Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency.
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.
Allergy Department, Hospital Universitario V del Rocío, Sevilla, Spain.|Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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González-Quevedo
Allergy Department, Hospital Universitario La Paz, Madrid, Spain.
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Larco
Allergy Department, Complexo Hospitalario Universitario de Vigo, Vigo, Spain.|Vall d´Hebrón Institut de Recerca (VHIR), Barcelona, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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Marcos
Allergy Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain.|Vall d´Hebrón Institut de Recerca (VHIR), Barcelona, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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Guilarte
Allergy Department, Hospital General Universitario Gregorio Marañon, Madrid, Spain.|Institute for Health Research, Gregorio Marañon, (IIS-GM), Biomedical Research Network on Rare Diseases-U761, Madrid, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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Baeza
Allergy Department, Hospital Universitario V del Rocío, Sevilla, Spain.|Instituto de Biomedicina de Sevilla (IBIS), Sevilla, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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Cimbollek
Allergy Department, Hospital Universitario La Paz, Madrid, Spain.|Hospital La Paz Institute for Health Research (IdiPaz), Biomedical Research Network on Rare Diseases-U754, Barcelona, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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López-Serrano
Allergy Department, Hospital Universitario V del Rocío, Sevilla, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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Piñero-Saavedra
Allergy Department, Hospital General Universitario Gregorio Marañon, Madrid, Spain.|Institute for Health Research, Gregorio Marañon, (IIS-GM), Biomedical Research Network on Rare Diseases-U761, Madrid, Spain.
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Rubio
Allergy Department, Hospital Universitario La Paz, Madrid, Spain.|Hospital La Paz Institute for Health Research (IdiPaz), Biomedical Research Network on Rare Diseases-U754, Barcelona, Spain.|Spanish Group for the Study of Bradykinin induced Angioedema (SGBA/GEAB).
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Caballero
D000293:Adolescent; D000328:Adult; D054179:Angioedemas, Hereditary / Q000628:therapy*; D036861:Delivery, Obstetric; D005260:Female; D006801:Humans; D011247:Pregnancy; D011248:Pregnancy Complications / Q000628:therapy*; D012189:Retrospective Studies
Anesthesia; C1 inhibitor; Delivery; Hereditary angioedema; Pregnancy; Treatment