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Living with Tricuspid atresia

A rare heart defect present at birth in which the valve between the right upper and lower heart chambers is missing or blocked.

Understanding Tricuspid atresia

Tricuspid atresia is a rare heart defect present at birth in which the tricuspid valve between the right heart chambers is absent or blocked.

The right lower chamber (ventricle) is then very small. It often comes with other heart differences, such as a hole between the heart's upper chambers, a hole between the lower chambers, narrowing of the path to the lungs, or switched main arteries. It is noticed during pregnancy or after birth.

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • Bluish skin color (cyanosis)
  • Small right lower heart chamber
  • Hole between the upper heart chambers (atrial septal defect)
  • Hole between the lower heart chambers (ventricular septal defect)
  • Switched main arteries (transposition of the great arteries)

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include heart and blood vessel specialists.

  3. Ask which tests can help

    Your care team will decide which exams and tests can confirm the diagnosis.

  4. Connect with expert centers

    Conquering CHD may keep lists of experts for this condition.

Care & treatment

Care team
Heart and blood vessel specialists (cardiologists)
Expert care
Expert lists: Conquering CHD
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 Conquering CHD
  • 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
Bring these questions to your next doctor visit
Which heart tests show how my child's heart is built?
Which specialists, such as a pediatric cardiologist, should we see?
What treatment options are there for my child's heart?

Quick facts

Symptoms usually startduring Pregnancy and as a Newborn

GARD doesn't describe a cause for this condition yet.

Also known as: congenital atresia of tricuspid valve; imperforate right atrioventricular valve; right atrioventricular valve atresia; tricuspid atresia (disease); tricuspid valve atresia

Patient organizations

Source: NIH GARD · Tricuspid atresiaGARD ID 5274 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Tricuspid atresia.

Explore the Tricuspid atresia blog

Explore research for Tricuspid atresia

Explore actively recruiting Tricuspid atresia studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Tricuspid atresia with HealthSpark. Share your city to explore research near you.

  • Recruiting studies from ClinicalTrials.gov
  • Share your city to look for research near you
  • 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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