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Living with Hypoplastic right heart syndrome

A heart condition present from birth in which the right side of the heart is underdeveloped, leading to low blood oxygen.

Understanding Hypoplastic right heart syndrome

Hypoplastic right heart syndrome is a rare heart condition present from birth that results in low blood oxygen levels.

The structures on the right side of the heart are underdeveloped. These are the tricuspid valve, right ventricle, pulmonary valve, and pulmonary artery. It is commonly seen with an atrial septal defect. The right side cannot give enough blood flow to the body, causing low oxygen and bluish skin (cyanosis). It differs from hypoplastic left heart syndrome. It is seen in newborns.

Signs & symptoms

GARD doesn't list specific symptoms for this condition. Its summary describes:

  • Low blood oxygen
  • Bluish skin color (cyanosis)

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 treatments or surgeries are planned?
Which specialists should be on our care team?
How will oxygen levels be monitored at home?

Quick facts

Symptoms usually startNewborn

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

Also known as: pseudotruncus arteriosus; right hypoplastic heart syndrome

Patient organizations

Source: NIH GARD · Hypoplastic right heart syndromeGARD ID 2922 · 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 Hypoplastic right heart syndrome.

Explore the Hypoplastic right heart syndrome blog

Explore research for Hypoplastic right heart syndrome

Explore actively recruiting Hypoplastic right heart syndrome studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Hypoplastic right heart syndrome 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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