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The internal thoracic organs and the abdominal organs exhibit abnormal left-right relations
in heterotaxy syndromes.
Isomerism: "isos" means equal, "meros" means part (from the Greek).
Isomerism refers to the similarity of bilateral structures that are normally dissimilar.
common atrium,
Heterotaxy syndromes,
Heterotaxy syndrome affects up to 1 in 10,000 live births and is associated with high mortality rates.
A chest radiograph allows appreciation of tracheobronchial anatomy, the position of the cardiac apex,
Discordance of the cardiac apex and abdominal viscera is often suggestive of heterotaxy.
The sinus node is usually present bilaterally in right isomerism and absent bilaterally in left isomerism,
and total anomalous pulmonary venous drainage into a systemic vein is seen in > 50% of cases.
Right atrial isomerism is usually associated with severe cyanotic heart disease in infancy.
Accompanying heart defects may include transposition of the great arteries, common atrioventricular
(AV) valve, ventricular hypoplasia or single ventricle physiology, pulmonary atresia and pulmonary v
ein
obstruction.
The syndrome is associated with a poor prognosis and high mortality rate (80%-90%) in the first year of
life.
ECG in Right Atrial Isomerism
The sinus nodes are paired because bilateral superior vena cavae are attached to
The atrioventricular conduction system is equipped with 2 nodes often but not always connected
by a sling of tissue.
Alternating QRS patterns can be seen due to antegrade conduction through either one of the two AV
nodes.
The P-wave axis is generally normal because the right sinus node is usually the dominant atrial pacemak
er.
The most common associated cardiovascular anomalies in polysplenia are noncyanotic congenital heart
defects, including atrial septal defect, endocardial cushion defects, partial anomalous pulmonary veno
us
The most distinctive and therefore the most diagnostically useful clinical feature is inferior vena caval
interruption with azygous continuation, in which the suprarenal segment of the inferior cava is absent,
Among fetuses with complete heart block and accompanying structural heart defects,
left atrial isomerism is the most common cause.
The normal sinus node is a right-sided structure located at the junction of a right superior vena cava
In left isomerism, vena caval connections are necessarily to a morphologic left atrium,
The atrial pacemaker is therefore ectopic, and the P-wave axis is abnormal.
The ectopic atrial pacemaker can shift from one site to another (wandering atrial pacemaker) or
has a significant impact on morbidity and mortality in the fetus and neonate,
1 of 20,000 newborns. Left atrial isomerism is the most commonly associated structural cardiac defect
.
a configuration associated with a left atrial ectopic focus irrespective of atrial situs.