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

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.

Management of pregnancy and delivery in patients with hereditary angioedema due to C1 inhibitor deficiency

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 (AU)

Antecedentes y Objetivo: Existe escasa información sobre la evolución del embarazo y el parto en pacientes con angioedema hereditario con déficit de C1 Inhibidor (AEH-C1INH). El objetivo del estudio fue describir el efecto de embarazo y parto en los síntomas de AEH-C1INH y la necesidad y seguridad de las terapias disponibles durante dicho período. Diseño: Revisión retrospectiva de datos registrados en 5 centros hospitalarios españoles expertos en AEH. Pacientes y Métodos: 61 mujeres con diagnóstico de AEH-C1INH antes o después de su(s) embarazo(s). Se rellenó un cuestionario específico. Fue evaluado: número de embarazos, evolución de síntomas de AEH durante embarazo(s) y parto(s), tipo de parto, tipo de anestesia durante el parto, tratamientos recibidos y su tolerancia. Resultados: Se revisaron 125 embarazos a término (en 89 embarazos las pacientes estaban sin diagnosticar de AEH) y 18 abortos. Hubo aumento en la frecuencia de síntomas de AEH en 59,2% de embarazos (74/125) y los síntomas estuvieron presentes a lo largo de todos los trimestres en el 40% (50/125). Se usó tratamiento preventivo en 9 de los 125 embarazos (7,2%). Nueve pacientes -en 11 embarazos- (8,8%) recibieron tratamiento para crisis agudas. 110 partos (88%) fueron vaginales, mientras que 15 (12%) fueron cesáreas. Se usó tratamiento profiláctico con concentrado de C1-Inhibidor (pdhC1INH) antes de 14 partos (11,2%). Se completaron 111 partos (88,8%) sin ningún tipo de premedicación y resultaron bien tolerados. Se usó anestesia en 51 partos (41,6%). Conclusiones: La influencia del embarazo en la expresión clínica de la enfermedad es variable, no obstante las crisis tienden a aumentar en frecuencia pero no en gravedad. El parto vaginal fue habitualmente bien tolerado. El pdhC1INH debe administrarse antes de un parto mediante cesárea y también se recomendaría en caso de parto vaginal si existiera algún factor de riesgo adicional. El pdhC1INH debe estar siempre disponible en la sala de partos (AU)

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