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Anesthetic and Obstetric Management of High-Risk Pregnancy pp 253–264Cite as

Hemoglobin Bart’s Hydrops Fetalis Syndrome

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Summary

Bart’s hydrops fetalis is a common disease in people of Southeast Asian origin. With the increasing migration of these populations to other parts of the world, it is important for all those involved in the care of obstetric population to be familiar with the management of such cases. One must pay particular attention to the blood indices available on the complete blood counts that are performed routinely in all obstetric cases. Choices must be made available to at-risk pregnancies. Timely termination of affected pregnancies would reduce the perinatal mortality and maternal morbidity associated with Bart’s hydrops fetalis. Affected pregnancies that progresses to the third trimester may be associated with maternal complications such as preeclampsia-like disease, anemia, and postpartum hemorrhage. With further research into in utero therapy, bone marrow transplantation, stem cell transplant, and gene therapy, in the future, fetuses with Bart’s hydrops may survive with a good outcome.

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References

  1. Fleischer AC, Killam AP, Boehm FH, et al. Hydrops fetalis: sonographic evaluation and clinical implications. Radiology 1981;141(1):163–168.

    CAS  PubMed  Google Scholar 

  2. Poeschmann RP, Verheijen RH, Van Dongen PW. Differential diagnosis and causes of non-immunological hydrops fetalis: a review. Obstet Gynecol Surv 1991;46:223–231.

    CAS  PubMed  Google Scholar 

  3. Macafee CA, Fortune DW, Beischer NA. Non-immunological hydrops fetalis. J Obstet Gynaecol Br Commonw 1970;77:226–237.

    CAS  PubMed  Google Scholar 

  4. Wysowski DK, Flynt JW Jr, Goldberg MF, Connell FA. Rh hemolytic disease. Epidemiologic surveillance in the United States, 1968 to 1975. JAMA 1979;242(13):1376–1379.

    Article  CAS  PubMed  Google Scholar 

  5. Andersen HM, Drew JH, Beischer NA, et al. Non-immune hydrops fetalis: changing contribution to perinatal mortality. Br J Obstet Gynaecol 1983;90(7):636–639.

    CAS  PubMed  Google Scholar 

  6. Wy Ca, Sajous CH, Loberiza F, Weiss MG. Outcome of infants with a diagnosis of hydrops fetalis in the 1990s. Am J Perinatol 1999;16(10): 561–567.

    Article  CAS  PubMed  Google Scholar 

  7. Yang YH, Teng RJ, Tang JR, et al. Etiology and outcome of hydrops fetalis. J Formos Med Assoc 1998;97(1):16–20.

    CAS  PubMed  Google Scholar 

  8. Anandakumar C, Biswas A, Wong YC, et al. Management of non-immune hydrops: 8 years’ experience. Ultrasound Obstet Gynecol 1996; 8(3):196–200.

    Article  CAS  PubMed  Google Scholar 

  9. Thumasathit B, Nondasuta A, Silpisornkosol S, et al. Hydrops fetalis associated with Bart’s hemoglobin in northern Thailand. J Pediatr 1978;73(1):132–138.

    Google Scholar 

  10. Liang ST, Wong VC, So WW, et al. Homozygous alpha-thalassaemia: clinical presentation, diagnosis and management. A review of 46 cases. Br J Obstet Gynaecol 1985;92(7):680–684.

    CAS  PubMed  Google Scholar 

  11. Heer N, Choy J, Vichinsky EP. The social impact of migration on disease. Cooley’s anemia, thalassemia, and new Asian immigrants. Ann NY Acad Sci 1998;850:509–511.

    Article  CAS  PubMed  Google Scholar 

  12. Petrou M, Brugiatelli M, Old J, et al. Alpha thalassaemia hydrops fetalis in the UK: the importance of screening pregnant women of Chinese, other South East Asian and Mediterranean extraction for alpha thalassaemia trait. Br J Obstet Gynaecol 1992;99(12):985–989.

    CAS  PubMed  Google Scholar 

  13. Prior JF, Jury KL, Erber WN. Alpha thalassaemia in western Australia. Br J Haematol 1999;105(suppl 1):81.

    Google Scholar 

  14. Chui DHK, Waye JS. Hydrops fetalis caused by alpha thalassaemia: an emerging health care problem. Blood 1998;91(7):2213–2222.

