Overview
Dextrocardia is a condition where the heart is positioned on the right side of the chest instead of the left side, where it normally sits. The word 'dextrocardia' comes from Latin and Greek words meaning 'right heart.' This is a congenital condition, meaning a person is born with it. In some people, dextrocardia occurs on its own and causes no health problems at all. In others, it is part of a broader condition called situs inversus totalis, where all the internal organs — including the stomach, liver, and spleen — are mirrored to the opposite side of the body. This mirroring can actually be quite well-tolerated. However, dextrocardia can also occur alongside structural heart defects, such as holes in the heart, abnormal heart valves, or problems with the major blood vessels. When these defects are present, symptoms like shortness of breath, bluish skin color, and poor growth in infants may appear. Some people with dextrocardia also have a condition called primary ciliary dyskinesia (PCD), which affects tiny hair-like structures in the airways and can cause repeated lung infections. Treatment depends entirely on whether other heart or organ problems are present. People with isolated dextrocardia or situs inversus totalis and no structural defects may need no treatment at all and can live completely normal lives. Those with associated heart defects may need medications, catheter-based procedures, or open-heart surgery. Regular follow-up with a heart specialist is important for anyone diagnosed with this condition.
Key symptoms:
Heart sounds heard on the right side of the chest instead of the leftShortness of breath (if structural heart defects are present)Bluish tint to the skin or lips (cyanosis), especially in newborns with heart defectsPoor feeding and slow weight gain in infantsFatigue or low energyFrequent lung or sinus infections (if primary ciliary dyskinesia is also present)Chronic cough or mucus buildup in the airwaysAbnormal positioning of other organs found on imagingHeart murmur detected by a doctorSwelling in the legs or abdomen (in cases with heart failure)
Clinical phenotype terms (26)— hover any for plain English
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Dextrocardia.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Dextrocardia.
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Caregiver Resources
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Questions for your doctor
Bring these to your next appointment
- Q1.Does my dextrocardia occur alone, or are there other heart or organ abnormalities I should know about?,Do I need any treatment, or is monitoring alone sufficient for my situation?,Should I be tested for primary ciliary dyskinesia or other related conditions?,Are there any activities or situations I should avoid because of my heart position?,What should I tell emergency doctors or surgeons about my anatomy before any procedure?,Should other family members be screened for this condition?,How often do I need follow-up appointments with a heart specialist?
Common questions about Dextrocardia
What is Dextrocardia?
Dextrocardia is a condition where the heart is positioned on the right side of the chest instead of the left side, where it normally sits. The word 'dextrocardia' comes from Latin and Greek words meaning 'right heart.' This is a congenital condition, meaning a person is born with it. In some people, dextrocardia occurs on its own and causes no health problems at all. In others, it is part of a broader condition called situs inversus totalis, where all the internal organs — including the stomach, liver, and spleen — are mirrored to the opposite side of the body. This mirroring can actually be q
At what age does Dextrocardia typically begin?
Typical onset of Dextrocardia is neonatal. Age of onset can vary across affected individuals.
Which specialists treat Dextrocardia?
25 specialists and care centers treating Dextrocardia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.