Here’s Dr. Carmelli with today’s Morning Report!

 

Dextrocardia and Situs Inversus

Definitions

  • Situs solitus: Normal position of organs/structures
  • Situs inversus: Opposite or reverse of normal position of organs/structures
  • Levocardia: Cardiac apex pointing to the left
  • Dextrocardia: Cardiac apex pointing toward the right
  • Cardiac displacement: A secondary shift of heart 2/2 eventration of a hemidiaphragm, agenesis of a lung, congenital absence of the pericardium…

 

Background

  • The incidence of Situs Inversus with dextrocardia is about 1/8000-1/25,000.
  • Unclear cause??? Sometimes its an autosomal recessive genetic condition, while other times it can be X-linked

 

Types

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Associated Illness

  • Situs inversus with dextrocardia: Often with structurally normal heart. However, 3-10% have a congenital heart disease:
    • Double outlet right ventricle, Endocardial cushion defect, Pulmonary stenosis, Single ventricle, Transposition of the great vessels (most common), Ventricular septal defect
  • Situs inversus w/ levocardia is rare, but 95% have a congenital heart disease

 

Kartageners

  • In 1933, Kartagener noticed a triad of sinusitis, bronchiectasis, and situs inversus, called Kartagener’s syndrome
  • This is a subgroup of primary ciliary dyskinesia, Prevalence of around one in 20,000 to 40,000 individuals, affecting 20% of patients with situs inversus
  • Of note, only 50% of pts with primary ciliary dyskinesia have situs inversus

 

Symptoms

  • Situs inversus with dextrocardia and a structurally normal heart is otherwise asymptomatic and has normal life longevity
  • However, note typical illness pain will be reversed:
    • Ischemic heart pain on the right, appendicitis pain on the LLQ, biliary colic pain on LUQ…etc

 

Diagnosis

  • Percussion and palpation – gastric tympany/cardiac dullness- right, hepatic dullness- left, and heart sounds are louder on the right. Apical pulse found on right LSB
  • Auscultation-1st/ 2nd heart sounds are louder on the right with S2 Splitting on the right. Cardiac murmur sites are the mirror images of normal.

 

CXR/ CT scan

  • Most Dextrocardia patients diagnosed after random CXR.
  • Look at apex, look for liver, stomach bubble, aortic arch, diaphragm height, IVC vs descending aorta…etc

 

EKG

  • Right axis deviation
  • Positive QRS complexes (with upright P and T waves) in aVR and maybe V1
  • Lead I: inversion of all complexes, (inverted P wave, QRS, T wave)- Limb lead reversal***
  • Absent R-wave progression in the chest leads (dominant S waves throughout)

 

Echo

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Treatment

  • Without Heart defects, no treatment required.
  • If heart defects are present, usually may need surgery during infancy.
  • Children with Kartagener syndrome will need repeated Tx with antibiotics for sinusitis

 

 

References

  • Situs Inversus. Wikipedia. https://en.wikipedia.org/wiki/Situs_inversus
  • Wilhelm A. Karani J. Situs Inversus Imaging. http://emedicine.medscape.com/article/413679-overview#showall
  • Kaneshiro N, Zieve D. Dextrocardia. MedlinePlus. http://www.nlm.nih.gov/medlineplus/ency/article/007326.htm
  • Webb G. Smallhorn J. Therrien J, Redington A. Congenital Heart Disease. Braunwald’s Heart Disease: A Textbook of Cardiovascular Medicine, 62, 1391-1445
  • Perloff J, Marelli A. Cardiac Malpositions. Clinical Recognition of Congenital Heart Disease, Chapter 3, 15-40
  • UpToDate.com: Primary ciliary dyskinesia (immotile-cilia syndrome)
  • UpToDate.com: ECG tutorial: Miscellaneous diagnoses
  • Lifeinthefastlane.com: Dextrocardia, ECG basics.
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Jay Khadpe MD

  • Editor in Chief of "The Original Kings of County"
  • Assistant Professor of Emergency Medicine
  • Assistant Residency Director
  • SUNY Downstate / Kings County Hospital

Latest posts by Jay Khadpe MD (see all)

Categories: Cardiovascular

Jay Khadpe MD

  • Editor in Chief of “The Original Kings of County”
  • Assistant Professor of Emergency Medicine
  • Assistant Residency Director
  • SUNY Downstate / Kings County Hospital

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