Living with Right atrial isomerism
A rare condition present from birth that affects the heart and the placement of organs, and often the spleen.
Understanding Right atrial isomerism
It is a type of heterotaxy (or laterality) disorder. It includes a missing or underdeveloped spleen, heart malformations, and an unusual arrangement of organs. It affects many organ systems and is noticed in newborns. GARD says most cases happen by chance.
Signs & symptoms
Symptoms vary from person to person. GARD lists these among the most common:
- Missing or small spleen (asplenia)
- Heart malformations
- A single shared upper heart chamber (common atrium)
- Heart pointing toward the right (dextrocardia)
- Lung veins connected in an unusual way
- Organs on the opposite side of the body
Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.
Diagnosis
- 1Talk with your doctor
Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.
- 2See a specialist
Specialists who may be involved include digestive system specialists, genetic specialists and heart and blood vessel specialists.
- 3Consider genetic testing
Changes in GDF1 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.
Care & treatment
- Care team
- Digestive system specialists (gastroenterologists); Genetic specialists (geneticists); Heart and blood vessel specialists (cardiologists)
- Expert care
- Ask about multidisciplinary care centers for rare conditions
- Treatment
- GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
- Research
- Clinical trials and observational studies may be options. See the research section below.
General information, not medical advice. Your care team makes the decisions.
Everyday life
Living with a rare condition is easier with support.
- Connect with Mended Hearts
- Keep notes on symptoms, changes and questions for appointments
- Ask about patient registries that help research
- Look into financial aid and travel resources for care visits
Quick facts
GARD says the exact cause is not known and most cases happen by chance. GARD lists the GDF1 gene and autosomal recessive inheritance, but says other causes have not been found.
Also known as: asplenia with cardiovascular anomalies; isomerism of right atrial appendage; ivemark syndrome; rai; right atrial isomerism (disease); right atrial isomerism (ivemark)
Patient organizations
- Mended HeartsRight atrial isomerism · United States
You are more than your diagnosis
Perspectives from people navigating life with Right atrial isomerism.
Explore research for Right atrial isomerism
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- Recruiting studies from ClinicalTrials.gov
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- Eligibility is confirmed by the study team
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Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.
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