Cardiovascular Journal of Africa: Vol 30 No 1 (January/February 2019)

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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 30, No 1, January/February 2019 8 AFRICA
Discussion
The study results are in keeping with published literature that
describes a RSH as an uncommon entity.1,3 RSH comprised only
1% of the total number of paediatric patients referred for cardiac
assessment at CHBAH over a 22-year period.
The majority of patients were diagnosed with dextrocardia.
There was no statistical significance between dextrocardia, the two
most common situs arrangements and their ACM. This differs
from other published data that have demonstrated an association
between dextrocardia, situs arrangements and ACM.10,13 As this
was a retrospective audit, pertinent patient information was not
obtainable in some cases. Therefore it is possible that some of the
study patients had undocumented ACM.
Eight of the study patients with situs inversus dextrocardia
without ACM were confirmed to have Kartagener syndrome.8,9
This highlights the importance of including a ciliopathy in the
differential diagnosis of patients with recurrent respiratory
infections and dextrocardia.
In this study, 7% of patients with situs inversus dextrocardia
and 22% of patients with situs ambiguous dextrocardia
underwent GIT surgery. Patients with situs inversus totalis can
present with abdominal signs and symptoms that differ from
patients with situs solitus. For example, appendicitis in patients
with situs inversus totalis and midgut rotation can present
with left lower-quadrant pain. Patients with situs ambiguous
are at risk of malrotation of the bowel and consequently
bowel ischaemia.11 This highlights the importance of timeous
recognition of different situs arrangements. Furthermore, as
seen in the study, RAI is associated with asplenia, placing these
patients at an increased risk for serious bacterial infections.12
The prevalence of situs ambiguous dextrocardia (16.7%)
was found to be similar to that previously reported.3,4,17 In the
study, both the RAI and LAI groups demonstrated some of
the more common serious cardiac and non-cardiac anomalies
(in particular GIT defects) requiring specialised paediatric
surgical services. These anomalies are in keeping with previously
published literature.12 The mortality rate was also in keeping with
published data, and was higher in the RAI group compared to
the LAI group.7,10,12
The risk for congenital heart lesions in patients with
dextroposition is reportedly the same as for the general
population.1,15,18 This was supported in the study results. Over a
quarter of the patients were diagnosed with Scimitar syndrome,
which is an unusual cause of dextroposition and is often
associated with cardiac lesions.19,20
The diagnosis of dextroversion is sometimes missed and
erroneously interchanged with dextroposition.
Conclusion
This study confirms the rarity of RSH, which are associated
with both cardiac and non-cardiac anomalies and requires a high
index of suspicion to make the diagnosis. Appropriate assessment
will allow for a streamlined approach and correct management.
References
1. Grant RP. The syndrome of dextroversion of the heart. Circulation
1958; 18(1): 25–36.
2. Bohun CM, Potts JE, Casey BM, Sandor GG. A population-based
study of cardiac malformations and outcomes associated with dextro-
cardia. Am J Cardiol 2007; 100(2): 305–309.
amjcard.2007.02.095.
3. Evans WN, Acherman RJ, Collazos JC, Castillo WJ, Rollins RC, Kip,
KT, et al. Dextrocardia: practical clinical points and comments on
terminology. Pediatr Cardiol 2010; 31(1):1–6.
s00246-009-9516-0.
4. Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia:
an analysis of cardiac structure in 125 patients. Int J Cardiol 2003;
88(2–3):143–155.
5. Khan MA, Almasham Y, Almoukirish A, Galal O, Momenah T.
Dextrocardia and associated cardiac malformations: Experience from
a tertiary centre in Saudi Arabia. 2013; Available at: http://livestreamsa.
= view&id= 308&page=24&sortby= title.
Accessed December 17, 2013.
6. Rare diseases: Genetic and Rare Diseases Information Centre.
Dextrocardia with situs inversus. (No date); Available at: https://
rarediseases.info.nih.gov/diseases/6268/dextrocardia-with-situs-inversus.
Accessed March 14, 2018.
7. Maldjian PD, Saric M. Approach to dextrocardia in adults: review. Am
J Roentgenol 2007; 188(6 Suppl): S39–S49; quiz S35–8.
org/10.2214/AJR.06.1179.
8. Bharati S, Lev M. Positional variations of the heart and its component
chambers. Circulation 1979; 59(5): 886–887.
9. Wilhelm A. Situs inversus imaging. 2011; Available at http://emedicine.
413679-overview. Accessed November 13, 2012.
10. Ghosh S, Yarmish G, Godelman A, Haramati LB, Spindola-Franco
H. Anomalies of visceroatrial situs. Am J Roentgenol 2009; 193(4):
1107–1117.
11. Akbulut S, Ulku A, Senol A, Tas M, Yagmur Y. Left-sided appendicitis:
Review of 95 published cases and a case report. World J Gastroenterol
2010; 16(44): 5598–5602.
12. Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: Imaging
of the heterotaxy syndrome. Radiographics 1999; 19(4): 837–852. http://
13. Kim SJ. Heterotaxy syndrome. Korean Circ J 2011; 41(5): 227–232.
14. Abdullah NL, Quek SC, Seto KY, Teo LLS. Clinics in diagnostic
imaging (160). Singapore Med J 2015; 56(4): 198–202. .
org/10.11622/smedj.2015059.
15. Fyler DC. Report of the New England regional infant cardiac program.
Pediatrics 1980; 65(suppl): 375–461.
16. Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG.
Research electronic data capture (REDCap) – A metadata-driven
methodology and workflow process for providing translational research
informatics support. J Biomed Inform 2009; 42(2): 377–381.
17. Roodpeyma SH, Abarashi M. Dextrocardia in children: Review of 15
cases. Iran J Pediatr 1999; 9(1): 41–47.
18. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote, L. Congenital
heart disease in the general population. Changing prevalence and
age distribution. Circulation 2007; 115(2): 163–172.
org/10.1161/CIRCULATION.AHA.106.627224.
19. Gao Y, Burrows PE, Benson LN, Rabinovitch M, Freedom RM.
Scimitar syndrome in infancy. J Am Coll Cardiol 1993; 22(3): 873–882.
20. Dusenbery SM, Geva T, Seale A, Valente AM, Zhou J, Sena L, et
al. Outcome predictors and implications for management of scim-
itar syndrome. Am Heart J 2013; 165(5): 770–777.
org/10.1016/j.ahj.2013.01.016.