An international registry of survivors with Hb Bart's hydrops fetalis syndrome

An international registry of survivors with Hb Bart's hydrops fetalis syndrome
Hb Barts Hydrops Fetalis was universally fatal until the advent of intrauterine transfusions. As the first cohort of survivors grows up, data about their long term outcomes can be collected and reported.
This is an updated report from a multicentre registry of survivors.
69 patients are in the registry including 39 who have survived past 5 years of age. Intrauterine transfusion resulted in a lower incidence of hydrops (17% vs 55%) but were not associated with differences in long term outcomes. As expected, all patients remained transfusion dependent except for those who underwent HSCT. 64% of patients had at least one congenital anomaly - limb defects being the most common. 31% of survivors had severe height and weight undergrowth. 11/33 survivors older than 5 who had neurodevelopmental testing had moderate to severe delay.
Registry data - no control over interventions.
Survivors with Hb Barts may have significant developmental issues as they grow older. Hematologists managing these patients should be aware of this complication in order to include it in counselling and to be able to implement early referral.