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PubMed This is a summary of 16 peer-reviewed journal articles Updated
Pediatric Cardiology

Building Your Care Team & Preparing for Interventions

At a Glance

Managing Hypoplastic Left Heart Syndrome (HLHS) requires a specialized multidisciplinary care team and treatment at a high-volume pediatric heart center. Between surgeries, parents must use an Interstage Home Monitoring Kit to closely track their baby's weight, oxygen levels, and heart rate.

Managing Hypoplastic Left Heart Syndrome (HLHS) requires more than just a surgeon; it requires a highly specialized, multidisciplinary ecosystem of care. Research shows that infants treated at high-volume centers—hospitals that perform a large number of these complex surgeries annually—often have better outcomes [1][2]. These centers are more likely to have dedicated “Single Ventricle Programs” that provide the intensive, specialized monitoring these babies need [3][4].

The Core Care Team

A comprehensive team for a child with HLHS extends far beyond the operating room. Essential specialists include:

  • Pediatric Cardiothoracic Surgeon: The lead for the staged surgeries [5].
  • Pediatric Cardiologist: Your primary partner for long-term heart health and monitoring [5].
  • Nutritionist/Dietitian: Growth is critical for heart surgery success; these specialists ensure your baby is meeting high caloric needs [6].
  • Neurodevelopmental Specialist: Children with HLHS are at higher risk for developmental or behavioral challenges [7][8]. Early and frequent assessments help catch and address these issues early [9].
  • Cardiac Social Worker/Psychologist: To address the significant emotional and psychosocial stress families experience [10][11].
  • Palliative Care Team: This team focuses on quality of life and symptom management alongside life-saving treatments [5].

Home Preparation: Your “Interstage Kit”

When you bring your baby home after the first surgery, you will essentially be running a mini-intensive care unit. Most specialized programs provide or recommend specific tools for your Interstage Home Monitoring Program (ISVMP) [12][4]:

  1. Medical-Grade Baby Scale: Unlike standard baby scales, these are highly precise and can detect even tiny changes in weight, which can indicate heart strain [4][13].
  2. Pulse Oximeter: A small sensor placed on the baby’s foot or hand to measure oxygen saturation (SpO2) and heart rate [4][14].
  3. The Medical Binder: A parent-maintained record of everything. This should include:
    • Daily Log: Records of weight, oxygen levels, heart rate, and exact feeding volumes [6].
    • Surgical Summaries: Copies of the operative reports for the Norwood and any subsequent procedures.
    • Medication List: Clear instructions on dosages and timing for daily medicines. This often includes heart medicines like Digoxin (which helps the heart pump stronger and manages heart rate) [15], and anticoagulants (blood thinners or daily Aspirin) which are universally required to prevent life-threatening blood clots in the shunts and Fontan pathways.
    • Contact Sheet: A list of 24/7 “red flag” phone numbers for the cardiology team.

Evaluating a Center

When choosing or vetting a center, look for participation in national quality initiatives. Organizations like the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) help hospitals standardize care and share data to improve survival rates for all HLHS babies [2]. A center’s willingness to be transparent about their survival statistics and their adherence to standardized monitoring protocols is a key indicator of high-quality care [1][16].

Common questions in this guide

What specialists should be on my child's HLHS care team?
A comprehensive HLHS team goes beyond a pediatric cardiothoracic surgeon and cardiologist. It should also include a nutritionist for growth management, a neurodevelopmental specialist, and psychological support for the family.
What is an interstage home monitoring kit for HLHS?
An interstage kit includes tools to safely monitor your baby at home between the first and second heart surgeries. It typically contains a medical-grade baby scale to track weight, a pulse oximeter to measure oxygen and heart rate, and a detailed medical binder.
Why are specialized baby scales needed for infants with HLHS?
Standard baby scales are not precise enough for infants recovering from complex heart surgery. A medical-grade scale can detect tiny changes in weight, which is crucial because poor weight gain or sudden fluid retention can be early signs of heart strain.
What questions should I ask when choosing a hospital for HLHS surgery?
You should ask about the hospital's annual volume of Norwood procedures and their specific 30-day and 1-year survival rates for infants with HLHS. It is also important to ask if they participate in national quality networks like the National Pediatric Cardiology Quality Improvement Collaborative.

Questions for Your Doctor

5 questions

  • •What is the annual volume of Norwood procedures performed at this center, and how many are performed by my specific surgeon?
  • •Can you provide your center's 30-day and 1-year survival statistics for infants with HLHS?
  • •Does this hospital have a formal neurodevelopmental follow-up program for children with complex heart defects?
  • •How does the multidisciplinary team (nutritionists, social workers, etc.) collaborate with us during the interstage period?
  • •Is this center a member of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC)?

