Congenital condition caused by malrotation of viscera
in utero. Can be classified further into situs inversus with dextrocardia or
situs inversus with levocardia. The classification is independent of the
cardiac apical position. The association of situs inversus and ciliary
dyskinesia is known as the Kartagener syndrome.
Present in 0.01% of the population. Patients present a normal
life expectancy. In the rare instance of cardiac anomalies, the life expectancy
is determined by the heart condition and not the presence of a situs inversus.
The male:female ratio is 1:1.
Autosomal recessive inheritance and congenital
Unknown. Abnormal rotation of the viscera in utero
results in the orientation of the lungs and abdominal organs being reversed.
Dextrocardia may or may not be present. The occurrence of situs inversus
viscerum with levocardia is associated with severe congenital heart disease.
Radiological and echographic demonstration of abnormal orientation
of organs with or without dextrocardia.
Situs inversus may remain asymptomatic throughout
life, with little impact on cardiorespiratory function. Situs inversus is a
feature of Kartagener syndrome, asplenia syndrome, and polysplenia syndrome.
Clinical features of these medical conditions may therefore be present. Congenital heart
disease described with situs inversus includes ventricular septal defect,
atrial septal defect, transposition of the great arteries, and conotruncal
Examine for features of Kartagener
syndrome (increased risk of pneumonia and perioperative respiratory
embarrassment). Consider chest radiograph. Full cardiac evaluation,
including history, examination, ECG, and echocardiography if congenital
heart disease is present. Cardiac catheter data as indicated clinically.
Anesthetic technique dictated by
presence or absence of cardiac disease. If part of Kartagener syndrome, a
technique to maximize ability to clear secretions and early use of
physiotherapy postoperatively should be considered.
There are no known specific
implications with this condition.
Situs Ambiguous: When the situs orientation cannot be determined, the
patient has situs ambiguous or heterotaxy. The liver may be midline, the
spleen absent or multiple, the axial morphology unclear, and the bowel
malrotated. Two primary subtypes include: right isomerism or asplenia
syndrome, and left isomerism or polysplenia syndrome.
Kartagener Syndrome: The association of
situs inversus and ciliary dyskinesia is known as the Kartagener syndrome.
Genetically transmitted polymalformative syndrome characterized by
bronchiectasis, situs inversus, and chronic sinusitis. Twenty percent of
patients with situs inversus are affected with Kartagener syndrome.
Mathew PJ, Sadera GS, Sharafuddin S, Pandit B. Anaesthetic considerations in Kartagener's
syndrome—a case report. Acta Anaesthesiol Scand
Sahajananda H, Sanjay OP, Thomas J, Daniel B: General anaesthesia for lobectomy in an
8-year-old child with Kartagener's syndrome. Paediatr Anaesth