Download PDF
CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2019; 29(03): 318-323
DOI: 10.4103/ijri.IJRI_341_19
Case Report

Left atrial isomerism associated with aneurysmal enlargement of right atrial appendage: A case report with literature review

Prateek Agarwal
Department of Radiodiagnosis, Kempegowda Institute of Medical Sciences, Bengaluru, Karnataka
,
Rajesh Kumar Agarwal
Department of Ultrasound, Meera Hospital, Shiv Marg, Bani Park, Jaipur, Rajasthan, India
› Author Affiliations Financial support and sponsorship Nil.
› Further Information
   Permissions and Reprints

Abstract

We present a prenatally diagnosed case of heterotaxy syndrome (HS) in which left atrial isomerism (LAI) was associated with an aneurysmal enlargement of the right atrial appendage (RAA). Although LAI is usually associated with complex cardiac and extracardiac anomalies, the association of LAI and right atrial appendage aneurysm (RAAA) is exceptional. Congenital RAAA itself is an idiopathic, very rare cardiac anomaly characterized by the enlargement of the appendage in the absence of any other cardiac or extra-cardiac defect. The prognosis of the heterotaxy is poor with associated major cardiac malformations and even cases with minor cardiac anomalies are at risk postnatally for complications like biliary atresia, intestinal rotational abnormalities, and immune disorders. In this case, the prenatal diagnosis of the isomerism was mainly based on the abnormalities of caval veins. Although no typical complex cardiac anomaly was present, the HS was associated with biliary atresia, polysplenia, and malrotation of the gut. Associated RAAA further imposed an additional risk of complications such as tachyarrhythmias, thromboembolic events, and aneurysmal rupture.

Keywords

Aneurysm - atrial appendage - heterotaxy - isomerism - polysplenia


Publication History

Received: 13 August 2019

Accepted: 10 September 2019

Article published online:
22 July 2021

© 2019. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Thieme Medical and Scientific Publishers Private Ltd.
A-12, Second Floor, Sector -2, NOIDA -201301, India

 

