Scimitar Syndrome Issued from a Consanguineous Marriage: A Rare Congenital Cardiomyopathy

authors:

avatar Rihab Machtache 1 , * , avatar Soukaina Safir 1 , avatar Sara Chibane 1 , avatar Pr Abdenasser Drighil 1 , avatar Pr Leila Azzouzi 1 , avatar Pr Rachida Habbal 1 , avatar Fadoua Kossal 2 , avatar Pr Salam 2

Department of Cardiology, Ibn Rochd University Teaching Hospital, Hassan II University, Casablanca, Morocco
Department of Radiology, Ibn Rochd University Teaching Hospital, Hassan II University, Casablanca, Morocco

how to cite: Machtache R , Safir S, Chibane S, Drighil P A, Azzouzi P L, et al. Scimitar Syndrome Issued from a Consanguineous Marriage: A Rare Congenital Cardiomyopathy. Int J Cardio Pract. 2020;5(1):e131488. doi: 10.5812/ijcp-131488.

Abstract

We present here a rare case of Scimitar syndrome on a full-term newborn female issued from consanguineous marriage and diagnosed at the age of two months who was suffering from polypnea since the age of two weeks. She was admitted at the age of two months for polypnea, non-dysmorphic facies, healthy systemic arterial tension, regular pulse, but the family history raises that before her birth, two newborns respectively boy and girl died both at an early age with dilated cardiomyopathy. After hospitalization, we realized chest radiography, which has shown opaque right lung and suspicion of scimitar syndrome. After that, an echocardiogram was performed, showing severe hypoplasia of the right pulmonary artery with pulmonary hypertension. The Thoracic computed tomography confirmed the diagnosis by revealing hypoplasia of the right pulmonary artery with pulmonary arterial hypertension, right pulmonary hypoplasia (bilobar), and abnormal partial right pulmonary venous return toward the vena cava under the diaphragm

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© 2020, Cardiovascular Research Center of Shahid Beheshti University of Medicl Sciences. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.