    CAS  PubMed  Google Scholar 

  15. Tongsong T, Wanapirak C, Sirivatanapa P, et al. Prenatal eradication of Hb Bart’s hydrops fetalis. J Reprod Med 2001;46(1):18–22.

    CAS  PubMed  Google Scholar 

  16. Lie-Injo LE. Alpha-chain thalassemia and hydrops fetalis in Malaya: report of five cases. Blood 1962;20:581.

    CAS  PubMed  Google Scholar 

  17. Higgs DR, Vickers MA, Wilkie AO, et al. A review of the molecular genetics of the human alpha-globin gene cluster. Blood 1989;73(5): 1081–1084.

    CAS  PubMed  Google Scholar 

  18. Xu XM, Cai XH, Li J. Molecular screening and prenatal diagnosis of the deletional alpha-thalassemia by polymerase chain reaction amplification. Zhonghua Yi Xue Za Zhi 1994;74(8):495–497.

    CAS  PubMed  Google Scholar 

  19. Lemmens-Zygulska M, Eigel A, Helbig B, et al. Prevalence of alpha-sthalassaemias in northern Thailand. Hum Genet 1996;98(3):345–347.

    Article  CAS  PubMed  Google Scholar 

  20. Ko TM, Hwa HL, Liu CW, et al. Prevalence study and molecular characterization of alpha-thalassemia in Filipinos. Ann Hematol 1999;78(8): 355–357.

    Article  CAS  PubMed  Google Scholar 

  21. Desai S, Colah R, Gupte S, Mohanty D. Is cellulose acetate electrophoresis a suitable technique for detection of Hb Bart’s at birth? Hum Hered 1997;47(4):181–184.

    CAS  PubMed  Google Scholar 

  22. Galanello R, Sanna MA, Maccioni L, et al. Fetal hydrops in Sardinia: implications for genetic counselling. Clin Genet 1990;38(5):327–331.

    Article  CAS  PubMed  Google Scholar 

  23. Mockenhaupt FP, Falusi AG, May J, et al. The contribution of alphas+-thalassaemia to anaemia in a Nigerian population exposed to intense malaria transmission. Trop Med Int Health 1999;4(4):302–307.

    Article  CAS  PubMed  Google Scholar 

  24. Pressley L, Higgs DR, Clegg JB, et al. A new genetic basis for hemoglobin-H disease. N Engl J Med 1980;303(24):1383–1388.

    Article  CAS  PubMed  Google Scholar 

  25. El-Kalla S, Baysal E. Alpha-thalassemia in the United Arab Emirates. Acta Haematol 1998;100(1):49–53.

    Article  CAS  PubMed  Google Scholar 

  26. Tan JA, Tay JS, Soemantri A, et al. Deletional types of alpha-thalassaemia in Central Java. Hum Hered 1992;42(5):289–292.

    Article  CAS  PubMed  Google Scholar 

  27. Tamary H, Klinger G, Shalmon L, et al. The diverse molecular basis and mild clinical picture of HbH disease in Israel. Ann NY Acad Sci 1998;850:432–435.

    Article  CAS  PubMed  Google Scholar 

  28. Galanello R, Aru B, Dessi C, et al. HbH disease in Sardinia: molecular, hematological and clinical aspects. Acta Haematol 1992;88(1):1–6.

    CAS  PubMed  Google Scholar 

  29. Martinez G, Ferreira R, Hernandez A, et al. Molecular characterization of HbH disease in the Cuban population. Hum Genet 1986;72(4): 318–319.

    Article  CAS  PubMed  Google Scholar 

  30. Chen FE, Ooi C, Ha SY, et al. Genetic and clinical features of hemoglobin H disease in Chinese patients. N Engl J Med 2000;343(8):544–550.

    Article  CAS  PubMed  Google Scholar 

  31. Yang CP, Hung IJ. Hematological data analysis in children with thalassemia trait or hemoglobin H disease in Taiwan. J Formos Med Assoc 1991;90(6):591–597.

    CAS  PubMed  Google Scholar 

  32. Bunyaratvej A, Butthep P, Fucharoen S, Saw D. Erythrocyte volume and haemoglobin concentration in haemoglobin H disease: discrimination between the two genotypes. Acta Haematol 1992;87(1–2):1–5.

