ABC | Volume 114, Nº5, May 2020

Statement Brazilian Cardiology Society Statement for Management of Pregnancy and Family Planning in Women with Heart Disease – 2020 Arq Bras Cardiol. 2020; 114(5):849-942 at which point the consequent complications of low cardiac output, arrhythmia, or hepatic disease arise; 185 these cases are considered WHO risk III. Pregnancy is not advised for patients whose arterial oxygen saturation is lower than 85% or patients with severe atrioventricular insufficiency, depressed ventricular function, or enteric protein loss; these cases are considered WHO-risk IV. Practice is to treat and prevent HF, arrhythmias, and thromboembolism. Obstetric and fetal evolution in patients who have undergone the Fontan procedure is uncertain and complicated due to the high incidence of spontaneous abortion, prematurity, small for gestational age newborns, and neonatal death. There is also a high risk of PPH, which is peculiar to this clinical situation. 185,186 Heredity:  Children of mothers with congenital heart disease have a higher risk of presenting congenital cardiac lesions, which vary according to the type of maternal defect and which are not necessarily the same as the maternal structural lesion. Fetal echo is used to detect the recurrence of congenital heart disease, which is around 2.7% to 10% of cases. 187 It has been verified that there are genetic syndromes associated which specific defects, such as IAC in Holt-Oram syndrome, conotruncal anomaly in DiGeorge syndrome, among others, which are transmissible. These data reinforce the recommendation for fetal echo as part of the prenatal routine for the group of women with hereditary congenital heart disease. 3.2.2. Key Points • Pregnancy planning requires determination of structural and functional diagnosis of heart disease based on laboratory and imaging examinations; • Preconception counseling should be based on WHO risk classification; • The presence of PH, cyanosis, arrhythmias, ventricular dysfunction, previous thromboembolic events, or HF adds risks to the WHO categories; • When indicated, surgical or percutaneous intervention should be performed before conception; • Pregnant women with WHO risk III/IV should be referred for specialized care in tertiary centers with the support of a Pregnancy Heart Team; • Heredity of congenital heart disease requires the performance of fetal echo as well as genetic and preconception counseling. 3.3. Cardiomyopathies Cardiomyopathies are cardiac muscle diseases that structurally and functionally compromise the heart in the absence of coronary artery disease, arterial hypertension, or valvular or congenital heart disease, which would justify the observed myocardial abnormality. According to phenotype, cardiomyopathies are classified as hypertrophic, dilated, restrictive, arrhythmogenic right ventricular, and non‑compaction. 188 This classification is fundamental for risk assessment and estimating prognosis of gestation, regardless of probable etiology. PPCM will be discussed subsequently. A retrospective study on cardiomyopathies during gestation has shown a 35% incidence of complications with 11%maternal mortality, data which are related to the form and the degree of myocardial impairment. 189,190 WHO risks III/IV includes cardiomyopathies with reduced left ventricular ejection fraction (LVEF) below 30%, with manifestations of HF, PH, and complex arrhythmias. 52 HF is the main complication, especially after the second trimester of gestation and during labor. During the immediate postpartum period, which is as sensitive as gestation, the following recommendations should be followed: caution with the use of oxytocic drugs; moderation when infusing fluids during the intrapartum period; attention to PPH, pain control, and infection prevention; and transfer to the ICU within the first 24 to 48 hours after delivery. 3.3.1. Dilated Cardiomyopathy Approximately 50% of cases of dilated cardiomyopathy are idiopathic, and 20% to 35% are hereditary; genetic mutations have been identified in almost 40%. 191 The following acquired causes stand out: viral myocarditis (coxsackievirus, parvovirus, echovirus, adenovirus), H1N1, Epstein-Barr virus, and other causes related to drug use. During family planning, when the patient intends to become pregnant, the following are recommended: (1) adjustments to maternal therapy regarding HF control, considering that essential drugs which are contraindicated during pregnancy (ACEI, ARB, neprilysin inhibitors, spirolactone, ivabradine) should be substituted; (2) patient awareness of the possible immediate and long-term impact of pregnancy on heart disease; (3) genetic counseling, given that the disease is associated with autosomal dominant inheritance, followed by autosomal recessive inheritance, and to X-chromosome linked diseases. 191 3.3.2. Hypertrophic Cardiomyopathy Global prevalence of hypertrophic cardiomyopathy (HCM) is around 0.02% to 0.2% of the population, 192 and it was estimated at 0.015% in a cohort study of pregnant women with heart disease. 145 Pregnancy constitutes a potential risk for women with HCM; its prognosis, however, is still uncertain. The great variation in the rate of cardiovascular complications during pregnancy, estimated between 5% and 40%, has been attributed to the heterogeneity of the phenotype of this heart disease. 193-195 Although they are often asymptomatic, the most frequent complaints of pregnant women with HCM are chest pain, dyspnea, syncope, and palpitation. The factors associated with worse prognosis for pregnancy are history of HF, ventricular arrhythmia, and sudden death in the family. Complications during pregnancy result from left ventricular outflow tract obstruction, diastolic dysfunction, and myocardial ischemia. Among the most frequent arrhythmias, the following stand out: atrial extrasystoles, sustained supraventricular tachyarrhythmias, and AF, which favor maternal hemodynamic instability. From the obstetric point of view, the most frequent complications are spontaneous abortion in approximately 20% of cases and low birth weight in 10%. 193-195 883