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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Pediatric Cardiology

Orientation and Validation: Your Child's HRHS Diagnosis

At a Glance

Hypoplastic Right Heart Syndrome (HRHS) is a rare congenital heart defect where the right side of the heart is underdeveloped. Newborns can be stabilized using Prostaglandin E1 (PGE1) to maintain lung blood flow while doctors plan for either a biventricular repair or single-ventricle surgery.

Receiving a diagnosis of Hypoplastic Right Heart Syndrome (HRHS) is a staggering experience. Whether you learned this news during a routine prenatal ultrasound or in the intense hours following your baby’s birth, the feeling of “the world stopping” is a common and valid response [1][2].

HRHS is a rare and complex congenital heart defect where the right side of the heart does not develop properly. While the news is heavy, it is important to know that medical advancements have transformed the outlook for children with this condition, and there is a clear, established path forward [3][4].

Stabilizing Facts for the First Days

When you are in the “red zone” of a new diagnosis, focus on these three evidence-based realities:

  1. There is a plan. While HRHS is rare, pediatric cardiologists have standardized protocols to stabilize your baby and support their circulation [5][6].
  2. The “Ductus” buys time. Babies have a natural “back door” in their circulation called the ductus arteriosus, which typically closes within the first few days of life. Doctors can use a medication called Prostaglandin E1 (PGE1) to keep this open, ensuring blood reaches the lungs while the team prepares the next steps [6][7].
  3. Modern outcomes are improving. 1-year and 5-year survival rates for complex heart defects have improved significantly due to multidisciplinary care and refined surgical techniques [4][3].

What is HRHS?

In a typical heart, the right side is responsible for pumping oxygen-poor blood to the lungs. In HRHS, the structures on the right side are underdeveloped or “hypoplastic” (too small). This usually involves:

  • The Tricuspid Valve: The “door” between the top and bottom chambers may be too small or missing entirely (Tricuspid Atresia) [8][5].
  • The Right Ventricle: The lower pumping chamber is often small and thick-walled, making it unable to pump enough blood to the lungs [5][9].
  • The Pulmonary Valve: The “exit door” to the lungs may be blocked (Pulmonary Atresia) [5][10].

HRHS vs. HLHS

You may hear more about Hypoplastic Left Heart Syndrome (HLHS) because it is more common. While both involve an underdeveloped side of the heart, they are biologically different [11]:

  • HLHS affects the left side, which pumps oxygen-rich blood to the body.
  • HRHS affects the right side, which pumps oxygen-poor blood to the lungs.
    Because the right side’s role is to pump to the lungs rather than the whole body, the surgical options and long-term management for HRHS can sometimes eventually bypass the right heart entirely using passive blood flow, a trajectory different from HLHS [11][12].

Understanding the Rarity

HRHS is significantly rarer than HLHS. While HLHS occurs in about 2 to 3 out of every 10,000 births, HRHS (and its components like Tricuspid Atresia) is less frequent [12][13]. Because of its rarity, it is vital that your child is cared for at a high-volume pediatric cardiac center where surgeons and cardiologists see these specific variations regularly [3].

The Emotional Journey: Fetal vs. Postnatal

The timing of your diagnosis impacts your immediate emotional experience and the medical “next steps”:

  • Prenatal Diagnosis: Finding out during pregnancy allows for time to meet the surgical team and plan for a controlled delivery at a specialized hospital [14]. Research shows that while prenatal diagnosis is stressful, it is associated with lower levels of maternal depressive symptoms later on because it allows for mental and logistical preparation [2].
  • Postnatal Diagnosis: If the diagnosis happens after birth, it often begins when the baby starts to rapidly deteriorate as the ductus arteriosus naturally begins to close within the first few days of life [15]. This sudden drop in oxygen causes the baby to become cyanotic (bluish in color) [16][9]. This is a traumatic event for parents, requiring urgent medical stabilization and immediate transfer to a cardiac intensive care unit [16][15].

