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 PH and Eisenmenger syndrome during the first trimester of gestation. Nonetheless, when the patient decides to continue with the pregnancy, the multidisciplinary team should follow the protocols 172 that include hospitalization after week 28 of gestation, enoxaparin (LMWH) use in a prophylactic dose (1 mg/kg daily), and oxygen therapy (supplemental oxygen for saturation below 92%) are essential measures for controlling hypotension, hypoxemia, and metabolic acidosis. Specific vasodilators, such as phosphodiesterase inhibitors (sildenafil), may lead to arterial hypotension, and they should be indicated individually in accordance with clinical situation and maternal tolerance. 173,174 Sildenafil or other phosphodiesterase inhibitors have been used, as well as the eventual addition of prostaglandins when symptoms persist. Endothelin receptor antagonists should be suspended during pregnancy. 175,176 Full-dose or prophylactic LMWH should be considered as substitute of warfarin during the first trimester and after 36 th week of gestation for patients whose already using it before conception (Figure 6). The antiplatelet agents (such as aspirin) or LMWH should be prescribed with great caution, because patients with PH present a high risk of hemoptysis and thrombocytopenia. Congenital heart diseases with obstructive structural lesions: Patients with severe left ventricular outflow tract obstructions should be advised to surgical or percutaneous correction previous of gestation. If the patient is, however, already pregnant, the triad of symptoms (HF, angina pectoris, and syncope) percutaneous or surgical intervention should be considered, even during gestation. 171 In patients with severe valve pulmonary stenosis whose present heart failure, the percutaneous balloon valvuloplasty is indicated, and it is safest during the second trimester of pregnancy, when the embryogenesis phase has passed; the fetal thyroid is still inactive, and the uterus still has a small volume, allowing for greater distance between the ionizing radiation and the conceptus during the procedure. Cyanotic heart diseases without pulmonary hypertension: General measures include restricting physical activity, supplementing oxygen, and preventing venous stasis due to the known risk of paradoxical embolism. The use of LMWH in prophylactic doses is recommended, because thromboembolism is one of the main complications. Iron supplementation may be used, depending on polycythemia, similarly to Eisenmenger syndrome. 175,176 Heart diseasewith shunt without pulmonary hypertension: Atrial septal defect (ASD) is well tolerated during pregnancy and is considered WHO- risk I. 177 Arrhythmias, which are generally supraventricular, are common and they may be controlled with digoxin, beta-blockers (propranolol or metoprolol), or CCB (verapamil) in fractionated low doses. Patients with uncorrected ASD are considered to present a risk of thromboembolism, which may suggest that LMWH should be used. Although it is not routine, symptomatic patients with left-right flow and hemodynamic instability may benefit from percutaneous closure of this defect. Patientswith small or operated interventricular communication (VSD) tolerate pregnancy well and are considered WHO-risk I, especially when ventricular function is normal. The evolution of atrioventricular septal or canal defects that have not been corrected depends on the magnitude of valve regurgitation and the size of communication between chambers. It is considered WHO- risk I. The most frequent complications are arrhythmias, pulmonary congestion, and HF in patients with ventricular dysfunction. Treatment includes use of digoxin, diuretics (furosemide), vasodilators (hydralazine), or beta-blockers (carvedilol). Coarctation of the aorta: Pregnancy is tolerated in patients with corrected coarctation of the aorta, which is considered WHO-risk II. 178,179 Nonetheless, in when it has not been corrected prior to conception, there are associated complications which lead to high risks to pregnancy, such as arterial hypertension with the additional risk of preeclampsia, aortic aneurysm, dissection of the aorta, and rupture of cerebral aneurysm, which goes on to become WHO‑risk IV. It is fundamental to control arterial pressure, using conventional therapy. Tetralogy of Fallot: Tetralogy of Fallot is the most common cyanotic heart disease in adults, and patients whose have undergone corrected surgery they tolerate pregnancy very well. In this group, risk factors are right ventricular dysfunction and pulmonary insufficiency, which adequately adapt to pregnancy in most cases. 179 The current practice of replacing the pulmonary valve in the population of patients with significant right ventricular dilatation has contributed to an increasing contingent of pregnant women with pulmonary biological prostheses. Cardiac arrhythmias are common events during the late postoperative period but do not compromise obstetric and fetal outcomes. 180 Experience with unoperated tetralogy of Fallot is very limited, and it should follow the recommendations for cyanotic heart diseases. Ebstein anomaly: Prognosis for pregnant women with Ebstein anomaly is related to the presence or absence of cyanosis and HF. Hemodynamic instability is associated with tricuspid insufficiency or right ventricular dysfunction. Pregnancy should be planned following surgical correction in symptomatic patients with HF or cyanosis. Pre-excitation syndrome is commonly associated with the anomaly, and arrhythmias may be a complicating factor during gestation, even in patients who have undergone operation. 181 Transposition of the great arteries (TGA): In cases of dextro-TGA, late evolution following atrial (Senning or Mustard procedure) or arterial (Jatene surgery) switch has been positive, and pregnancy is well tolerated. 182 The presence of right ventricular dysfunction or significant tricuspid insufficiency is an important factor for poor prognosis and restricting pregnancy. 175 Practice for treating complications should follow conventional recommendations. In cases with levo-TGA, also known as ventriculoarterial and atrioventricular discordance or ventricular inversion, evolution of pregnancy depends on FC, systemic right ventricular function, arrhythmias, and associated lesions. 183 In adults, the main concern is ventricular dysfunction. 184 For these young women, pregnancy should be advised against. Fontan procedure: Successful gestations have been reported in patients who have undergone Fontan surgery, although there is a risk if Fontan circulation is not adequate, 882