Philippine E-Journals

Intrauterine Fetal Blood Transfusion in Hemoglobin Bart’s Hydrops Fetalis Syndrome

Maria Angelica T. Gamo | Angelita R. Teotico

 

Abstract:

Hemoglobin Barts syndrome, or alpha-thalassemia major, is a rare but fatal syndrome that results from the deletion of all four genes of the alpha-globin loci. Due to the poor prognosis of the disease with no known definitive interventions, patients and their spouses with a fetus diagnosed with hydrops fetalis have often elected for pregnancy termination. Newer studies show that in utero fetal transfusion have led to improvements in perinatal management and outcomes of patients with fetal Hemoglobin Barts disease, providing a possible viable option for patients who are prenatally diagnosed with fetal alpha-thalassemia major. This paper presents a case of a 19-year-old primigravid who was referred to our institution at 28 3/7 weeks age of gestation for sonographic findings of double outlet right ventricle and was later diagnosed with hydrops fetalis. The patient and her spouse underwent work-up and were both found to be alpha-thalassemia carriers. On peripheral blood smear, the fetus was found to have microcytic, hypochromic red blood cells, with spherocytosis, poikilocytosis, and anisocytosis. She underwent two courses of in utero fetal transfusion. At 31 1/7 weeks age of gestation, the patient showed a non-reassuring fetal pattern consisting of persistent late decelerations on fetal heart monitoring. She underwent emergency cesarean section and delivered subsequently to a weak baby of ambiguous gender. The newborn expired shortly after. This paper aims to improve awareness and knowledge with regards to current management of pregnant patients diagnosed with hydrops fetalis arising from Hemoglobin Barts disease appropriate for our setting.