What Happens Next?

Your team’s primary goal is to ensure blood reaches your baby’s lungs. This may involve:

  1. Monitoring: Frequent echocardiograms (ultrasounds of the heart) to map the exact anatomy [17].
  2. Stabilization: Using PGE1 to maintain blood flow [6].
  3. Surgical Planning: Your doctors will decide if your baby’s right ventricle can eventually be “repaired” (a biventricular repair) or if they will need a series of surgeries to bypass the right side of the heart (the single-ventricle pathway) [5][9].

You do not have to process all of this at once. Focus on the next hour, the next question, and the next step your team provides. Health systems are increasingly integrating mental health support into cardiology care to help families navigate this weight [18][19].

Common questions in this guide

What is Hypoplastic Right Heart Syndrome (HRHS)?
HRHS is a rare congenital heart defect where the right side of the heart is underdeveloped. This typically involves a small right ventricle and blocked or narrowed tricuspid and pulmonary valves, which prevents the heart from effectively pumping blood to the lungs.
How is HRHS different from HLHS?
While both conditions involve an underdeveloped side of the heart, HRHS affects the right side that pumps oxygen-poor blood to the lungs. HLHS affects the left side, which pumps oxygen-rich blood to the entire body.
What happens immediately after a baby is born with HRHS?
Doctors prioritize stabilizing the baby's oxygen levels and ensuring blood reaches the lungs. They typically administer an intravenous medication called Prostaglandin E1 (PGE1) to keep the ductus arteriosus open, which acts as a temporary backdoor for blood flow until surgery.
What are the surgical options for HRHS?
Depending on how developed the right ventricle is, surgeons will either plan a biventricular repair to fix the right side or a series of surgeries called the single-ventricle pathway to bypass the right side of the heart entirely.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Is my baby's diagnosis specifically Tricuspid Atresia, Pulmonary Atresia with Intact Ventricular Septum (PA-IVS), or another variation?
  2. 2.Is the right ventricle 'pump-capable,' or are we planning for a single-ventricle surgical pathway?
  3. 3.Will my baby need a Prostaglandin E1 (PGE1) infusion immediately after birth to keep the ductus arteriosus open?
  4. 4.Does our surgical team have experience with HRHS, and what are their specific outcomes for this rare condition?
  5. 5.What are the target oxygen saturation levels for my baby, and how will they be monitored?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (19)
  1. 1

    Navigating family life with Hypoplastic Left Heart Syndrome: A qualitative study.

    Green MD, Prevost-Reilly A, Parker DM, Carpenter-Song E

    PLOS mental health 2024; (1(7)) doi:10.1371/journal.pmen.0000208.

    PMID: 40575012
  2. 2

    Prenatal diagnosis of critical congenital heart disease associated with lower postpartum depressive symptoms: a case-control study.

    Vieira DR, Ruschel PP, Schmidt MM, Zielinsky P

    Jornal de pediatria 2025; (101(1)):54-60 doi:10.1016/j.jped.2024.06.011.

    PMID: 39128828
  3. 3

    Adult congenital heart disease: Past, present and future.

    Brida M, Gatzoulis MA

    Acta paediatrica (Oslo, Norway : 1992) 2019; (108(10)):1757-1764 doi:10.1111/apa.14921.

    PMID: 31254360
  4. 4

    Outcome of cardiac surgery in adults with congenital heart disease: A single center experience.

    Abouelella RS, Habib EA, AlHalees ZY, et al.

    Journal of the Saudi Heart Association 2019; (31(3)):145-150 doi:10.1016/j.jsha.2019.05.003.

    PMID: 31198399
  5. 5

    Pulmonary Atresia With Intact Ventricular Septum With Borderline Tricuspid Valve: How Small Is Too Small.

    LaPar DJ, Bacha E

    Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual 2019; (22()):27-31 doi:10.1053/j.pcsu.2019.02.007.

