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Situs Anomalies
Michael A. Gibbs, MD, Lead Editor
Carolinas Medical Center Imaging Mastery Project
Dominic Nicacio, MD, Andrew Yde, MD,
Jorge Alegria, MD Laszlo Littmann, MD
Departments of Emergency Medicine & Internal Medicine
Sanger Heart & Vascular Institute
Carolinas Medical Center
Visit Our Website
www.EMGuidewire.com
For A Complete Archive Of Chest X-Ray Presentations And Much More!
Airway
Bones
Cardiac
Diaphragm
Effusion
Foreign body
Gastric
Hilum
It’s All About The Anatomy!
Embedded References
Maldijan PD. Approach To Dextrocardia In Adults. American Journal Of Radiology. 2007; 188;S39-
S49.
Winter JE. Recognition of Anterior STEMI in Dextrocardia and the Importance of Right-Sided Chest
Leads. Journal of The American College of Cardiology. 2020; 2(8):1222-1226.
Gentile BA. Situs Inversus Totalis. New England Journal of Medicine. 2019; 380:e45.
Celik AI. Acute Anterior Myocardial Infarction in a Patient with Dextrocardia and Situs Inversus.
Journal of the American College of Cardiology. 2020; 2(8):1220-1221.
Situs Nomenclature
Determined by the relationship between the atria and adjacent organs:
• Situs solitus
• Situs solitus with dextrocadia
• Situs inversus with dextrocardia
• Situs inversus with levocardia
• Situs ambiguous
Situs Solitus
• Normal anatomy
Situs Solitus With Dextrocardia
• Mirror image positioning of the heart only
Situs Inversus With Dextrocardia [Totalis]
• Mirror image positioning of all thoracoabdominal viscera
Situs Inversus With Levocardia
• Mirror image positioning of all abdominal viscera
• Descending aorta on the right (concordant with situs inversus)
Situs Ambiguous
• Also called situs heterotaxy, any arrangement of organs between the spectrum
of situs solitus (normal positioning) and situs inversus totalis
dextrocardia, the descending aorta, cardiac apex, and stomach are all on the right. In situs solitus with dextrocardia, the descending aorta and stomach are on the left (normal), but the
cardiac apex is on the right. In situs inversus with levocardia, the descending aorta and stomach are on the right (situs inversus), but the cardiac apex is on the left.
Figure 3-8 Schematic illustrations of the anatomic relationships of the descending aorta, left atrium, cardiac apex, and stomach in the four basic cardiac positions (normal and three
malpositions). In situs solitus, the descending aorta, left atrium, cardiac apex, and stomach are all on the left. In situs inversus with dextrocardia, the descending aorta, left atrium, cardiac
apex, and stomach are all on the right. In situs solitus with dextrocardia, the descending aorta, left atrium, and stomach are on the left (normal), but the cardiac apex is on the right. In situs
inversus with levocardia, the descending aorta, left atrium, and stomach are on the right (situs inversus), but the cardiac apex is on the left. (RA = right atrium; LA = left atrium; RV = right
Situs Solitus With
Dextrocardia
3-11 X-ray from a 2-year-old girl in situs inversus with levocardia. The stomach (S) is on the right and the liver (L) is on the left, but the heart (apex) is to the left of mid
midiaphragm is lower than the right hemidiaphragm because the cardiac apex is on the left. The descending thoracic aorta (DAo) is on the right (concordant for situs inversus
of the ascending aorta (AAo) indicates a discordant d-bulboventricular loop.
Situs Inversus And Levocardia: The stomach (S) is on the right and the liver (L) on the left. The left
hemidiaphragm is lower because the cardiac apex is no the left. The descending thoracic aorta (DAo) is on
the right (concordant with situs inversus, but the ascending aortic (AAo) is on the left.
Primary Ciliary Dyskinesia
• Primary ciliary dyskinesia is an inherited disorder of abnormally
functioning cilia
• Abnormally functioning cilia of the epithelial cells necessary for
correct thoracoabdominal orientation during embryogenesis
• Approximately 25% of patients with situs inversus totalis have
primary ciliary dyskinesia
• Kartagener’s Syndrome defines a subset of primary ciliary dyskinesia
patients with with classic triad: situs inversus totalis, sinusitis and
bronchiectasis
Kartagener’s Syndrome
Epidemiology
Dextrocardia Situs Inversus Totalis
Incidence 1 in 12,000 1 in 10,000
Genetic Inheritance Inconclusive Autosomal recessive in patients
with Primary Ciliary Dyskinesia
Risk Factors  Males and females are affected equally
 Maternal cocaine use is associated with an increased
incidence
Associated Conditions  Transposition of great vessels, double inlet ventricles,
tricuspid atresia, polysplenia
Clinical Presentation
Dextrocardia1,2,3:
 Asymptomatic
 Characteristic ECG findings
Situs Inversus1,2,3:
 Opposite sided abdominal complaints in acute hepatobiliary,
gastric, and appendiceal pathology
Kartagener’s syndrome1,2:
 Chronic sinusitis and otitis media
 Bronchiectasis
 Infertility common in males
1Right-sided heart tones.
