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Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency

González-Quevedo T1,6,10,*, Larco JI2*, Marcos C4,7,10, Guilarte M3,7,10, Baeza ML5,8,10, Cimbollek S1,6,10, López-Serrano MC2,9,10, Piñero-Saavedra M1,10, Rubio M5,8, Caballero T2,9,10

Both authors contributed equally to this study and to the manuscript
1Allergy Department, Hospital Universitario V del Rocío, Sevilla, Spain
2Allergy Department, Hospital Universitario La Paz, Madrid, Spain
3Allergy Department, Hospital Universitario Vall d’Hebron, Barcelona, Spain
4Allergy Department, Complexo Hospitalario Universitario de Vigo, Vigo, Spain
5Allergy Department, Hospital General Universitario Gregorio Marañon, Madrid, Spain
6Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain
7Vall d´Hebrón Institut de Recerca (VHIR), Barcelona, Spain
8Institute for Health Research, Gregorio Marañon, (IIS-GM), Biomedical Research Network on Rare Diseases-U761, Madrid, Spain
9Hospital La Paz Institute for Health Research (IdiPaz), Biomedical Research Network on Rare Diseases-U754, Barcelona, Spain
10Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB)

J Investig Allergol Clin Immunol 2016; Vol 26(3) : 161-167
doi: 10.18176/jiaci.0037

Background and Objective: There is little information on pregnancy and delivery in patients with hereditary angioedema due to C1 inhibitor deficiency (C1INH-HAE). The aim of this study was to describe the effect of pregnancy and deliveries on symptoms of C1INH-HAE and review the need for and safety of treatments available during the study period.

Methods: Retrospective review using a purpose-designed questionnaire of 61 C1INH-HAE patients from 5 hospitals specialized in the management of HAE in Spain. The outcomes measured were number of pregnancies, changes in symptoms during pregnancy and delivery, mode of delivery, type of anesthesia during delivery, treatments received, and tolerance of treatments.

Results: We reviewed 125 full-term pregnancies (89 without a prior diagnosis of C1INH-HAE), 14 miscarriages, and 4 induced abortions. Patients reported an increased frequency of C1INH-HAE symptoms in 59.2% of pregnancies (74/125) and the presence of symptoms throughout pregnancy in 40% (50/125). Prophylactic C1INH-HAE therapy was used during 9 (7.2%) of the 125 pregnancies. Nine patients—in 11 pregnancies (8.8 %)—received treatment for acute attacks. Most deliveries (n=110, 88%) were vaginal. A cesarean section was necessary in 15 cases (12%). Short-term prophylaxis with pdhC1INH was administered before 14 deliveries (11.2 %); 111 deliveries (88.8 %) were performed without premedication and were well tolerated. Anesthesia was used in 51 deliveries (40.8%).

Conclusions: Pregnancy has a variable influence on the clinical expression of C1INH-HAE. Attacks tend to occur more frequently but not to increase in severity. Vaginal delivery was mostly well tolerated. pdhC1INH prophylaxis should be administered prior to cesarean delivery and is also recommended before vaginal delivery if there are additional risk factors. pdhC1INH should always be available in the delivery room.

Key words: Hereditary angioedema. Pregnancy. Delivery. C1 inhibitor. Anesthesia. Treatment.