  • References

  • 1 Berg C, Geipel A, Smrcek J, Krapp M, Germer U, Kohl T. et al. Prenatal diagnosis of cardiosplenic syndromes: A 10-year experience. Ultrasound Obstet Gynecol 2003; 22: 451-9
    CrossrefPubMedGoogle Scholar
  • 2 Fröber R, Eichhorn KH, Schleußner E, Schlembach D. Heterotaxy Syndrome: An Embryologic Study. Ann Clin Lab Res 2016; 4: 1
    CrossrefGoogle Scholar
  • 3 Jacobs JP, Anderson RH, Weinberg PM, Walters III HL, Tchervenkov CI, Del Duca D. et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 2007; 2: 1-28
    CrossrefPubMedGoogle Scholar
  • 4 Berg C, Geipel A, Kohl T, Smrcek J, Germer U, Baschat AA. et al. Fetal echocardiographic evaluation of atrial morphology and the prediction of laterality in cases of heterotaxy syndromes. Ultrasound Obstet Gynecol 2005; 26: 538-45
    CrossrefPubMedGoogle Scholar
  • 5 Buca DIP, Khalil A, Rizzo G, Familiari A, Di Giovanni S, Liberati M. et al. Outcome of prenatally diagnosed fetal heterotaxy: Systematic review and meta-analysis. Ultrasound Obstet Gynecol 2018; 5: 323-30
    CrossrefPubMedGoogle Scholar
  • 6 Aryal MR, Hakim FA, Giri S, Ghimire S, Pandit A, Bhandari Y. et al. Right atrial appendage aneurysm: A systematic review. Echocardiography 2014; 31: 534-9
    CrossrefPubMedGoogle Scholar
  • 7 Perloff JK. The cardiac malpositions. Am J Cardiol 2011; 108: 1352-61
    CrossrefPubMedGoogle Scholar
  • 8 Yim D, Nagata H, Lam CZ, Grosse-Wortmann L, Seed M, Jaeggi E. et al. Disharmonious patterns of heterotaxy and isomerism. How Often Are the Classic Patterns Breached ? Circ Cardiovasc Imaging 2018; 11: e006917
    CrossrefPubMedGoogle Scholar
  • 9 Anderson RH. Isomerism of the atrial appendages. Am J Cardiol 1999; 83: 818-9
    CrossrefPubMedGoogle Scholar
  • 10 Pepes S, Zidere V, Allan LD. Prenatal diagnosis of left atrial isomerism. Heart 2009; 95: 1974-7
    CrossrefPubMedGoogle Scholar
  • 11 Colloridi V, Pizzuto F, Ventriglia F, Giancotti A, Pachi A, Gallo P. Prenatal echocardiographic diagnosis of right atrial isomerism. Prenat Diagn 1994; 14: 299-302
    CrossrefPubMedGoogle Scholar
  • 12 Tawfik AM, Batouty NM, Zaky MM, Eladalany MA, Elmokadem AH. Polysplenia syndrome: A review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat 2013; 35: 647-53
    CrossrefPubMedGoogle Scholar
  • 13 Saito T, Watanabe M, Kojima T, Matsumura T, Fujita H, Kiyosue A. et al. Successful blood sampling through azygos continuation with interrupted inferior vena cava. A case report and review of the literature. Int Heart J 2011; 52: 327-30
    CrossrefPubMedGoogle Scholar
  • 14 Costa S, Carriço A, Valente F. Prenatal diagnosis of left isomerism with normal heart. BMJ Case Reports 2017; DOI: 10.1136/bcr-2017-2230971.
    CrossrefGoogle Scholar
  • 15 Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T. Polysplenia syndrome detected in adulthood: Report of eight cases and review of the literature. Abdom Imaging 1999; 24: 178-84
    CrossrefPubMedGoogle Scholar
  • 16 Low JP, William D, Chaganti JR. Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice-A case report and literature review. Br J Radiol 2011; 84: e217-20
    PubMedGoogle Scholar
  • 17 Ho SY, Fagg N, Anderson RH, Cook A, Allan L. Disposition of the atrioventricular conduction tissues in the heart with isomerism of the atrial appendages: Its relation to congenital complete heart block. J Am Coll Cardiol 1992; 20: 904-10
    CrossrefPubMedGoogle Scholar
  • 18 Ozawa Y, Asakai H, Shiraga K, Shindo T, Hirata Y, Hirata Y. et al. Cardiac rhythm disturbances in heterotaxy syndrome. Pediatr Cardiol 2019; 40: 909-13
    CrossrefPubMedGoogle Scholar
  • 19 Ware AL, Miller DV, Porter CB, Edwards WD. Characterization of atrial morphology and sinus node morphology in heterotaxy syndrome: An autopsy-based study of 41 cases (1950-2008). Cardiovasc Pathol 2012; 21: 421-7
    CrossrefPubMedGoogle Scholar
