Dextrocardia with situs inversus

Overview

Type of disease: Rare conditions

Dextrocardia with situs inversus is a rare heart defect present at birth (congenital). It is the simplest type of dextrocardia and if no other birth defects are present and all organs work properly, it causes no health issues. In dextrocardia, the heart is a mirror image of a normal heart, so it is on the right side of the chest rather than the left (where the heart normally resides). Situs inversus means the other organs of the body are also in mirror image. Usually when all organs are present, just in mirror image arrangement, all the organs work properly. Sometimes the small hairs (cilia) in the nose and airways do not work correctly which may increase the risk for certain infections.

Usually with dextrocardia with situs inversus, there are no symptoms since all organs function normally. It may be picked up accidentally when a chest X-ray or some other imaging study (MRI, CT scan, ultrasound) is done for another reason. It may be found if tests are performed to find the cause of repeated respiratory or sinus infections if the cilia are not working correctly. The cause of dextrocardia with situs inversus is not known but it sometimes runs in families.

An electrocardiogram (EKG) showing an inversion of electrical waves can confirm the diagnosis as can any imaging study. Without other birth defects, a baby born with dextrocardia with situs inversus will likely live a normal, healthy life. If there is a problem with the cilia (little hairs in the nose and airways), antibiotics may be needed to prevent and fight respiratory and sinus infections. Research is ongoing, so talk to your baby’s heart doctor and other specialist(s) about the most current treatment options if cilia or other birth defects are involved. A genetic counselor and support organizations are good sources of information and can help connect you with other families affected by dextrocardia.

Connect. Empower. Inspire.