2Sequelea of any other associated congenital heart disease.
3Characteristic ECG findings.
Situs Anomalies
Cases Studies From Carolinas Medical Center
Normal Position Situs Solitus
Liver
Diaphragm Higher
On The Right
Gastric Bubble
Cardiac Apex
Descending Aorta
On The Left
Spleen
61-Year-Old
At Cardiology
Appointment.
Diaphragm Higher
On The Left
Situs Inversus With Dextrocardia
Liver
Gastric Bubble
LiverSpleen
Abdominal Transposition
Dextrocardia
Situs Inversus Totalis
Healthy 33-
Year-Old
With Chest
Pain.
Situs Inversus With Dextrocardia
Gastric Bubbler
Diaphragm Higher
On The Left
Liver
Healthy 42-
Year-Old Has
An Incidental
Finding On
Chest X-Ray
Situs Inversus With Dextrocardia
Gastric Bubbler
Liver
65-Year-Old
At His
Cardiology
Follow-Up
Visit.
Diaphragm Higher
On The Left
Situs Inversus With Dextrocardia
Gastric Bubbler
Liver
Healthy 45-
Year-Old
Undergoes
Preoperative
Screening.
Situs Solitus With Dextrocardia
Gastric Bubble
Liver
33-Year-Old
With
Congenitally
Corrected
Transposition
Of The Great
Vessels.
Situs Solitus With Dextrocardia
Gastric Bubble
Liver
Liver
Situs Solitus And Dextrocardia
Classic ECG Findings
• Inverted complexes in I, aVL
• Upright complexes in aVR
• Low voltage V3-V6
• No R wave progression
• Right axis deviation
• Normal EKG with lead reversal
If You Have Interesting Cases Of Situs Anomalies We Invite You To Send A Set
Of Digital PDF Images And A Brief Clinical History To:
michael.gibbs@atriumhealth.org
Your De-Identified Case(s) Will Be Posted On Our Education Website And You
And Your Institution Will Be Recognized!

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EMGuideWire's Radiology Reading Room: Situs Anomalies

  • 1. Situs Anomalies Michael A. Gibbs, MD, Lead Editor Carolinas Medical Center Imaging Mastery Project Dominic Nicacio, MD, Andrew Yde, MD, Jorge Alegria, MD Laszlo Littmann, MD Departments of Emergency Medicine & Internal Medicine Sanger Heart & Vascular Institute Carolinas Medical Center
  • 2. Visit Our Website www.EMGuidewire.com For A Complete Archive Of Chest X-Ray Presentations And Much More!
  • 4. It’s All About The Anatomy!
  • 5. Embedded References Maldijan PD. Approach To Dextrocardia In Adults. American Journal Of Radiology. 2007; 188;S39- S49. Winter JE. Recognition of Anterior STEMI in Dextrocardia and the Importance of Right-Sided Chest Leads. Journal of The American College of Cardiology. 2020; 2(8):1222-1226. Gentile BA. Situs Inversus Totalis. New England Journal of Medicine. 2019; 380:e45. Celik AI. Acute Anterior Myocardial Infarction in a Patient with Dextrocardia and Situs Inversus. Journal of the American College of Cardiology. 2020; 2(8):1220-1221.