  • 20 Baschat AA, Gembruch U, Knopfle G, Hansmann M. First-trimester fetal heart block: A marker for cardiac anomaly. Ultrasound Obstet Gynecol 1999; 14: 311-4
    CrossrefPubMedGoogle Scholar
  • 21 Escobar-Diaz MC, Tworetzky W, Friedman K, Lafranchi T, Fynn-Thompson F, Alexander ME. et al. Perinatal outcome in fetuses with heterotaxy syndrome and atrioventricular block or bradycardia. Pediatr Cardiol 2014; 35: 906-13
    CrossrefPubMedGoogle Scholar
  • 22 Giang DTC, Rajeesh G, Vaidyanathan B. Prenatal diagnosis of isolated interrupted inferior vena cava with azygos continuation to superior vena cava. Ann Pediatr Cardiol 2014; 7: 49-51
    CrossrefPubMedGoogle Scholar
  • 23 Bronshtein M, Khatib N, Blumenfeld Z. Prenatal diagnosis and outcome of isolated interrupted inferior vena cava. Am J Obstet Gynecol 2010; 202: 398e1-4
    CrossrefPubMedGoogle Scholar
  • 24 Patel V, Nanda NC, Rajdev S, Mehmood F, Velayudhan D, Vengala S. Live/real time three-dimensional transthoracic echocardiographic assessment of Ebstein’s anomaly. Echocardiography 2005; 22: 847-54
    CrossrefPubMedGoogle Scholar
  • 25 Toufan M, Pourafkari L, Akbarzadeh F, Nader N. A case of right atrial appendage aneurysm mimicking a pericardial cyst on echocardiogram. Echo Res Pract 2014; 1: K5-8
    CrossrefPubMedGoogle Scholar
  • 26 Gottschalk I, Stressig R, Ritgen J, Herberg U, Breuer J, Vorndamme A. et al. Extracardiac anomalies in prenatally diagnosed heterotaxy syndrome. Ultrasound Obstet Gynecol 2016; 47: 443-9
    CrossrefPubMedGoogle Scholar
  • 27 Chalouhi GE, Muller F, Dreux S, Ville Y, Chardot C. Prenatal non-visualization of fetal gallbladder: Beware of biliary atresia. Ultrasound Obstet Gynecol 2011; 38: 237-8
    CrossrefPubMedGoogle Scholar
  • 28 Shen O, Sela HY, Nagar H, Rabinowitz R, Jacobovich E, Chen D. et al. Prenatal diagnosis of biliary atresia: A case series. Early Hum Dev 2017; 111: 16-9
    CrossrefPubMedGoogle Scholar
  • 29 Shen O, Rabinowitz R, Yagel S, Gal M. Comment on “Relationship of nonvisualization of the fetal gallbladder and amniotic fluid digestive enzymes analysis to outcome”. Prenat Diagn 2012; 32: 1119-20
    CrossrefPubMedGoogle Scholar
  • 30 Ruiz A, Robels A, Salva F, Filgueira A, Diaz C, Juan M. et al. Prenatal nonvisualization of the gallbladder: A diagnostic and prognostic dilemma. Fetal Diagn Ther 2017; 42: 150-2
    CrossrefPubMedGoogle Scholar
  • 31 Burc L, Vulillard E, Guibourdenche J, Cont M, Garel L, Proquet D. et al. Prenatal diagnosis and follow up of biliary atresia. BJOG 2001; 108: 1108-10
    PubMedGoogle Scholar
  • 32 McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000; 355: 25-9
    CrossrefPubMedGoogle Scholar
  • 33 Hildebrand H, Gunzenhauser D, Weber K, Frese W, Fröber R, Wetter D. Heterotaxia syndrome without congenital cardiac defects in dilated cardiomyopathy. Dtsch Med Wochenschr 2007; 132: 931-7
    Article in Thieme ConnectPubMedGoogle Scholar
  • 34 Prendiville TW, Barton LL, Thompson WR, Fink DL, Holmes KW. Heterotaxy syndrome: Defining contemporary disease trends. Pediatr Cardiol 2010; 3: 1052-8
    CrossrefPubMedGoogle Scholar
  • 35 Kheiri M, Lesieur E, Dabadie A, Colombani M, Capelle M, Sigaudy S. et al. Prenatal diagnosis of bowel malposition using T2-weighted fetal MRI sequences. Diagn Interv Imaging 2016; 97: 857-61
    CrossrefPubMedGoogle Scholar
  • 36 Nemec SF, Brugger PC, Nemec U, Bettelheim D, Kasprian G, Amann G. et al. Situs anomalies on pre-natal MRI. Eur J Radiol 2012; 81: e495-501
    CrossrefPubMedGoogle Scholar
  • 37 Loomba R, Shah PH, Anderson RH. Fetal magnetic resonance imaging of malformations associated with heterotaxy. Cureus 2015; 7: e269
    PubMedGoogle Scholar
  • 38 Hofmann R, Heilmann A, Häusler HJ, Dähnert I, Kamin G, Lachmann G. Congenital idiopathic dilatation of the right atrium: Antenatal appearance, postnatal management, long-term follow-up and possible pathomechanism. Fetal Diagn Ther 2012; 32: 256-61
    CrossrefPubMedGoogle Scholar