Vorapong Phupong MD,*
Affiliation : * Department of Obstetrics and Gynecology, Faculty of Medicine, Chulalongkorn University
Background : Thalassemia is a common single gene disorder in Southeast Asia. α-thalassemia is a group of
syndrome  characterized  by  deficient  production  of  the α-globin  chain.  Individuals  with  heterozygous α-
thalassemia-1 are at risk of having a fetus that has Hemoglobin Bart’s hydrops fetalis (Hb Bart’s). Usually,
when  the  hemoglobin  electrophoresis  in  heterozygous α-thalassemia-1  is  normal,  the  Mean  Corpuscular
Volume (MCV) is lowered. We report a case of increased cardiothoracic ratio that led to a diagnosis of Hb
Bart’s in a couple who had normal hemoglobin electrophoresis and low MCV.
Case Report :  A  23-year-old  woman,  gravida  2,  Para  0-0-1-0,  initially  presented  for  antenatal  care  at  13
weeks  pregnancy.  Her  MCV  was  67  fentolitre,  DiChlorophenol-IndolPhenol  (DCIP)  test  was  negative  and
hemoglobin  electrophoresis  was  normal.  Her  husband’s  MCV  was  67  fentolitre,  and  hemoglobin  electro-
phoresis was normal. Cardiomegaly (an increased of the cardiothoracic ratio) was detected by ultrasonogram
at  25  weeks  of  gestation.  She  and  her  husband  were  comprehensively  counseled  after  an  Hb  Bart’s  was
suspected. A cordocentesis was performed and the fetal blood was tested for hemoglobin electrophoresis. The
result  was  later  known  and  confirmed  as  Hb  Bart’s.  The  couple  decided  to  terminate  the  pregnancy.  The
induced abortion was successful and the patient was discharged on the second day after the abortion. She was
well at the 4-week follow-up.
Conclusion :  A  prenatal  ultrasonographic  screening  should  be  conducted  in  couples  who  are  suspected  of
being α-thalassemia-1  carriers  when  DNA  study  of α-globin  gene  cannot  be  performed.  The  increase  of
cardiothoracic ratio will help detect an early stage of Hb Bart’s.
Keywords : Cardiothoracic ratio, Hemoglobin Bart’s, Hydrops fetalis, Alpha thalassemia
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