    CAS  PubMed  Google Scholar 

  33. Wongchanchailert M, Laosombat V, Maipang M. Hemoglobin H disease in children. J Med Assoc Thail 1992;75(11):611–618.

    CAS  Google Scholar 

  34. Basu D, Singh T, Chopra K, et al. Hemoglobin H disease: a report of five cases. Indian Pediatr 1993;30(6):791–793.

    CAS  PubMed  Google Scholar 

  35. Weatherall DJ. Thalassemia in the next millennium. Keynote address. Ann NY Acad Sci 1998;850:1–9.

    Article  CAS  PubMed  Google Scholar 

  36. Ko TM, Xu X. Molecular study and prenatal diagnosis of alpha-and beta-thalassemias in Chinese. J Formos Med Assoc 1998;97(1):5–15.

    CAS  PubMed  Google Scholar 

  37. Merritt D, Jones RT, Head C, et al. Hb Seal Rock [(alpha 2) 142 term → Glu, codon 142 TAA → GAA]: an extended alpha chain variant associated with anemia, microcytosis, and alpha-thalassemia-2 (−3.7 kb). Hemoglobin 1997;21(4):331–344.

    CAS  PubMed  Google Scholar 

  38. Jiang NH, Liang S, Wen XJ, et al. Hb Westmead: an alpha 2-globin gene mutation detected by polymerase chain reaction and Stu I cleavage. Hemoglobin 1991;15(4):291–295.

    CAS  PubMed  Google Scholar 

  39. Wenning MR, Kimura EM, Costa FF, et al. Alpha-globin genes: thalassemic and structural alterations in a Brazilian population. Braz J Med Biol Res 2000;33(9):1041–1045.

    Article  CAS  PubMed  Google Scholar 

  40. Lau YL, Chan LC, Chan YY, et al. Prevalence and genotypes of alpha and beta thalassaemia carriers in Hong Kong: implications for population screening. N Engl J Med 1997;336:1298–1301.

    Article  CAS  PubMed  Google Scholar 

  41. Hsieh FJ, Ko TM, Chen HY. Hydrops fetalis caused by severe alpha thalassaemia. Early Hum Dev 1992;29(1–3):233–236.

    Article  CAS  PubMed  Google Scholar 

  42. Modell B, ed. Guidelines for the Control of Haemoglobin Disorders. Geneva: World Health Organization Hereditary Diseases Programme, 1994.

    Google Scholar 

  43. Kitsirisakul B, Steger HF, Sanguansermsri T. Frequency of alpha-thalassemia-1 of the Southeast Asian-type among pregnant women in northern Thailand determined by PCR technique. Southeast Asian J Trop Med Public Health 1996;27(2):362–363.

    CAS  PubMed  Google Scholar 

  44. Fucharoen S, Winichagoon P. Haemoglobinopathies in Southeast Asia: molecular biology and clinical medicine. Hemoglobin 1997;21(4):299–319.

    CAS  PubMed  Google Scholar 

  45. Hofgartner WT, West Keefe SF, Tait JF. Frequency of deletional alpha-thalassemia genotypes in a predominantly Asian-American population. Am J Clin Pathol 1997;107(5):576–581.

    CAS  PubMed  Google Scholar 

  46. Taylor JM, Dozy A, Kan YW, et al. Genetic lesion in homozygous alpha thalassaemia (hydrops fetalis). Nature (Lond) 1974;251(5474):392–393.

    Article  CAS  Google Scholar 

  47. Ottolenghi S, Lanyon WG, Paul J, et al. The severe form of alpha thalassaemia is caused by a hemoglobin gene deletion. Nature (Lond) 1974;251(5474):389–392.

    Article  CAS  Google Scholar 

  48. Fischel-Ghodsian N, Vickers MA, Seip M, et al. Characterization of two deletions that remove the entire human zeta-alpha globin gene complex (—Thai and —FIL). Br J Haematol 1998;70(2):233–238.

    Google Scholar 

  49. Sophocleous T, Higgs DR, Aldridge B, et al. The molecular basis for the haemoglobin Bart’s hydrops fetalis syndrome in Cyprus. Br J Haematol 1981;47(1):153–156.

    CAS  PubMed  Google Scholar 

  50. Kattamis C, Metaxotou-Mavromati A, Tsiarta E, et al. Haemoglobin Bart’s hydrops syndrome in Greece. Br Med J 1980;281(6235):268–270.

    CAS  PubMed  Google Scholar 

  51. Gurgey A, Altay C, Beksac MS, et al. Hydrops fetalis due to homozygosity for alpha-thalassemia-1,-(alpha)-20.5 kb: the first observation in a Turkish family. Acta Haematol 1989;81(3):169–171.

