Pregnancy with Severe Anaemia and Pulmonary Hypertension: A Presentation of Hb E –ß Thalassemia

The interaction of hbe with ß-thalassaemia results in hbeß-thalassaemia, an extremely heterogeneous clinical condition. hbe-ß-thalassaemia is the most common form of ß-thalassaemia in southeast asia and accounts for approximately 50% of cases of transfusion –dependent cases of haemolytic anaemia. still an undiagnosed population exist in our midst often presenting with complications of chronic haemolysis along with anaemia. obstetricians are further perplexed while managing these patients in pregnancy with added foetal risks. pulmonary hypertension, cardiac arrhythmia, systemic iron overload from chronic blood transfusion usually evolves from the disease itself.the risk of the foetus inheriting the trait of either ²-thalassemia or hb-e exists(25% in each pregnancy) along the possibility of being homozygous for this disorder if the father bears the carrier status(25 % in each pregnancy) cannot be overlooked. here we report a 20-year old primigravida with hb e- ß thalassemia presenting at 40 weeks of pregnancy with severe anaemia (4 gm/dl) and respiratory distress. the patient also had hepatosplenomegaly and cholelithiasis. the patient had remained undiagnosed upto the time of presentation and had remarkably received no blood transfusion since childhood despite the history of recurrent jaundice. the patient was further investigated and found to have moderate pulmonary hypertension and mild tricuspid regurgitation. after correction of her anaemia and supportive cardiac care, she delivered a male child of 2.75kg by caesarean section. her cardiac condition also significantly improved after delivery.