Literature DB >> 35607583

Incidental Finding of Heterotaxy Syndrome in a Patient With Pulmonary Embolism: A Case Report and Concise Review.

Mohamed Mahmoud1, Khadija El Kortbi2, Hayoung Wang3, Joseph Wang4.   

Abstract

Heterotaxy syndrome, also called atrial isomerism, is a rare congenital condition in which the internal organs are abnormally arranged across the left-right axis of the body. It is classified into polysplenia syndrome or left atrial isomerism and asplenia syndrome or right atrial isomerism. It is associated with high morbidity and mortality due to the severity of cardiac anomalies. It is important to be aware of the syndrome findings as they can be incidentally found on imaging in adults. Here, we report a case of a 33-year-old female who presented with worsening shortness of breath, found to have a pulmonary embolism, and heterotaxy was incidentally identified on her imaging. A concise review follows.
Copyright © 2022, Mahmoud et al.

Entities:  

Keywords:  asplenia with cardiac abnormalities; cardiac malformations; congenital hear disease; heterotaxia; heterotaxy syndrome (hs); left atrial isomerism; polysplenia; pulmonary emboli; right atrial isomerism (rai); situs ambiguus

Year:  2022        PMID: 35607583      PMCID: PMC9122844          DOI: 10.7759/cureus.24326

Source DB:  PubMed          Journal:  Cureus        ISSN: 2168-8184


  14 in total

1.  Outcome after orthotopic cardiac transplantation in adults with congenital heart disease.

Authors:  J M Lamour; L J Addonizio; M E Galantowicz; J M Quaegebeur; D M Mancini; M R Kichuk; A Beniaminovitz; R E Michler; A Weinberg; D T Hsu
Journal:  Circulation       Date:  1999-11-09       Impact factor: 29.690

2.  Thrombocytosis in asplenia syndrome with congenital heart disease: a previously unrecognized risk factor for thromboembolism.

Authors:  Kenichiro Yamamura; Kunitaka Joo; Shouichi Ohga; Hazumu Nagata; Kazuyuki Ikeda; Jun Muneuchi; Mamie Watanabe; Toshiro Hara
Journal:  Int J Cardiol       Date:  2012-06-22       Impact factor: 4.164

Review 3.  Impact of Era, Type of Isomerism, and Ventricular Morphology on Survival in Heterotaxy: Implications for Therapeutic Management.

Authors:  Rohit S Loomba; Karan Nijhawan; Robert Anderson
Journal:  World J Pediatr Congenit Heart Surg       Date:  2016-01

4.  Disharmonious Patterns of Heterotaxy and Isomerism: How Often Are the Classic Patterns Breached?

Authors:  Deane Yim; Hazumu Nagata; Christopher Z Lam; Lars Grosse-Wortmann; Mike Seed; Edgar Jaeggi; Shi-Joon Yoo
Journal:  Circ Cardiovasc Imaging       Date:  2018-02       Impact factor: 7.792

Review 5.  Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature.

Authors:  G Gayer; S Apter; T Jonas; M Amitai; R Zissin; T Sella; P Weiss; M Hertz
Journal:  Abdom Imaging       Date:  1999 Mar-Apr

6.  Biventricular repair in patients with heterotaxy syndrome.

Authors:  H G Lim; E A Bacha; G R Marx; A Marshall; F Fynn-Thompson; J E Mayer; P Del Nido; F A Pigula
Journal:  J Thorac Cardiovasc Surg       Date:  2009-02       Impact factor: 5.209

7.  Risk factors for heart disease associated with abnormal sidedness.

Authors:  Karen S Kuehl; Christopher Loffredo
Journal:  Teratology       Date:  2002-11

Review 8.  The heterotaxy syndrome: associated congenital heart defects and management.

Authors:  Ravi Agarwal; Roy Varghese; Vimala Jesudian; Jeswin Moses
Journal:  Indian J Thorac Cardiovasc Surg       Date:  2020-03-27

Review 9.  Non-cardiac issues in patients with heterotaxy syndrome.

Authors:  Shyam S Kothari
Journal:  Ann Pediatr Cardiol       Date:  2014-09

10.  Heterotaxy Polysplenia Syndrome in Adulthood: Focused Review and a Case Report.

Authors:  Gustavo Lagrotta; Melanie Moises
Journal:  Cureus       Date:  2020-01-30
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