    Article  CAS  PubMed  Google Scholar 

  52. Olivieri NF. Fetal erythropoiesis and the diagnosis and treatment of hemoglobin disorders in the fetus and child. Semin Perinatol 1997;21(1): 63–69.

    CAS  PubMed  Google Scholar 

  53. Yong KN, Wadsworth D, Langlois S, et al. Thalassemia carrier screening and prenatal diagnosis among the British Columbia (Canada) population of Chinese descent. Clin Genet 1999;55(1):20–25.

    Article  CAS  PubMed  Google Scholar 

  54. Modell B, Petrou M, Layton M, et al. Audit of prenatal diagnosis for haemoglobin disorders in the United Kingdom: the first 20 years. BMJ 1997;315(7111):779–784.

    CAS  PubMed  Google Scholar 

  55. Guideline. The laboratory diagnosis of haemoglobinopathies. Br J Haematol 1998;101(4):783–792.

    Google Scholar 

  56. Mach-Pascual S, Darbellay R, Pilotto PA, Beris P. Investigation of microcytosis: a comprehensive approach. Eur J Haematol 1996;57(1):54–61.

    Article  CAS  PubMed  Google Scholar 

  57. Yeo GS, Tan KH, Liu TC. Screening for beta thalassaemia and HbE traits with the mean red cell volume in pregnant women. Ann Acad Med Singapore 1994;23(3):363–366.

    CAS  PubMed  Google Scholar 

  58. Sin SY, Ghosh A, Tang LC, Chan V. Ten years’ experience of antenatal mean corpuscular volume screening and prenatal diagnosis for thalassaemias in Hong Kong. J Obstet Gynaecol Res 2000;26(3):203–208.

    Article  CAS  PubMed  Google Scholar 

  59. Skogerboe KJ, West SF, Smith C, et al. Screening for alpha-thalassemia. Correlation of hemoglobin H inclusion bodies with DNA-determined genotype. Arch Pathol Lab Med 1992;116(10):1012–1018.

    CAS  PubMed  Google Scholar 

  60. Lam YH, Ghosh A, Tang MH, Chan V. The risk of alpha-thalassaemia in offspring of beta-thalassaemia carriers in Hong Kong. Prenatal Diagn 1997;17(8):733–736.

    Article  CAS  Google Scholar 

  61. Liu YT, Old JM, Miles K, et al. Rapid detection of alpha-thalassaemia deletions and alpha-globin gene triplication by multiplex polymerase chain reactions. Br J Haematol 2000;108(2):295–299.

    Article  CAS  PubMed  Google Scholar 

  62. Ko TM, Tseng LH, Hwa HL, et al. Misdiagnosis of homozygous alpha-thalassaemia 1 may occur if polymerase chain reaction alone is used in prenatal diagnosis. Prenatal Diagn 1997;17(6):505–509.

    Article  CAS  Google Scholar 

  63. Tongsong T, Wanapirak C, Srisomboon J, et al. Antenatal sonographic features of 100 alpha-thalassemia hydrops fetalis fetuses. J Clin Ultrasound 1996;24(2):73–77.

    Article  CAS  PubMed  Google Scholar 

  64. Lam YH, Ghosh A, Tang MH, et al. Early ultrasound prediction of pregnanies affected by homozygous alpha-thalassaemia-1. Prenatal Diagn 1997;17(4):327–332.

    Article  CAS  Google Scholar 

  65. Lam YH, Tang MH, Lee CP, Tse HY. Prenatal ultrasonographic prediction of homozygous type 1 alpha-thalassemia at 12 to 13 weeks of gestation. Am J Obstet Gynecol 1999;180(1 pt 1):148–150.

    Article  CAS  PubMed  Google Scholar 

  66. Ghosh A, Tang MH, Lam YH, et al. Ultrasound measurement of placental thickness to detect pregnancies affected by homozygous alpha-thalassaemia-1. Lancet 1994;344(8928):988–989.

    Article  CAS  PubMed  Google Scholar 

  67. Lam YH, Tang MH, Lee CP, Tse HY. Nuchal translucency in fetuses affected by homozygous alpha-thalassemia-1 at 12–13 weeks of gestation. Ultrasound Obstet Gynecol 1999;13(4):238–240.

    Article  CAS  PubMed  Google Scholar 

  68. Lam YH, Tang MH, Lee CP, Tse HY. Echogenic bowel in fetuses with homozygous alpha-thalassemia-1 in the first and second trimesters. Ultrasound Obstet Gynecol 1999;14(3):180–182.

