ABC | Volume 113, Nº6, December 2019

Original Article Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge Walkiria Samuel Avila, 1 V eronica Martins Ribeiro, 1 Eduardo Giusti Rossi, 1 Maria Angelica Binotto, 1 Maria Rita Bortolotto, 2 Carolina Testa, 2 Rossana Francisco, 2 Ludhmilla Abraão Hajjar, 1 Nana Miura 1 Instituto de Coração do Departamento de Cardiopneumologia da Faculdade de Medicina da Universidade de São Paulo, 1 São Paulo, SP – Brazil Clinica Obstétrica do Departamento de Obstetricia e Ginecologia da Faculdade de Medicina da Universidade de São Paulo, 2 São Paulo, SP – Brazil Mailing Address: Walkiria Samuel Avila • Intituto de Coração - Cardiopneumologia – Av. Dr. Eneas Carvalho Aguiar, 44. Postal Code 05403-000, São Paulo, SP – Brazil E-mail: walkiria@incor.usp.br Manuscript received July 18, 2018, revised manuscript January 15, 2019, accepted March 10, 2019 DOI: 10.5935/abc.20190197 Abstract Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions. Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Methods: During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performedbefore gestationwere: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05. Results: Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia. Conclusions: Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers. (Arq Bras Cardiol. 2019; 113(6):1062-1069) Keywords: Pregnancy, Heart Defects, Congenital/complications Maternal Mortality, Fetal Mortality, Maternal and Fetal Outcomes. Introduction In the last decade, the continuous and progressive improvement in the surgical and late postoperative treatment has allowed an increased number of children with complex congenital heart disease (CCC) to reach childbearing age. The registry of 1000 cases of pregnant women followed at the Heart Institute of São Paulo (InCor) 1 between 1989 and 1999 showed that during that 10-year period, congenital heart diseases corresponded to 19.2% of the cases, which represented the second most frequent structural cardiac lesion. Among them, less than 1% were congenital complex lesions. However, over the last 50 years, there has been a significant tendency in our country to an increase in the percentage of CCC during pregnancy, as observed throughout the world, as shown by the European Registry for Cardiac Diseases in Pregnancy ( ROPAC), in which 20% of 66% of pts with congenital heart disease had complex heart defects. 2 This setting required a risk stratification scheme to predict adverse outcomes in pregnant women with congenital heart diseases seeking guidance to conceive. The modified World Health Organization (WHO) 3-4 classification is the most well‑accepted risk stratification model for pregnancy in congenital heart disease patients, and it considers CCC as risk III, which means medical advice against pregnancy. Despite this advice, both desired and unplanned pregnancy rates have been gradually rising, thus increasing the number of pregnant women with CCC. The scarcity of publications on pregnancy evolution in these women motivated this study. 1062