2, and to the superior caval vein and the left atrium in 1; one patient had a right pulmonary (scimitar) vein occluded at the insertion into the inferior caval vein but connected to the right upper pulmonary vein via a fistula. There were two left-sided variants, one with anomalous left drainage to the coronary sinus and a second to the innominate vein.

Among all, three patients had an antenatal diagnosis and seven presented between 11 and 312 months of age; 90% of the patients were symptomatic at first assessment.

All the patients underwent cardiac catheterisation; collateral embolisation was performed in 50% of the patients. Surgical repair of the anomalous vein was carried out in two patients, one patient had a right pneumonectomy, and one patient was lost to follow-up. There was no mortality reported in the remainder of patients during the study period.

Conclusion

The heterogeneity of this small series confirms the consistent occurrence of an anomalous arterial supply to the affected lung but considerable variation in pulmonary venous drainage.

Keywords

Scimitaranomalous pulmonary venous drainagehypoplastic lungscimitar variant
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 5 , June 2016 , pp. 941 - 947
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Copyright
© Cambridge University Press 2015 

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