    Article  CAS  PubMed  Google Scholar 

  69. Lam YH, Tang MH. Limb reduction defects as the sonographic manifestation of hemoglobin Bart’s disease at 10 weeks of gestation. Ultrasound Obstet Gynecol 2000;16(6):587–589.

    Article  CAS  PubMed  Google Scholar 

  70. Lam YH, Tang MH. Sonographic diagnosis of limb reduction defects in a fetus with haemoglobin Bart’s disease at 12 weeks of gestation. Prenatal Diagn 1999;19(10):983–985.

    Article  CAS  Google Scholar 

  71. Lam YH, Tang MH, Tse HY. Ductus venosus Doppler study in fetuses with homozygous alpha-thalassemia-1 at 12 to 13 weeks of gestation. Ultrasound Obstet Gynecol 2001;17(1):30–33.

    Article  CAS  PubMed  Google Scholar 

  72. Diukman R, Golbus MS. In utero stem cell therapy. J Reprod Med 1992;37(6):515–520.

    CAS  PubMed  Google Scholar 

  73. Westgren M, Ringden O, Eik-Nes S, et al. Lack of evidence of permanent engraftment after in utero fetal stem cell transplantation in congenital hemoglobinopathies. Transplantation 1996;61(8):1176–1179.

    Article  CAS  PubMed  Google Scholar 

  74. Eddleman K. In utero transfusion and transplantation in alpha thalassemia. In: Migliaccio AR (ed) Stem Cell Therapy of Inherited Disorders. Rome, 1996.

    Google Scholar 

  75. Barbour V. The balance of risk and benefit in gene-therapy trials. Lancet 2000;355(9201):384.

    Article  CAS  PubMed  Google Scholar 

  76. Rawal N. Single segment combined subarachnoid and epidural block for caesarean section. Can Anaesth Soc J 1986;32(2):254–255.

    Article  Google Scholar 

  77. Tay DH, Tay SM, Thomas E. High-volume spinal anaesthesia. A dose-response study of bupivacaine 0.125%. Anaesth Intensive Care 1992;20(4):443–447.

    CAS  PubMed  Google Scholar 

  78. Vercauteren MP, Coppejans HC, Hoffmann VH, et al. Prevention of hypotension by a single 5-mg dose of ephedrine during small-dose spinal anesthesia in prehydrated cesarean delivery patients. Anesth Analg 2000; 90(2):324–327.

    Article  CAS  PubMed  Google Scholar 

  79. Crowhurst JA, Birnbach DJ. Small-dose neuraxial block: heading toward the new millennium. Anesth Analg 2000;90(2):241–242.

    Article  CAS  PubMed  Google Scholar 

  80. Birnbach DJ. Obstetric anesthesiology in the new millennium. Anesth Analg 2000;90(5):1241–1243.

    Article  CAS  PubMed  Google Scholar 

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Authors
  1. Easaw Thomas
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  2. George S.H. Yeo
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  3. Tony Y.T. Tan
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Editors and Affiliations

  1. Harvard Medical School, USA

    Sanjay Datta MD, FFARCS(ENG) (Professor Anesthesia and Director of Obstetric Anesthesia) & David L. Hepner MD (Assistant Professor of Anesthesia and Assistant Director) (Professor Anesthesia and Director of Obstetric Anesthesia) &  (Assistant Professor of Anesthesia and Assistant Director)

  2. Brigham and Women’s Hospital, Boston, Massachusetts, 20115, USA

    Sanjay Datta MD, FFARCS(ENG) (Professor Anesthesia and Director of Obstetric Anesthesia) (Professor Anesthesia and Director of Obstetric Anesthesia)

  3. Preadmitting Test Center, Brigham and Women’s Hospital, Boston, Massachusetts, 20115, USA

    David L. Hepner MD (Assistant Professor of Anesthesia and Assistant Director) (Assistant Professor of Anesthesia and Assistant Director)

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Thomas, E., Yeo, G.S., Tan, T.Y. (2004). Hemoglobin Bart’s Hydrops Fetalis Syndrome. In: Datta, S., Hepner, D.L. (eds) Anesthetic and Obstetric Management of High-Risk Pregnancy. Springer, New York, NY. https://doi.org/10.1007/0-387-21572-7_17

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  • DOI: https://doi.org/10.1007/0-387-21572-7_17

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