The impact of in utero transfusions on perinatal outcomes in patients with alpha thalassemia major: the UCSF registry.

Alpha thalassemia major (ATM) is a hemoglobinopathy that usually results in perinatal demise if in utero transfusions (IUTs) are not performed. We established an international registry (NCT04872179) to evaluate the impact of IUTs on survival to discharge (primary outcome) as well as perinatal and neurodevelopmental secondary outcomes. Forty-nine patients were diagnosed prenatally, 11 were diagnosed postnatally, and all 11 spontaneous survivor genotypes had preserved embryonic zeta-globin levels. We compared 3 groups of patients; group 1, prenatally diagnosed and alive at hospital discharge (n = 14), group 2, prenatally diagnosed and deceased perinatally (n = 5), and group 3, postnatally diagnosed and alive at hospital discharge (n = 11). Group 1 had better outcomes than groups 2 and 3 in terms of the resolution of hydrops, delivery closer to term, shorter hospitalizations, and more frequent average or greater neurodevelopmental outcomes. Earlier IUT initiation was correlated with higher neurodevelopmental (Vineland-3) scores (r = -0.72, P = .02). Preterm delivery after IUT was seen in 3/16 (19%) patients who continued their pregnancy. When we combined our data with those from 2 published series, patients who received ≥2 IUTs had better outcomes than those with 0 to 1 IUT, including resolution of hydrops, delivery at ≥34 weeks gestation, and 5-minute appearance, pulse, grimace, activity, and respiration scores ≥7. Neurodevelopmental assessments were normal in 17/18 of the ≥2 IUT vs 5/13 of the 0 to 1 IUT group (OR 2.74; P = .01). Thus, fetal transfusions enable the survival of patients with ATM and normal neurodevelopment, even in those patients presenting with hydrops. Nondirective prenatal counseling for expectant parents should include the option of IUTs.

Interval to spontaneous delivery after elective removal of cerclage.

OBJECTIVE: The purpose of this study was to estimate the time interval between elective cerclage removal and spontaneous delivery. METHODS: Singleton pregnancies with McDonald cerclage were evaluated for the interval between elective cerclage removal (36-37 weeks) and spontaneous delivery. We also compared spontaneous delivery within 48 hours after cerclage removal between women with ultrasound-indicated vs history-indicated cerclage. RESULTS: We identified 141 women with elective cerclage removal. The mean interval between removal and delivery was 14 days. Only 11% of women delivered within 48 hours. Women with ultrasound-indicated cerclage were more likely to deliver within 48 hours, compared with women with history-indicated cerclage (odds ratio, 5.14; 95% confidence interval, 1.10-24.05). CONCLUSION: The mean interval between elective cerclage removal and spontaneous delivery is 14 days. Women with cerclage who achieved 36-37 weeks should be counseled that their chance of spontaneous delivery within 48 hours after elective cerclage removal is only 11%.