12 Left circumflex alcapa in scimitar syndrome – an under-recognised association?

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Abstract

Introduction

Scimitar syndrome is a well-known, but exceedingly rare condition, characterised by anomalous pulmonary venous return of all or part of the right lung to the IVC, normally in association with right lung hypoplasia, dextroposition of the heart and right pulmonary artery hypoplasia. Anomalous coronary anatomy is not, however, a well-described association. We reviewed the coronary anatomy of all patients diagnosed with scimitar syndrome, at a single institution, over a 24-year period.

Methods

Retrospective review of medical records, cardiac imaging and operative notes of all patients diagnosed with scimitar syndrome at a single institution between 1992 and 2016.

Results

54 patients were identified. Within this cohort 3 patients (5.5%, 1 male, 2 female) had anomalous origin of the left circumflex coronary artery from the pulmonary artery (ALCAPA), all arising close to the pulmonary bifurcation.

Conclusions

There are many common and uncommon variants of scimitar syndrome. The relatively high incidence (5.5%) of anomalous origin of the left circumflex coronary from the pulmonary artery is not well described and should be borne in mind when reviewing cross-sectional imaging of these patients, particularly during the neonatal period, when coronary anatomy is often particularly difficult to assess.

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