Questions for You

2 questions

  • •Am I comfortable with the level of communication and support I've received from this team so far?
  • •Do I feel empowered to act as the central coordinator of my child's medical binder and home records?

References

References (16)
  1. 1

    A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations: An Analysis of 83 Patients (2015-2021).

    Bleiweis MS, Peek GJ, Philip J, et al.

    World journal for pediatric & congenital heart surgery 2022; (13(5)):664-675 doi:10.1177/21501351221088030.

    PMID: 35511494
  2. 2

    Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative.

    Clauss SB, Anderson JB, Lannon C, et al.

    Current opinion in pediatrics 2015; (27(5)):555-62 doi:10.1097/MOP.0000000000000263.

    PMID: 26208236
  3. 3

    Effectiveness of interstage home monitoring on healthcare outcomes in infants with hypoplastic left heart syndrome: a systematic review protocol.

    Andries C, Palokas M

    JBI database of systematic reviews and implementation reports 2017; (15(10)):2502-2506 doi:10.11124/JBISRIR-2016-003285.

    PMID: 29035962
  4. 4

    Multimodal Imaging from Fetal to Adult Life: A Comprehensive Approach to Hypoplastic Left Heart Syndrome (HLHS).

    Moscatelli S, Sabatino J, Leo I, et al.

    Journal of cardiovascular development and disease 2025; (12(9)) doi:10.3390/jcdd12090349.

    PMID: 41002628
  5. 5

    Provider perspective on the role of palliative care in hypoplastic left heart syndrome.

    Lowenstein S, Macauley R, Perko K, Ronai C

    Cardiology in the young 2020; (30(3)):377-382 doi:10.1017/S1047951120000128.

    PMID: 32146916
  6. 6

    Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management: A Scientific Statement From the American Heart Association.

    Rudd NA, Ghanayem NS, Hill GD, et al.

    Journal of the American Heart Association 2020; (9(16)):e014548 doi:10.1161/JAHA.119.014548.

    PMID: 32777961
  7. 7

    Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology.

    Sananes R, Goldberg CS, Newburger JW, et al.

    Pediatrics 2021; (147(2)) doi:10.1542/peds.2020-014589.

    PMID: 33441486
  8. 8

    Behavior and Quality of Life at 6 Years for Children With Hypoplastic Left Heart Syndrome.

    Goldberg CS, Hu C, Brosig C, et al.

    Pediatrics 2019; (144(5)) doi:10.1542/peds.2019-1010.

    PMID: 31628208
  9. 9

    Cognitive Development of School-Age Hypoplastic Left Heart Syndrome Survivors: A Single Center Study.

    Oberhuber RD, Huemer S, Mair R, et al.

    Pediatric cardiology 2017; (38(6)):1089-1096 doi:10.1007/s00246-017-1623-8.

    PMID: 28508919
  10. 10

    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
  11. 11

    Caring for a Child with Hypoplastic Left Heart Syndrome: Parent and Medical Perspective.

    Nicholas B, Nicholas C, Allen RL, Cua CL

    Cardiology and therapy 2022; (11(1)):9-12 doi:10.1007/s40119-021-00246-2.

    PMID: 34826115
  12. 12

    Association of a Home Monitoring Program With Interstage and Stage 2 Outcomes.

    Gardner MM, Mercer-Rosa L, Faerber J, et al.

    Journal of the American Heart Association 2019; (8(10)):e010783 doi:10.1161/JAHA.118.010783.

    PMID: 31112448
  13. 13

    Utilizing technology to expand home monitoring to high-risk infants with CHD.

    Hartman D, Ebenroth E, Farrell A

    Cardiology in the young 2023; (33(7)):1124-1128 doi:10.1017/S1047951122002232.

    PMID: 35836381
  14. 14

    Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies.

    Nieves JA, Uzark K, Rudd NA, et al.

    Critical care nurse 2017; (37(2)):72-88 doi:10.4037/ccn2017763.

    PMID: 28365652
  15. 15

    Attributable mortality benefit of digoxin treatment in hypoplastic left heart syndrome after the Norwood operation: An instrumental variable-based analysis using data from the Pediatric Health Information Systems Database.

    O'Byrne ML, Song L, Huang J, et al.

    American heart journal 2023; (263()):35-45 doi:10.1016/j.ahj.2023.05.005.

    PMID: 37169122
  16. 16

    Improvement in Interstage Survival in a National Pediatric Cardiology Learning Network.

    Anderson JB, Beekman RH, Kugler JD, et al.

    Circulation. Cardiovascular quality and outcomes 2015; (8(4)):428-36 doi:10.1161/CIRCOUTCOMES.115.001956.

    PMID: 26058717

This page provides educational information on building a care team and preparing for home monitoring for infants with HLHS. It does not replace professional medical advice from your pediatric cardiologist or surgical team.

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