    PMID: 31027561
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    Current state of the art in hypoplastic left heart syndrome.

    Birla AK, Brimmer S, Short WD, et al.

    Frontiers in cardiovascular medicine 2022; (9()):878266 doi:10.3389/fcvm.2022.878266.

    PMID: 36386362
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    Noninvasive Cardiac Output Monitoring in Newborn with Hypoplastic Left Heart Syndrome.

    Gatelli IF, Vitelli O, Chiesa G, et al.

    American journal of perinatology 2020; (37(S 02)):S54-S56 doi:10.1055/s-0040-1713603.

    PMID: 32898883
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    Strain and Rotational Mechanics in Children With Single Left Ventricles After Fontan.

    Lopez C, Mertens L, Dragulescu A, et al.

    Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography 2018; (31(12)):1297-1306 doi:10.1016/j.echo.2018.09.004.

    PMID: 30344011
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    Clinical Status and Outcome of Isolated Right Ventricular Hypoplasia: A Systematic Review and Pooled Analysis of Case Reports.

    Hirono K, Origasa H, Tsuboi K, et al.

    Frontiers in pediatrics 2022; (10()):794053 doi:10.3389/fped.2022.794053.

    PMID: 35529333
  10. 10

    Is there a transcatheter solution for a sick neonate with hypoplastic right heart syndrome?: Pulmonary valve perforation in a neonate with hypoplastic right ventricle with pulmonary atresia, restrictive VSD-a case report.

    Barwad P, Prasad K, Vijay J, Naganur S

    The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology 2020; (72(1)):64 doi:10.1186/s43044-020-00097-7.

    PMID: 32990873
  11. 11

    Common and divergent cellular aetiologies underlying hypoplastic left heart syndrome and hypoplastic right heart syndrome.

    Yu Y, Wang C, Ye S, et al.

    European heart journal 2025; (46(20)):1946-1949 doi:10.1093/eurheartj/ehaf121.

    PMID: 40048662
  12. 12

    Outcomes in Hypoplastic Left Heart Syndrome.

    Metcalf MK, Rychik J

    Pediatric clinics of North America 2020; (67(5)):945-962 doi:10.1016/j.pcl.2020.06.008.

    PMID: 32888691
  13. 13

    Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation.

    Grozdanov D, Piber N, Borgmann K, et al.

    The Journal of thoracic and cardiovascular surgery 2025; (169(5)):1317-1326.e3 doi:10.1016/j.jtcvs.2024.09.047.

    PMID: 39368733
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    From Fetal Diagnosis to Staged Percutaneous Palliation: A Case of Double Right-Valve Dysplasia.

    Bonanni F, Cordisco A, Calabri GB, et al.

    JACC. Case reports 2025; (30(40)):105852 doi:10.1016/j.jaccas.2025.105852.

    PMID: 41165639
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    [Application of arterial duct stent in ductus-dependent hypoplastic right heart syndrome].

    Luo G, Liu A, Wang KL, et al.

    Zhonghua er ke za zhi = Chinese journal of pediatrics 2020; (58(4)):319-323 doi:10.3760/cma.j.cn112140-20190907-00571.

    PMID: 32234140
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    [Analysis of 15 cases of ductus arteriosus stent placement without a guiding catheter through femoral artery approach].

    Luo G, Pan SL, Ji ZX, et al.

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    PMID: 39979105
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    Echocardiographic and pathomorphological features in fetuses with ductal-dependent congenital heart diseases.

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    PMID: 31389092
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    "There's no acknowledgement of what this does to people": A qualitative exploration of mental health among parents of children with critical congenital heart defects.

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  19. 19

    Mental Health Treatment in Adults with Congenital Heart Disease in Germany: An Online, Cross-Sectional Study of Status, Needs, and Treatment Reasons.

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This page is for informational purposes only to help parents and families understand an HRHS diagnosis. Always consult your pediatric cardiologist and surgical team for your baby's specific care plan.

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