Literature DB >> 27294094

Not all right-sided hearts are the same-the importance of identifying the correct diagnosis.

Faisal Mujib Siddiqui1, Edmundo Raul Rubio1, Vishal M Patel1, Sameh Aziz1, Susanti Ie1.   

Abstract

Scimitar syndrome is characterized by an anomalous venous return with the characteristic chest roentgenogram (CxR) appearance of the anomalous vein draining into the inferior vena cava (IVC). This appears as a curvilinear opacity paralleling the right border of the heart resembling a curved sword or Scimitar. A 27-year-old white woman with a reported history of dextrocardia was admitted after a drug overdose. Examination demonstrated an obtunded woman with tachycardia and right sided heart sounds. Her CxR revealed a right sided heart image with two curvilinear opacities in the retrocardiac area. Chest computed tomography (CT) demonstrated that these opacities join to represent an anomalous vein draining into IVC. Furthermore, an anomalous systemic artery arising from the abdominal aorta was seen to supply the right lower lobe. The patient was eventually diagnosed with Scimitar syndrome. This syndrome affects 1-3 in 100,000 live births while nearly half of the patients remain asymptomatic with some initially being misdiagnosed as dextrocardia, such as in our case. Correctly diagnosing these patients is of paramount importance as some can develop severe pulmonary hypertension and right ventricular failure. In turn, close ongoing echocardiographic monitoring can help identify those that may benefit from surgical interventions to prevent them from developing these complications.

Entities:  

Keywords:  Partial anomalous venous return; Scimitar syndrome; congenital pulmonary venolobar syndrome (CPVS); dextrocardia

Year:  2016        PMID: 27294094      PMCID: PMC4885893          DOI: 10.21037/atm.2016.05.29

Source DB:  PubMed          Journal:  Ann Transl Med        ISSN: 2305-5839


  19 in total

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4.  Three-dimensional computed tomography showing partial anomalous pulmonary venous connection complicated by the scimitar syndrome.

Authors:  Teruo Inoue; Michiko Ichihara; Toshihiko Uchida; Yoshihiko Sakai; Terumi Hayashi; Shigenori Morooka
Journal:  Circulation       Date:  2002-02-05       Impact factor: 29.690

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8.  Correlation of plasma B-type natriuretic peptide with shunt volume in children with congenital heart disease involving left-to-right shunt.

Authors:  Maria Kavga; George Varlamis; Andreas Giannopoulos; Kyriaki Papadopoulou-Legbelou; Sotirios Varlamis; Georgios Bompotis; Vassiliki Koulourida; Nikolaos Nikolaides
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9.  A systematic classification of the congenital bronchopulmonary vascular malformations: dysmorphogeneses of the primitive foregut system and the primitive aortic arch system.

Authors:  Meng-Luen Lee; Hung-Chi Lue; Ing-Sh Chiu; Han-Yao Chiu; Lon-Yen Tsao; Ching-Yuan Cheng; Albert D Yang
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10.  Congenital bronchopulmonary vascular malformations, "sequestration" and beyond.

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  1 in total

1.  Variants in myelin regulatory factor (MYRF) cause autosomal dominant and syndromic nanophthalmos in humans and retinal degeneration in mice.

Authors:  Sarah J Garnai; Michelle L Brinkmeier; Ben Emery; Tomas S Aleman; Louise C Pyle; Biliana Veleva-Rotse; Robert A Sisk; Frank W Rozsa; Ayse Bilge Ozel; Jun Z Li; Sayoko E Moroi; Steven M Archer; Cheng-Mao Lin; Sarah Sheskey; Laurel Wiinikka-Buesser; James Eadie; Jill E Urquhart; Graeme C M Black; Mohammad I Othman; Michael Boehnke; Scot A Sullivan; Gregory L Skuta; Hemant S Pawar; Alexander E Katz; Laryssa A Huryn; Robert B Hufnagel; Sally A Camper; Julia E Richards; Lev Prasov
Journal:  PLoS Genet       Date:  2019-05-02       Impact factor: 5.917

  1 in total

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