Hemoglobin Barts

**Semantic type:** Disease or Syndrome

**Definition:** A severe and rare form of alpha thalassemia characterized by the absence of alpha globin chains. It results in hydrops fetalis, severe anemia, hepatosplenomegaly, heart defects, and genitourinary abnormalities. It leads to death in utero or shortly thereafter.

**Synonyms:** - Bart's Hemoglobinopathy - Hemoglobin Bart's Hydrops