ABC | Volume 113, Nº6, December 2019

Original Article Avila et al. Pregnancy and complex congenital heart disease Arq Bras Cardiol. 2019; 113(6):1062-1069 Table 3 – Baseline characteristics of 40 pregnant women Clinical status Age (years), mean ± dp 16 to 41 (mean 24.5 ± 3.4) Oxygen saturation (%) 76 – 99 (mean 88.5) Hemoglobin (mg/dL) 10,5 – 22,0 (mean 14.8) Hematocrit (%) 32 – 69 (mean 47) Functional Class (NYHA) (%) I and II: 35 (79%) pts III: 9 (21%) pts IV: 0 (0%) pts Previous surgical repair 34 (77.3%) pts No previous surgical repair 8 (20%) pts Hypoxemia (Sat% < 92%) 19 (47.5%) pts Dp: standard deviation; NYHA: New York Heart Association; Sat %: oxygen saturation (measured by digital oximeter); Pt: patient. presented as median and interquartile interval and compared using the Mann-Whitney test. The values of p < 0.5 were considered significant. The SPSS software version 18.0 was used for the statistic calculations. This research was approved by the Institutional Review Board of Hospital das Clínicas of the School of Medicine of the University of São Paulo - SDC protocol 4563/17/063. Results The baseline clinical characteristics of 40 patients at the beginning of pregnancy and the types of structural cardiac lesions and previous surgical repairs, obstetric and fetal outcomes of the 42 pregnancies are shown in tables 3 and 4, respectively. The analysis of the structural or functional cardiac lesion recorded at the beginning of the pregnancy (table 4) showed: hypoplastic right ventricle in cases 2, 16, 20, 23 and 26; left ventricular dysfunction (EF < 50%) in cases 11 and 20; valvular, infundibular or supravalvular stenosis, with gradient > 50 mmHg in cases 14, 20, 24, 25, 26, 30, 31, 35 and 37; important valvular regurgitation in cases 17, 19, 27, 28 and 32. Eight (20.0%) patients were unoperated. The anatomical and functional analysis showed that 16 (40%) patients were considered as univentricular hearts. Maternal and fetal outcomes (Figure 1) Maternal and fetal success was considered in 17 (40.5%) cases, when the mother and healthy newborn were discharged from the hospital after delivery without complications. Figure 1 shows the maternal-fetal evolution and complications. Heart failure occurred in cases 5, 6, 15, 24 and 41 (Table 4) and was treated with hospitalization, strict hygiene-dietetic measures, furosemide, carvedilol or metoprolol associated or not to digitalis, when indicated. Electrical cardioversion was needed for atrial flutter treatment in case 21 (Table 4). The hospitalization duration for the treatment of complications or for childbirth planning varied between 21 and 68 days (average of 45 days). There were two maternal deaths (4.7%) related to obstetric complications: hemorrhage after delivery and preeclampsia, cases 34 and 38, respectively (Table 4). The obstetric complications are shown in Figure 1. The fetal losses correspond to miscarriages in cases 12, 17 and 37, stillbirth in case 34 and neonatal death in cases 14, 16 (premature babies) and 35 (Table 4). The delivery occurred on average at 37 weeks of gestation; 24 (54.5%) were Caesarian section due to obstetric raisons or progressive maternal clinical worsening. Among the live newborns, there were two (4.7%) cases of congenital heart disease: one with recurrence of maternal heart disease (case 25) and the other with tetralogy of Fallot (case 32); neither of the cases were preterm babies. Among of the predictive variables of maternal and fetal outcomes, hypoxemia showed a significant correlation with worse pregnancy prognosis, while prior surgery (whether the mother had been submitted to previous surgery or not) and univentricular function showed no correlation with the maternal and fetal outcomes (Table 5). Discussion This study included one substancial series of pregnant CCC patients, submitted to the multidisciplinary protocol at the Heart Disease and Pregnancy tertiary care center. This studied group represented 9.6% of 435 pregnancies in women with congenital heart diseases included in the InCor-Registry during the last decade. It is undeniable that higher post-operative survival of these patients will result in an increasing number of pregnancies in women with CCC in the near future. The CCC considered in this study were included in theWHO risk category III, 3,4 which means pregnancy is discouraged, justified by the rates of 25.5% of maternal complications and 70% of poor fetal outcome. These considerations are according to the results of this study, which recorded only 40% of successful pregnancies, i.e., healthy mothers and newborns without complications. The high rates of maternal events (36%) and fetal events (43%) are the bases for theWHO guideline that advises against pregnancy in this group of patients. However, the global experience in this clinical situation is increasing and it represents a major challenge for clinicians. Occasionally, women become pregnant without prior counselling or sometimes they desire a pregnancy despite the advice against it. 7 The diversity of the anatomical and functional conditions of the heart defects in CCC restrict the creation of management protocols for eventual complications during pregnancy, delivery and postpartum. 7 However, knowledge of the most common complications that occur in the late postoperative period of CCC helps in the management of pregnancy in these patients. In this regard, a study about the causes of death in patients with CCC showed that heart failure, sudden death, ischemic heart disease and infective endocarditis were the most common ones. In addition, the most significant anatomical lesions (except for Eisenmenger's syndrome) were the transposition of the great arteries and the Fontan circulation. 8 A study of 120 necropsies of congenital heart disease patients 9 confirmed heart failure as the main cause of death, since the ventricular remodeling in response to volumetric and pressure overload during life favors fibrosis, hypertrophy and a reduced number of myocardial interstitial capillaries. Thromboembolism, which is the second cause of 1064