Type of disease: Rare conditions

Dextrocardia is a group of rare heart defects present at birth (congenital). The simplest type occurs when the heart is a mirror image of a normal heart and still functions correctly. Usually the other organs of the body are also in mirror image, but again all organs work correctly. Sometimes babies with dextrocardia also have a problem with the fine hair (cilia) found in the nose and air passages (Kartagener syndrome). More commonly, dextrocardia is found with other heart defects including double outlet right ventricle, endocardial cushion defect, pulmonary atresia, single ventricle, transposition of the great vessels, or ventricular septal defects. Heterotaxy may also be present which is when the organs of the baby are not in the right place and do not work properly.

Symptoms depend on the type of dextrocardia and other birth defects present. If the heart is a complete mirror image, no symptoms may be present. If the condition is more complicated, symptoms may include pale or bluish skin, difficulty breathing, yellowing of the skin or eyes (jaundice), fatigue, slow growth and weight gain, and repeated lung and sinus infections. The cause of dextrocardia is unknown but it can run in families.

Tests including CT scans, electrocardiogram (EKG), cardiac MRI, echocardiogram (ultrasound of heart) and X-rays can confirm the diagnosis and look for other birth defects. A complete mirror image dextrocardia with no other heart defects will not need treatment. If other defects are present, treatment options, including different types of medications and surgery, will depend on the type and severity of the other defects. Research is ongoing so talk to your baby’s heart doctor and other specialist(s) about the most current treatment options. A genetic counselor and support organizations are good sources of information and can help connect you with other families affected by dextrocardia.

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