It is commonly associated with severe defects of the heart and related abnormalities including pulmonary hypoplasia.

[citation needed] Kartagener syndrome may also be present in patients with dextrocardia but this must be in the setting of situs inversus and may include male infertility.

Dextrocardia situs ambiguus presents a surgical challenge not per se due to associated cardiac malformation, but because achieving adequate exposure is difficult.

This presents a challenge to the surgeons operating on the right-sided cardiac structures in a case of dextrocardia situs ambiguus.

This apparent presentation is typically caused by the accidental lead placement of the left and right arm electrodes.