Multimodality Approach to a Complex Scimitar Syndrome: How Advanced Diagnostics Can Guide Therapeutic Strategies

Alberto Clemente; Gaia Viganò; Luigi Festa; Ettore Remoli; Chiara Marrone; Duccio Federici; Vitali Pak; Dante Chiappino; Giuseppe Santoro; Lamia Ait-Ali

doi:10.1016/j.jaccas.2022.03.027

Multimodality Approach to a Complex Scimitar Syndrome: How Advanced Diagnostics Can Guide Therapeutic Strategies

JACC Case Rep. 2022 May 18;4(10):596-603. doi: 10.1016/j.jaccas.2022.03.027.

Authors

Affiliations

¹ Department of Radiology, CNR (National Council of Research)/Tuscany Region "Gabriele Monasterio" Foundation (FTGM), Massa, Italy.
² Pediatric Cardiac Surgery, CNR (National Council of Research)/Tuscany Region "Gabriele Monasterio" Foundation (FTGM), Massa, Italy.
³ Pediatric Cardiology and GUCH Unit, CNR (National Council of Research)/Tuscany Region "Gabriele Monasterio" Foundation (FTGM), Massa, Italy.
⁴ Institute of Clinical Physiology, National Research Council, Pisa, Italy.

Abstract

We report an unusual association of scimitar syndrome with right diaphragmatic hernia, left-sided aortic arch with "aberrant right subclavian artery" in a 2-year-old boy who underwent stepwise transcatheter occlusion of a significant aortopulmonary collateral followed by surgical treatment for the repair of the diaphragmatic hernia and esophageal compression. (Level of Difficulty: Advanced.).

Keywords: APC, aortopulmonary collateral; ARSA, aberrant right subclavian artery; CCT, cardiac computed tomography; CDH, congenital diaphragmatic hernia; CMR, cardiac magnetic resonance; HPF, hepatopulmonary fusion; SS, scimitar syndrome; aberrant right subclavian artery; anomalous pulmonary venous return; congenital heart disease; diaphragmatic hernia; scimitar syndrome.

Publication types

Case Reports