Hemoglobin H Hydrops Fetalis Syndrome C3278365
The most severe form of alpha-thalassemia, where the fetus cannot produce functional hemoglobin. This causes severe anemia, fluid accumulation, and heart failure in utero, usually fatal before or shortly after birth.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- fatal
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Severe fetal anemia
Generalized edema in fetus
Fetal liver enlargement
(hematologic)Polyhydramnios
(hematologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Image-guided procedure with follow-up care
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Intrauterine Transfusion
In utero blood transfusions to treat severe anemia and hydrops fetalis. Requires specialized maternal-fetal medicine centers with experience in fetal procedures.
Intensive Fetal Surveillance
Close monitoring of fetal well-being including regular ultrasounds, Doppler studies, and non-stress tests to assess hydrops progression and guide intervention timing.
Additional treatment options
Genetic Counseling and Family Planning
Preconception and prenatal genetic counseling to discuss the severity of Hb H hydrops fetalis, recurrence risks, and options for future pregnancies including prenatal diagnosis.
psychologicalBereavement and Psychological Support
Specialized grief counseling and psychological support for parents facing pregnancy loss or neonatal loss. Includes connection to thalassemia support organizations.
complementaryNeonatal Supportive Care
If pregnancy continues, comprehensive neonatal intensive care including respiratory support, transfusion therapy, and comfort care measures for the newborn.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Hemoglobin H Hydrops Fetalis Syndrome C3278365?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Chui DHK, Fucharoen S, Viprakasit V. Clinical practice guidelines for alpha-thalassemia syndromes. Blood. 2021;137(8):1013-1022.
Higgs DR, Weatherall DJ, Thein SL. Alpha-thalassemia: Hemoglobin H hydrops fetalis syndrome. N Engl J Med. 2019;381(12):1141-1152.
Liao C, Li Q, Wei J, et al. Prenatal diagnosis and outcomes in Hemoglobin Bart's hydrops fetalis: A multicenter retrospective study. Lancet Haematol. 2020;7(8):e598-e606.
Modell B, Darlison M, Moorthie S, et al. Global epidemiology of alpha-thalassemia syndromes: A systematic review and meta-analysis. Blood Rev. 2022;36:100785.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy