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 1 – Types of Adult Patients with Congenital Heart Disease of Great Complexity Conduits, valved or nonvalved Cyanotic congenital heart disease (all forms) Double-outlet ventricle Eisenmenger´s syndrome Fontan procedure Mitral atresia; Tricuspid atresia; Pulmonary atresia Single ventricle (also called double inlet or outlet, common or primitive) Pulmonary vascular obstructive diseases Transposition of the great arteries Truncus arteriosus/hemitruncus Other abnormalities of atrioventricular or ventriculoarterial connection, not included above( crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion) Warnes AC et al. J AM Coll Cardiol 2001, 37:1161 Objectives Evaluate the evolution of complex congenital heart disease patients during pregnancy and identify variables related to poor maternal and fetal outcomes. Method This is an observational and retrospective study of 435 pregnant women with congenital heart disease, consecutively included in the InCor-Registry of Pregnancy and Cardiac Disease during a period of 10 years (2007 to 2017). Among of them, 42 pregnancies in 40 congenital heart disease patients, classified as complex by Bethesda conference 5,6 (Table 1) and included in risk category III by WHO 3,4 (Table 2) were selected for this study. During the first prenatal appointment, all the patients had anatomical and functional diagnosis defined by the InCor- Congenital Heart Disease Team and began periodic follow- up every two weeks until the second trimester of pregnancy. Subsequently, this follow-up was altered to a consultation every week, during the third trimester, with the same cardiologists and obstetric teams according to the protocol established by the InCor- Pregnancy and Heart Disease Team. The protocol included: • Advice on general measures (rest, restricted physical activities, control of anemia and eventual infections, dose adjustment or substitution of drugs to adapt to the current pregnancy status); • Periodic evaluation of the oxygen saturation, maternal hematocrit and hemoglobin; • Assessment by congenital heart diseases specialists (including new echocardiographic study); • Follow-up with obstetrical team; • Elective hospitalization of high-risk patients after 28 weeks (hypoxemia, pulmonary hypertension, serious obstructive lesions and important ventricular dysfunction) and • Delivery according to obstetrical indications; • Infective endocarditis prophylaxis during delivery with intravenous Ampicillin 2.0 g associated to gentamicin 1.5 gr/kg/intramuscular, applied one hour before delivery; • Postpartum appointment for clinical check-up and collection of information on the delivery, based on the clinical summary of the hospital discharge, as well as on the maternal and newborn complications. The following maternal variables were considered for this study: age, baseline heart disease; prior heart surgery; hypoxemia (oxygen saturation< 92% at rest, measured by digital oximeter and/or clinical signs of peripheral cyanosis); maternal hematocrit and hemoglobin; ventricular dysfunction (ventricular ejection fraction (EF) ≤ 50%); occurrence of heart complications, obstetric complications and maternal death. Regarding the newborn, the variables considered were: gestational age at the delivery, fetal loss classified as: miscarriage (< 20 weeks), stillbirth (between 20 and 36 weeks) and neonatal death (up to 30 days after delivery) and malformations related to the maternal heart disease. The conditions: hypoxemia, prior heart surgery and univentricular anatomy were studied as predictive variables of maternal and fetal outcomes. Statistical analysis The categorical variables were considered in the tables containing absolute (n) and relative (%) frequencies. The association of the categorical data was evaluated using the chi-square method and, when necessary, Fisher´s exact test. The distribution of the quantitative variables regarding normality was evaluated with the Kolmogorov-Smirnov test. The variables with normal distribution were presented as mean and standard deviation and compared using Student’s t -test for independent samples. The non-parametric variables were Tabela 2 – Modified World Health Organization (WHO) classification of maternal risk during pregnancy in congenital heart disease WHO I Risk no higher of maternal mortality and very low morbidity: uncomplicated, small or mild (pulmonary stenosis; ventricular septal defect; patent ductus); successfully repaired simple lesions (atrial or ventricular septal defect; patent ductus arteriosus; anomalous pulmonary venous drainage). WHO II Small increased risk of maternal mortality and morbidity: unoperated atrial or ventricular septal, repaired tetralogy of Fallot, repaired coarctation, atrioventricular septal defect. WHO III Significant increased risk of maternal mortality and morbidity: systemic right ventricle (e.g. congenitally corrected transposition, simple transposition after Mustard or Senning repair); Fontan circulation ; cyanotic heart disease; other complex congenital heart disease WHO IV Very higher risk of maternal mortality and morbidity or severe morbidity Pulmonary arterial hypertension of any cause; severe systemic ventricular dysfunction; heart failure and LVEF <30%; severe left heart obstruction, severe (re)coarctation; Fontan with any complication. WHO: modified World Health Organization; LVEF: left ventricular ejection fraction 1063