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 maternal evolution. Beta-blocker (propranolol or metoprolol succinate) use in low doses favors lower oxygen consumption, as a function of less cardiac work. 3.6.2.1. Preconception Evaluation In risk stratification for future pregnancy, the presence of coronary artery aneurysm, myocardial ischemia, and ventricular dysfunction should be considered. 3.6.2.2. Key Points • Existence of moderate coronary aneurysm (> 3 mm and < 6 mm) in one or more arteries indicates permanent use of low doses of aspirin; • Giant (> 8 mm) or multiple aneurysms, in addition to aspirin, require association with an anticoagulant; • In cases of myocardial ischemia, association of aspirin, an anticoagulant, and/or CCB is recommended. 3.6.3. Pulmonary Hypertension PH is a physiopathological condition that leads to debilitating symptoms and lower life expectancy, caused by compromised pulmonary circulation. It is defined as average resting pulmonary artery pressure (PAP) ≥ 25 mmHg, measured by right heart catheterization. It is a progressive disease, predominant in the female sex, and it may occur during the reproductive period. In general, it leads to right ventricular insufficiency with a risk of death during pregnancy, but especially during the postpartum period. 243,244 Pregnancy in women with PH is considered high risk and maternal and the neonatal complications rate achieve 50 to 70% respectively, and it has beenassociatedwithmortality rates reaching nearly 30%. 245 In view of this, pregnancy is contraindicated. Physiological changes of pregnancy, especially the decreased peripheral vascular resistance, increased cardiac output, and hypercoagulability, are reasons for maternal hemodynamic instability. In addition to this, there is the activity of sex hormones, such as beta-estradiol, progesterone, and testosterone, in pulmonary circulation; on one hand, they attenuate pulmonary vasoconstriction, and, on the other hand, they activate angiogenic factors that stimulate the proliferation of smooth muscle cells in pulmonary vasculature, predisposing them to reverse vascular remodeling. This physiopathological complexity of PH during gestation may be resumed in a single primary aspect, namely, the compensatory physiological vasodilatory response of pulmonary vasculature, which becomes decreased or absent, leading to a significant increase in pulmonary pressure and resistance. The inability of the pulmonary vascular bed to accommodate increased cardiac output results in significant disproportion in right ventricular afterload and failure. 246 The classification of PH was simplistic, divided into two groups: primary and secondary, according to identification of risk factors. However, since 1998, the WHO has proposed modifications to the classification of PH in order to allow different types of the disease to be grouped based on their physiopathology, response to treatment, and prognosis 247 (Table 25). It is worth remembering that, in this classification, the term PAH is described as a subgroup of PH, characterized by left ventricular filling pressure < 15 mmHg and pulmonary vascular resistance > 3 Wood units. Regarding diagnosis of PH, symptoms such as dyspnea, chest pain, lower limb edema, palpitation, and dry cough may be attributed to pregnancy, but the presence of syncope attributes more severity to the disease. 248 ECG and chest X-ray show right chamber overload. Transthoracic echo estimates PAP, evaluates right ventricular function, and identifies other structural heart diseases, making it possible to classify the type of PH. Definitive diagnosis is done by means of right heart catheterization and pressure measurements. 246,247 Family planning in patients with PH includes advising against pregnancy by clarifying the maternal and fetal risks, as well as the choice of an efficacious and safe means of contraception. There is no evidence to date regarding a pulmonary arterial pressure level (cutoff point) for determining prognosis for a future pregnancy. However, the pregnancy outcome is very different when subgroups for classification of PH are taken into consideration. 248 It is worth emphasizing that patients included in category 2 (Table 25), such as those with mitral stenosis, aortic stenosis, and cardiomyopathies, receive different treatment and counseling than patients included in the other categories. For this reason, risk stratification according to category and treatment strategy for pregnancy should receive interdisciplinary support in a tertiary hospital that has specialists in PH, so that the best practice may be adopted. Excluding pregnant women included in category 2, the first proposal over the course of the first trimester in patients with PAH is to interrupt pregnancy, with an emphasis on clarifying the risks of maintaining pregnancy and those of therapeutic abortion procedure. In the event that the patient does not accept this advice, the following practice is currently recommended: 249 1. Weekly interdisciplinary consultation starting at week 16 of gestation; 2. Individualized pharmacological therapy for PH; 3. Periodic evaluation of ECG, echo, and BNP during the second and third trimesters; 4. Hospitalization starting at week 28 for therapy with intermittent oxygen in accordance with arterial oxygen saturation, anticoagulation, maternal-fetal monitoring, and delivery planning; 5. Route of delivery is indicated by the obstetrician; 6. General anesthesia is preferable; 7. Anesthesia with blocks (epidural or spinal anesthesia) is contraindicated. Recommended pharmacological therapy is use of prostacyclins and their analogues and type 5 phosphodiesterase inhibitors, which seem to be safe during gestation. CCB are a safe and efficacious alternative for the subgroup of patients who present documented vasoreactivity and NYHA FC I/II without severe ventricular dysfunction; nevertheless, it is necessary to be attentive to their negative inotropic effects, in addition to arterial hypotension, which may limit their use. 250,251 890