  • 6. Situs Nomenclature Determined by the relationship between the atria and adjacent organs: • Situs solitus • Situs solitus with dextrocadia • Situs inversus with dextrocardia • Situs inversus with levocardia • Situs ambiguous
  • 7. Situs Solitus • Normal anatomy Situs Solitus With Dextrocardia • Mirror image positioning of the heart only Situs Inversus With Dextrocardia [Totalis] • Mirror image positioning of all thoracoabdominal viscera Situs Inversus With Levocardia • Mirror image positioning of all abdominal viscera • Descending aorta on the right (concordant with situs inversus) Situs Ambiguous • Also called situs heterotaxy, any arrangement of organs between the spectrum of situs solitus (normal positioning) and situs inversus totalis
  • 8. dextrocardia, the descending aorta, cardiac apex, and stomach are all on the right. In situs solitus with dextrocardia, the descending aorta and stomach are on the left (normal), but the cardiac apex is on the right. In situs inversus with levocardia, the descending aorta and stomach are on the right (situs inversus), but the cardiac apex is on the left. Figure 3-8 Schematic illustrations of the anatomic relationships of the descending aorta, left atrium, cardiac apex, and stomach in the four basic cardiac positions (normal and three malpositions). In situs solitus, the descending aorta, left atrium, cardiac apex, and stomach are all on the left. In situs inversus with dextrocardia, the descending aorta, left atrium, cardiac apex, and stomach are all on the right. In situs solitus with dextrocardia, the descending aorta, left atrium, and stomach are on the left (normal), but the cardiac apex is on the right. In situs inversus with levocardia, the descending aorta, left atrium, and stomach are on the right (situs inversus), but the cardiac apex is on the left. (RA = right atrium; LA = left atrium; RV = right
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  • 14. 3-11 X-ray from a 2-year-old girl in situs inversus with levocardia. The stomach (S) is on the right and the liver (L) is on the left, but the heart (apex) is to the left of mid midiaphragm is lower than the right hemidiaphragm because the cardiac apex is on the left. The descending thoracic aorta (DAo) is on the right (concordant for situs inversus of the ascending aorta (AAo) indicates a discordant d-bulboventricular loop. Situs Inversus And Levocardia: The stomach (S) is on the right and the liver (L) on the left. The left hemidiaphragm is lower because the cardiac apex is no the left. The descending thoracic aorta (DAo) is on the right (concordant with situs inversus, but the ascending aortic (AAo) is on the left.
  • 15. Primary Ciliary Dyskinesia • Primary ciliary dyskinesia is an inherited disorder of abnormally functioning cilia • Abnormally functioning cilia of the epithelial cells necessary for correct thoracoabdominal orientation during embryogenesis • Approximately 25% of patients with situs inversus totalis have primary ciliary dyskinesia • Kartagener’s Syndrome defines a subset of primary ciliary dyskinesia patients with with classic triad: situs inversus totalis, sinusitis and bronchiectasis
  • 17. Epidemiology Dextrocardia Situs Inversus Totalis Incidence 1 in 12,000 1 in 10,000 Genetic Inheritance Inconclusive Autosomal recessive in patients with Primary Ciliary Dyskinesia Risk Factors  Males and females are affected equally  Maternal cocaine use is associated with an increased incidence Associated Conditions  Transposition of great vessels, double inlet ventricles, tricuspid atresia, polysplenia
  • 18.
  • 19. Clinical Presentation Dextrocardia1,2,3:  Asymptomatic  Characteristic ECG findings Situs Inversus1,2,3:  Opposite sided abdominal complaints in acute hepatobiliary, gastric, and appendiceal pathology Kartagener’s syndrome1,2:  Chronic sinusitis and otitis media  Bronchiectasis  Infertility common in males 1Right-sided heart tones. 2Sequelea of any other associated congenital heart disease. 3Characteristic ECG findings.
  • 20. Situs Anomalies Cases Studies From Carolinas Medical Center
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  • 22. Normal Position Situs Solitus Liver Diaphragm Higher On The Right Gastric Bubble Cardiac Apex Descending Aorta On The Left Spleen
  • 24. Diaphragm Higher On The Left Situs Inversus With Dextrocardia Liver Gastric Bubble
  • 27. Situs Inversus With Dextrocardia Gastric Bubbler Diaphragm Higher On The Left Liver
  • 28. Healthy 42- Year-Old Has An Incidental Finding On Chest X-Ray
  • 29. Situs Inversus With Dextrocardia Gastric Bubbler Liver
  • 31. Diaphragm Higher On The Left Situs Inversus With Dextrocardia Gastric Bubbler Liver
  • 33. Situs Solitus With Dextrocardia Gastric Bubble Liver
  • 35. Situs Solitus With Dextrocardia Gastric Bubble Liver
  • 36. Liver Situs Solitus And Dextrocardia
  • 37. Classic ECG Findings • Inverted complexes in I, aVL • Upright complexes in aVR • Low voltage V3-V6 • No R wave progression • Right axis deviation • Normal EKG with lead reversal
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  • 46. If You Have Interesting Cases Of Situs Anomalies We Invite You To Send A Set Of Digital PDF Images And A Brief Clinical History To: michael.gibbs@atriumhealth.org Your De-Identified Case(s) Will Be Posted On Our Education Website And You And Your Institution Will Be Recognized!