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

Rihab  Machtache; Soukaina Safir; Sara Chibane; Pr Abdenasser Drighil; Pr Leila Azzouzi; Pr Rachida Habbal; Fadoua Kossal; Pr Salam

doi:10.5812/intjcardiovascpract-131488

International Journal of Cardiovascular Practice

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https://doi.org/10.5812/intjcardiovascpract-131488

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

Author(s):

Rihab Machtache ^1,*,

Soukaina Safir¹,

Sara Chibane¹,

Pr Abdenasser Drighil¹,

Pr Leila Azzouzi¹,

Pr Rachida Habbal¹,

Fadoua Kossal²,

Pr Salam²

1Department of Cardiology, Ibn Rochd University Teaching Hospital, Hassan II University, Casablanca, Morocco

2Department of Radiology, Ibn Rochd University Teaching Hospital, Hassan II University, Casablanca, Morocco

International Journal of Cardiovascular Practice:Vol. 5, issue 1; e131488

Published online:Jun 15, 2020

Article type:Case Report

Received:Dec 29, 2019

Accepted:Feb 27, 2020

How to Cite:Rihab Machtache Soukaina SafirSara ChibanePr Abdenasser DrighilPr Leila AzzouziPr Rachida HabbalFadoua KossalPr Salamet al.Scimitar Syndrome Issued from a Consanguineous Marriage: A Rare Congenital Cardiomyopathy.Int J Cardiovasc Pract.2020;5(1):e131488.https://doi.org/10.5812/intjcardiovascpract-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

Keywords

Scimitar Syndrome

Consanguinity

Cardiomyopathies

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Author(s):

Rihab Machtache :[PubMed][Scholar]
Soukaina Safir:[PubMed][Scholar]
Sara Chibane:[PubMed][Scholar]
Pr Abdenasser Drighil:[PubMed][Scholar]
Pr Leila Azzouzi:[PubMed][Scholar]
Pr Rachida Habbal:[PubMed][Scholar]
Fadoua Kossal:[PubMed][Scholar]
Pr Salam:[PubMed][Scholar]