Dextrocardia

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ORPHA:1666Q24.0
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37Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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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
DextrocardiaHP:0001651T-wave inversionHP:0010872Situs inversus totalisHP:0001696Congenital malformation of the great arteriesHP:0011603Abnormality of the spleenHP:0001743Abnormal lung lobationHP:0002101Abnormality of the pulmonary arteryHP:0004414Abnormal pulmonary situs morphologyHP:0011615Abnormality of abdominal situsHP:0011620Abnormal renal morphologyHP:0012210Abnormal reproductive system morphologyHP:0012243
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Dextrocardia.

View clinical trials →

No actively recruiting trials found for Dextrocardia at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Dextrocardia community →

Specialists

Showing 25 of 37View all specialists →
SM
Stuart Rich, MD
Specialist
PI on 1 active trial
MM
Matthew Meyer, MD
Specialist
PI on 1 active trial
ID
Ivy Modrau, MD, dr.med.
Specialist
PI on 1 active trial
HM
Heymut Omran, MD
Specialist
PI on 1 active trial
MM
Margaret W Leigh, MD
CHAPEL HILL, NC
Specialist
PI on 1 active trial
MM
Margaret Rosenfeld, MD
EVANSTON, IL
Specialist
PI on 1 active trial
MM
Michael Knowles, MD
CHAPEL HILL, NC
Specialist
PI on 2 active trials
KM
Kenneth Olivier, MD, MPH
ROCHESTER, MN
Specialist
PI on 1 active trial
KS
Kim G Nielsen, Dr Med Sci
Specialist
PI on 1 active trial
KM
Kenneth R. Olivier, MD, MPH
ROCHESTER, MN
Specialist
PI on 1 active trial
SP
Stephanie M. Ware, MD, PhD
Specialist
PI on 1 active trial
MM
Myrofora Goutaki, MD-PhD
Specialist
PI on 1 active trial
JF
Jamil A Aboulhosn, MD, FACC
LOS ANGELES, CA
Specialist
PI on 1 active trial
MM
Michael Loebinger, MD
Specialist
PI on 1 active trial
KM
Kevin Hill, MD
Specialist
PI on 1 active trial
RP
Roni R Nielsen, MD PhD
Specialist
PI on 2 active trials

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Dextrocardia.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Dextrocardia

No recent news articles for Dextrocardia.

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

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.